[Federal Register Volume 59, Number 28 (Thursday, February 10, 1994)]
[Unknown Section]
[Page 0]
From the Federal Register Online via the Government Publishing Office [www.gpo.gov]
[FR Doc No: 94-2844]


[[Page Unknown]]

[Federal Register: February 10, 1994]


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Part IV





Department of Health and Human Services





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Social Security Administration



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20 CFR Part 404




Revised Medical Criteria for Determination of Disability, 
Cardiovascular System; Final Rules
DEPARTMENT OF HEALTH AND HUMAN SERVICES

Social Security Administration

20 CFR Part 404

[Regulations No. 4]
RIN 0960-AA99

 
Revised Medical Criteria for Determination of Disability, 
Cardiovascular System

AGENCY: Social Security Administration, HHS.

ACTION: Final rules.

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SUMMARY: These amendments revise the criteria in the Listing of 
Impairments (the listings) that we use to evaluate cardiovascular 
impairments for adults and children who claim Social Security or 
Supplemental Security Income (SSI) benefits based on disability under 
title II and title XVI of the Social Security Act (the Act). The 
revisions reflect advances in medical knowledge, treatment, and methods 
of evaluating cardiovascular impairments.

EFFECTIVE DATE: These rules are effective February 10, 1994.

FOR FURTHER INFORMATION CONTACT: Irving Darrow, Esq., Legal Assistant, 
Office of Regulations, Social Security Administration, 6401 Security 
Boulevard, Baltimore, MD 21235, (410) 966-0512.
SUPPLEMENTARY INFORMATION: The Act provides, in title II, for the 
payment of disability benefits to workers insured under the Act. Title 
II also provides child's insurance benefits for persons who became 
disabled before age 22 and widow's and widower's insurance benefits 
based on disability for widows/widowers and surviving divorced spouses 
of insured individuals. In addition, the Act provides, in title XVI, 
for SSI payments to persons who are disabled and have limited income 
and resources. For workers insured under title II, for children of 
workers insured under title II who become disabled before age 22, for 
widows/widowers and surviving divorced spouses claiming widow's or 
widower's insurance benefits based on disability under title II, and 
for adults claiming SSI benefits based on disability, ``disability'' 
means inability to engage in any substantial gainful activity. For 
eligibility for SSI benefits as a disabled child under age 18, 
``disability'' means that the impairment substantially reduces the 
child's ability to function independently, appropriately, and 
effectively in an age-appropriate manner. Under both the title II and 
title XVI programs, disability must be due to a medically determinable 
physical or mental impairment or combination of impairments which can 
be expected to result in death or which has lasted or can be expected 
to last for a continuous period of at least 12 months.
    The listings contained in appendix 1 to subpart P of part 404 are 
incorporated by reference in subpart I of part 416. The listings are 
divided into part A and part B. The medical criteria in part A are 
applied in evaluating impairments of persons age 18 or over. The 
criteria in part A may also be applied in evaluating impairments in 
persons under age 18 if the disease processes have a similar effect on 
adults and younger persons. Part B contains medical criteria for 
evaluating impairments of persons under age 18 when the criteria in 
part A do not give appropriate consideration to the particular effects 
of the disease processes in childhood. In evaluating disability for a 
person under age 18, we first use the criteria in part B and, if the 
criteria in part B do not apply, we use the criteria in part A. (See 
Secs. 404.1525 and 416.925.)
    When parts of the listings were last revised and published in the 
Federal Register on December 6, 1985 (50 FR 50068), we indicated in the 
preamble that medical advancements in disability evaluation and 
treatment and program experience would require that the listings be 
periodically reviewed and updated. Accordingly, we published 
termination dates ranging from 4 to 8 years for each of the specific 
body system listings. These dates currently appear in the introductory 
paragraphs of the listings; the latest extension for the expiration 
date for part A of the cardiovascular regulation appeared in the 
Federal Register of July 6, 1993 (58 FR 36133). We are now updating the 
cardiovascular system listings in 4.00 (part A) and 104.00 (part B) and 
extending the effective date of these revised listings for 4 years from 
the date of their publication. Therefore, 4 years after publication of 
the final rules, these regulations will no longer be effective unless 
extended by the Secretary or revised and promulgated again.
    We published these regulations in the Federal Register on July 9, 
1991 (56 FR 31266) as a Notice of Proposed Rulemaking (NPRM). 
Interested persons, organizations, Government agencies, and other 
groups were given 60 days to comment. The comment period ended 
September 8, 1991. Twenty-four commenters provided comments on the 
NPRM. Below we discuss the significant differences between the final 
rule and the proposed rule, and we also respond to the substantive 
public comments we received on the NPRM.

Explanation of the Final Rules

    We have updated these final rules to provide criteria reflecting 
state-of-the-art medical science and technology. The basic approach 
underlying the final listings is to place less emphasis on the 
diagnosis of disease, and to emphasize the impact of the impairment(s) 
on a person's ability to perform gainful activity or, in the case of a 
child under the SSI program, on the child's ability to perform age-
appropriate activities.
    The listings contain examples of some of the most frequently 
encountered impairments in the disability program. The criteria include 
specific symptoms, signs, and laboratory findings that are considered 
to characterize impairments severe enough to prevent a person from 
doing work-related activities, or in the case of a child claiming SSI 
benefits under title XVI of the Act, an impairment severe enough to 
prevent the child from functioning independently, appropriately, and 
effectively in an age-appropriate manner. The listings help to ensure 
that determinations of disability have a sound medical basis, that 
claimants receive equal treatment through the use of specific criteria, 
and that people who are disabled can be readily identified and awarded 
benefits if all other factors of entitlement or eligibility are met.
    Claimants may be found disabled based on medical factors alone if 
their impairment(s) meets or equals one of the sets of medical 
criteria. If the severity of a claimant's impairment(s) does not meet 
or equal the severity in the medical listings, we then assess the 
claimant's residual functional capacity to determine what he or she can 
still do; or in the case of a child claiming SSI benefits under title 
XVI of the Act, we perform an individualized functional assessment. 
Using the residual functional capacity assessment, we determine whether 
the person retains the capacity to perform past relevant work; if not, 
we determine his or her capacity to do any other work that exists in 
the national economy, considering the individual's age, education, and 
work experience. If the person is a child claiming SSI benefits under 
title XVI of the Act, we use the individualized functional assessment 
to determine whether he or she has an impairment(s) of comparable 
severity to one that would disable an adult. Thus, we do not deny any 
claim of disability on the sole basis that the individual's 
impairment(s) does not meet or equal in severity the criteria of a 
listing.
    As in the NPRM, the final rules contain some criteria in the 
listings for chronic heart failure (4.02) and ischemic heart disease 
(4.04) based on exercise test results; however, in response to 
comments, we have removed the general rules requiring the purchase of 
exercise testing in all cases in which exercise testing poses no 
significant risk to the individual. A number of commenters, nearly all 
of whom were not physicians, were concerned with our proposal to expand 
the use of the exercise test and expressed the view that other 
evidence, particularly angiography, should be afforded as much 
consideration. Although we agree with the commenters that cardiac 
angiography is the best diagnostic test for the presence of coronary 
atherosclerosis, the angiogram provides only confirmatory anatomic 
information supporting the diagnosis of coronary atherosclerosis and 
does not help in any way to quantify functional performance. Moreover, 
even angiography has some limitations because of inter-observer 
variability.
    In these listings, we do not use the exercise test for diagnostic 
purposes. The purpose of these listings is to subordinate the 
diagnostic use of the exercise test and to recognize its usefulness as 
a functional test of aerobic capacity. When viewed in this light, 
exercise testing is useful for defining the safe limits of exercise, 
the aerobic exertional threshold of angina pectoris, or left 
ventricular failure. If a person can exercise to greater than 5 METS 
without evidence of overt failure, ischemic discomfort, or hypotension, 
it can be reasonably inferred that this is a safe level of aerobic 
exertion. Even at less than 5 METS, an exercise test is some evidence 
of an individual's capacity--or lack of capacity--for aerobic exertion. 
However, we agree with the commenters that one should not rely solely 
on the results of an exercise test; as with any evidence, exercise test 
results must be considered in the context of the entire record.
    Even though we have retained in the final rules some criteria for 
exercise testing, we want to emphasize that we do not deny any 
individual's claim solely because his or her impairment(s) does not 
meet or equal in severity any listing. Under the sequential evaluation 
processes set out in Secs. 404.1520, 416.920, and 416.924 of our 
regulations, we provide every claimant whose severe impairment or 
combination of impairments does not meet or equal in severity a listing 
with an individualized assessment of his or her functioning and an 
opportunity to establish that he or she is disabled. This assessment of 
functioning considers all relevant evidence.
    In response to public comments, we have also deleted all of the 
references in the listings to the New York Heart Association (NYHA) 
Functional Criteria, which we had proposed to use in several listings 
under 4.02, 4.04, and 4.06. Instead, under final listings 4.02 and 
4.04, we have replaced the references with narrative criteria that 
describe the functional classes; in final listing 4.06, we deleted the 
references to functioning entirely because they were unnecessary. We 
describe the criteria and our reasons for making these changes later in 
this preamble.
    We made a number of other changes in response to the public 
comments, which are all described below or in the public comments 
section of this preamble. In addition, it was apparent to us from some 
of the comments that it would be helpful and clearer if we used the 
same or similar language in Parts A and B of the listings when we 
intended the provisions to be analogous. We have, therefore, made a 
number of conforming revisions in Parts A and B. In the majority of 
cases, these changes are not substantive; we describe all of them below 
in the summary of provisions or in the public comments section of this 
preamble.
    Finally, we have made a number of minor editorial changes 
throughout the rules to correct errors in the NPRM, to make the rules 
internally consistent, and to conform the style of these listings to 
our other listings. For instance, we deleted the word ``listing'' 
before all references to specific listings because that is the style 
throughout appendix 1. We also revised the opening paragraphs of 
several of the listings in Parts A and B so that they use the same or 
similar language to introduce the subparagraphs.
    The following is a summary of the provisions of the final rules and 
the changes we have made from both the text of the NPRM and the text of 
our former rules.

Revisions to Part A (Adult Portion) of Appendix 1

4.00  Preface

4.00A  Introduction
    We have reorganized the introductory portions of the cardiovascular 
listings to facilitate their use. Final 4.00A, ``Introduction,'' is a 
new paragraph that explains the basic approach used in evaluating 
cardiovascular impairments. It includes general information about the 
kinds and extent of documentation we generally require, and stresses 
the importance of a longitudinal clinical record to the assessment of 
severity and duration, when such a record is available.
    We made a number of changes from the NPRM in response to public 
comments. There are now four paragraphs in the final 4.00A. The first 
paragraph is a general paragraph based on the first two sentences of 
the NPRM. We revised the first sentence from the NPRM to make it more 
listings-specific; that is, to state that the ``listings in this 
section describe impairments resulting from cardiovascular disease.'' 
We also changed the word ``heart'' to ``cardiovascular'' because it 
more accurately describes the content of the listings. In the second 
sentence, we added a clause which says that a longitudinal record of 3 
months is not necessary if the claim can be decided favorably based on 
the current evidence. The third sentence is new. It reaffirms our 
general policy that all relevant evidence must be considered in 
assessing disability.
    The second and third paragraphs of final 4.00A address issues of 
treatment. They reorganize and revise the third and fourth sentences 
from the NPRM, and expand their discussion. The first sentence of the 
second paragraph, which corresponds to the fourth sentence in the NPRM, 
now states that many individuals with listing-level impairments will 
have received the benefit of a medically prescribed therapeutic 
program; this is in contrast to the NPRM statement that ``most'' 
individuals with cardiovascular disease have ``usually'' received such 
treatment. In response to comments that asked whether we would consider 
treating source opinions in our determinations, we added the clause, 
``in addition to information about the nature and severity of the 
impairment,'' to the end of the second sentence, which includes 
language from our rules on the evaluation of treating source opinions. 
We expanded the provisions at the end of the second paragraph to state 
more clearly the reasons why it is important to establish a 
longitudinal record and document treatment and response.
    We added the third paragraph in response to comments which pointed 
out that some people do not receive treatment, and that some do not 
have an ongoing relationship with the medical community. The new 
paragraph provides that it is still important to establish a 
longitudinal record in these cases unless the case can be decided 
favorably based on the available evidence. It also explains that, even 
though individuals who do not receive treatment cannot be found to have 
impairments that ``meet'' the criteria of several of the listings, they 
may still have impairments that are equivalent in severity to listed 
impairments, or that are disabling at the last step of the sequential 
evaluation process. To be consistent with the adult rules, we also 
added a fifth paragraph to final 104.00A of the preface to the 
childhood listings; the paragraph contains the same provisions as the 
adult rules.
    The fourth paragraph is a new paragraph that emphasizes the 
importance of the last point in all cases in which impairments do not 
meet the criteria of a listing. We added this paragraph in response to 
many commenters who thought that we would deny claimants whose 
impairments did not meet the requirements in the listings, or who 
thought that the listings criteria were the only criteria we considered 
in the steps of sequential evaluation beyond the listings step. It is a 
reminder that claimants whose impairments do not ``meet'' any listing 
may still be found to have impairments that ``equal'' a listing, and 
that the listings can only be used to find a person disabled, never 
``not disabled.'' The new paragraph stresses the importance of an 
individualized residual functional capacity assessment, which may or 
may not result in a finding of disability, whenever a person's severe 
impairment(s) does not meet or equal the severity of a listing. To be 
consistent with the adult rules, we also added a sixth paragraph to 
104.00A of the preface to the childhood listings emphasizing the 
importance of equivalence determinations and individualized functional 
assessments when childhood impairments do not meet a listing.
    We edited slightly and have moved the fifth sentence from proposed 
4.00A to final 4.00C2d because it is guidance relevant to the purchase 
of exercise testing.
4.00B  Cardiovascular Impairment
    We moved the discussion that was in 4.00A of our former rules to 
final 4.00B, which we have retitled ``Cardiovascular Impairment.'' We 
changed the title from ``Cardiac Impairment'' to ``Cardiovascular 
Impairment'' because the section in fact addresses both kinds of 
impairments. We revised and expanded this discussion of the 
consequences of heart disease to increase its scope and to conform to 
current clinical concepts. The discussion now also includes congenital 
heart disease, because more individuals with this condition are living 
into adulthood.
    The final rule is the same as that proposed in the NPRM except that 
we added ``near syncope'' in 4.00B3 in response to a comment and, in a 
technical correction, we deleted the reference to ``extremities (4.11-
4.12)'' in the paragraph after paragraph 4. Impairment of the 
extremities does not result in cardiac impairment.
4.00C  Documentation
    Final 4.00C, ``Documentation,'' consolidates and augments the 
guides formerly in 4.00F, G, H, and I. We have expanded the discussion 
in 4.00Clb on the needed descriptions of protocols for various types of 
exercise tests, including treadmill, bicycle, and arm ergometry.
    The final rules in 4.00C1a are the same as those in the NPRM. In 
4.00C1b, we made three changes in response to public comments: In final 
4.00C1b(1), we changed the word ``should'' to ``must'' to state the 
rule more straightforwardly; in final 4.00C1b(5), we updated the 
reference to the American Heart Association standards; and in final 
4.00C1b(6) we added text which recognizes that information will not 
always be available in the existing medical evidence about whether an 
individual supported himself or herself on the handrails or about the 
Borg scale.
    In responding to a comment, we also made a technical correction in 
4.00C1b(4), which had inadvertently misstated how hyperventilation 
studies are performed. The NPRM stated that the posthyperventilation 
electrocardiogram (ECG)--that is, the ECG taken to assess the effects 
of hyperventilation--should be deferred until at least 10 minutes after 
exercise; because hyperventilation is performed before exercise, this 
would have made the instruction meaningless. We revised the criterion 
to explain that it is the exercise that should be deferred for 10 
minutes after hyperventilation, which is what we intended to state. 
Finally, we made minor editorial changes in the section, such as 
substituting ``ECG'' for the word ``electrocardiogram'' in 4.00C1b(1) 
and changing the word ``references'' to ``reference'' in the last 
sentence of the paragraph.
    We substantially revised 4.00C2 from the language of the NPRM in 
response to comments. We changed the heading of the section to 
``Purchasing exercise tests'' in order to narrow the focus of the 
section. We revised the first sentence of final 4.00C2a to state more 
accurately that it is well recognized that exercise testing is the best 
tool currently available for estimating maximal aerobic capacity, 
instead of the more general statement about the assessment of 
functional capacity we had proposed. We did this because we agreed with 
those commenters who pointed out that exercise testing does not assess 
all kinds of functional capacities, only certain functions. We deleted 
without replacement the proposed second and third sentences of the 
paragraph. The proposed second sentence was the sentence which stated 
that exercise tests are the primary basis for evaluating functional 
capacity under this listing, which was an inaccurate statement. The 
third sentence was an absolute requirement to include existing exercise 
test results in the evidence. Aside from the fact that it was not a 
rule having to do with the purchase of exercise testing, it also left 
no leeway for the possibility that we would be unable to obtain the 
evidence despite diligent efforts; we deleted the sentence (instead of 
moving it) because inherent in our rules for gathering evidence is the 
need to make every reasonable effort to obtain any relevant evidence, 
including exercise test results.
    We then revised the remainder of the paragraph to better explain 
our rules on the purchase of exercise testing and when such purchase 
could be appropriate. We explain these revisions in more detail, and 
our reasons for making them, in the public comments section of this 
preamble. The final rule provides that before purchasing any tests when 
exercise is involved, a program physician, preferably one experienced 
in the care of patients with cardiovascular disease, should review the 
clinical record to determine whether the test presents a significant 
risk to the individual. This requirement recognizes that technically 
sophisticated medical reports of record need to be reviewed by a 
physician to assess potential risk of exercise testing.
    Final 4.00C2b replaces former 4.00G2. We have removed the 
requirement that the targeted heart rate should not be less than 85 
percent of maximum predicted heart rate during exercise testing because 
the listings do not use the exercise test for diagnosis but for 
functional evaluation.
    In a technical change for consistency with final 4.00C1b, we 
revised final 4.00C2b(1) to state that a purchased exercise test must 
be performed using a generally accepted protocol, that the protocol 
that was followed must be reported, and that the test must meet the 
requirements of 4.00C1b and 4.00C2b. As in final 4.00C1b(6), we deleted 
the requirement for information about whether an individual supported 
himself or herself on the handrails and about the Borg scale. We also 
deleted the proposed requirement for a description of whether the 
individual was allowed to support himself or herself on the handrails 
for the reasons we explained in the comments and responses. In response 
to a comment, we added a sentence about the significance of isolated 
systolic hypertension at the end of 4.00C2b(4); and we deleted proposed 
4.00C2(5) and renumbered proposed 4.00C2b(6) as final 4.00C2(5). In 
4.00C2b(5), we made conforming changes to permit the use of generally 
accepted standards for the exercise laboratory.
    Final 4.00C2c discusses risk factors that will preclude the 
purchase of exercise testing; it replaces the first paragraph of former 
4.00G3. In response to comments, we have expanded the list of risk 
factors in final 4.00C2c to include several conditions from the prior 
rules that were not in the NPRM; we have also added cardiomyopathies to 
the list, and made clear that the risk factors listed are only 
examples; we changed the word ``should'' to ``will'' in the opening 
clause to make it clear that we will not purchase exercise tests in the 
situations listed. We removed the example of arterial dissection after 
coronary angioplasty because it was medically inappropriate. We explain 
our reason for this change in the public comments section of this 
preamble. When the risk factors in 4.00C2c are not present, a claimant 
may still be at risk for exercise testing, and the program physician 
retains the authority to decide whether a claimant would be at risk. 
However, we revised and expanded the section to state more plainly that 
it should be a rare case in which the opinion of a treating physician 
that a claimant would be at risk to exercise would be overridden and, 
if it is, that the program physician's reasons for overriding the 
opinion must be documented. We also expanded the section to indicate 
that we will purchase an exercise test only if it poses no risk to the 
individual and the claim cannot otherwise be favorably decided.
    The NPRM language of 4.00C2d, which provided guidance for deferral 
of exercise testing following surgery, is unchanged in the final rule. 
We did, however, add a cross-reference to 4.00D for clarity. We also 
edited slightly and moved the fifth sentence of proposed 4.00A into 
this section, as previously described.
    In a technical correction, we moved proposed 4.00C2e and 
redesignated it as final 4.00C2e(2). The paragraph discusses factors 
that limit the interpretation of exercise tests. Therefore, we believe 
that it more logically belongs under the heading of ``Evaluation,'' 
rather than ``Purchasing exercise tests.'' We describe other changes to 
the paragraph below, under its new designation of 4.00C2e(2).
    Because of the redesignation of proposed 4.00C2e as 4.00C2e(2), we 
redesignated proposed 4.00C2f as final 4.00C2e(1). Final 4.00C2e(1) 
provides rules on the evaluation of exercise testing. We made a number 
of revisions and additions from the NPRM in response to comments. We 
deleted the parenthetical statement in the first sentence that 
characterized the work level at which the test becomes abnormal as an 
``ischemic threshold.'' We added a new second sentence which provides 
that the ability or inability to complete an exercise test is not, by 
itself, evidence that a person is free from ischemic heart disease and 
that the results of exercise testing must be considered in the context 
of all of the other evidence in the individual's case record. We also 
revised the last sentence of the NPRM to place the focus squarely on 
individuals who are under the care of treating physicians. We now state 
more clearly the rule from the NPRM that, if the person has a treating 
physician who has not ordered an exercise test, and there is no 
apparent reason why the person would be at risk, contact must be made 
with the source to determine the source's opinion whether exercise 
testing involves significant risk, or whether there is some other 
reason why the treating physician did not have the test performed, such 
as a financial bar. We also deleted references to the ``examining 
physician'' in response to a comment that pointed out that the rule was 
unclear; moreover, after we had published the NPRM, we published final 
rules entitled, ``Standards for Consultative Examinations and Existing 
Medical Evidence'' (56 FR 36932, August 1, 1991), in which we do not 
include the term ``examining physician'' in our definitions of 
``medical sources.'' We have also added a new sentence that underscores 
the need to follow the rules in 4.00C2c in those rare situations in 
which a treating source's opinion is overridden. Finally, we added a 
sentence setting forth the responsibility of the program physician when 
an individual does not have a treating physician. We explain all of 
these changes and additions in the public comments section of this 
preamble.
    Final 4.00C2e(2) (proposed 4.00C2e), which discusses factors that 
may limit exercise test interpretation, replaces the second and third 
paragraphs of 4.00G3 of the former listings. In addition to the 
redesignation of the paragraph, there are two changes from the NPRM. We 
added a parenthetical example, ``(e.g., 2 weeks of bedrest),'' to 
illustrate ``prolonged periods of physical inactivity''; this was in 
response to a comment that asked us to define the term and is an 
example we use elsewhere in the preface. We also deleted the example of 
Wolff-Parkinson-White syndrome, which we have instead restored to final 
4.00C2c as an example of a risk factor that precludes the purchase of 
exercise testing.
    We have moved the discussion on other studies in former 4.00I to 
4.00C3 and have expanded the discussion on the use of echocardiograms 
and radionuclide studies. Our former criteria stated that the results 
of these tests are considered but are not determinative. The new final 
criteria explain that there are several imaging techniques, including 
two-dimensional echocardiography, which can provide a reliable estimate 
of ejection fraction. Purchase of these tests is now permitted in 
selected cases; for example, when the available evidence is not 
adequate to assess the severity of ventricular dysfunction or 
myocardial ischemia. In response to a comment, however, we have added a 
clause to the last sentence of the first paragraph of the section 
reminding adjudicators that purchase of these tests is permitted only 
when the claim cannot be favorably decided on any other basis.
    In addition to the foregoing new clause, there are two other 
changes in final 4.00C3 from the NPRM. We added a new second paragraph 
in response to comments about the value of the measurement of aerobic 
capacity by oxygen uptake during maximal exercise rather than 
estimation of aerobic capacity from the level of exercise attained 
(i.e., speed and grade of treadmill exercise test) without measurement 
of oxygen consumption. In the third paragraph of the final rule (the 
second paragraph in the NPRM), we added a reference to ``silent'' 
ischemia at the end of the first sentence. We explain our reasons for 
these revisions in the public comments section of this preamble.
    Final 4.00C4, on cardiac catheterization, replaces and updates the 
rules that were in 4.00H1 of the former listings. There are two 
technical changes in the final rules from the NPRM. We deleted the 
parenthetical statement ``(by catheter)'' from the heading in 4.00C4a 
because coronary arteriography is always performed by catheter; 
therefore, the parenthetical statement was redundant and could have 
been confusing. In the heading of 4.00C4b, we changed the parenthetical 
statement to ``(by angiography).'' The language means the same thing as 
``by catheter'' but is more current.
4.00D  Treatment and Relationship to Functional Status
    We have revised the heading of final 4.00D to, ``Treatment and 
relationship to functional status,'' so that it has the same title as 
the corresponding section in part B, 104.00C. The change is 
nonsubstantive, and it is made only for consistency. This is also true 
of the deletion of the parenthetical phrase, ``medical, surgical, or 
prescribed program of progressive physical activity,'' from the 
heading; the language still appears in the body of 4.00D1 and was, 
therefore, merely redundant in the heading. As part of our response to 
several comments that led us to focus the discussions more on the 
listing level and the need to consider all relevant evidence, we have 
also incorporated into final 4.00D1 the guidance we proposed in 104.00C 
of part B.
    Final 4.00D also continues to state, as in the NPRM, that 
evaluation should usually be deferred for a period of up to 3 months to 
assess the effect of treatment. We added that the 3 months will be from 
the date of treatment to clarify when the deferral period begins. 
However, in response to public comments, we have clarified final 4.00D1 
to state that evaluation need not be deferred if the claim can be 
favorably decided based on the available evidence. We deleted the 
4.00D2 we had proposed in the NPRM in response to public comments that 
asked us to remove the absolute requirement for exercise test evidence. 
In final 4.00D2 (which was designated as 4.00D3 in the NPRM), we expand 
the criteria formerly in 4.00J for evaluation 3 months after cardiac 
surgery and provide guides for evaluation postangioplasty.
    For the above reasons, we renumbered proposed 4.00D4 as final 
4.00D3. In final 4.00D3, which addresses cardiac transplantation, we 
deleted the second sentence, which would have required us to obtain 
records of endocardial biopsies performed during the first few months 
after the transplant. We agreed with commenters who pointed out that, 
inasmuch as an individual is considered disabled under listing 4.09 for 
1 year following transplant, these early records are unnecessary. We 
have also corrected the cross-reference at the end of the section, 
which was to Sec. 416.994(c)(1)(i) in the NPRM, to 
Sec. 416.994(b)(1)(i); there is no longer a Sec. 416.994(c)(1)(i) in 
our rules.
4.00E  Clinical Syndromes
    In final 4.00E, we address the clinical syndromes formerly 
discussed in 4.00B, C, D, E and K. In 4.00E1, we have added a 
discussion on chronic heart failure with and without congestion. 
Because congestion (fluid retention) is now often controlled by potent 
diuretic medications, we eliminated the requirement for the presence of 
congestion at the time of adjudication. We now clarify that either 
there is or has been congestion, but it need not be present at the time 
of adjudication. We also moved the material on ``hypertensive vascular 
disease'' formerly in 4.00C to 4.00E2 and updated the terminology to 
the more comprehensive term, ``hypertensive cardiovascular disease.'' 
In response to a comment from a physician, we expanded the guidance in 
final 4.00E1a to indicate that chronic heart failure may be manifested 
by pulmonary congestion, systemic congestion, or both. There are no 
other changes from the NPRM in either final 4.00E1 or 4.00E2.
    Final 4.00E3, on ischemic heart disease, combines and expands the 
guides in 4.00D and E of the former listings. The terminology ``chest 
pain of cardiac origin'' has been reworded, for clarity, to 
``discomfort of myocardial ischemic origin.'' This revision also 
recognizes that the discomfort may be precipitated by emotion as well 
as exertion. Descriptions of noncoronary conditions that may cause 
ischemic discomfort and noncardiac chest conditions that may produce 
symptoms mimicking that of myocardial ischemia have been added to help 
in the evaluation of chest discomfort. The final section also indicates 
that chest discomfort of nonischemic origin may result from other 
cardiac conditions, such as pericarditis and mitral valve prolapse.
    In response to comments, we have made two changes to final 4.00E3 
from the NPRM. We added a new 4.00E3d, which consists of a single 
sentence reminding adjudicators to be alert to the possibility of so-
called silent ischemia or that the individual may be symptom-free 
because of the avoidance of activities that bring on symptoms. In final 
4.00E3f, we added ``chest wall syndrome'' to the list of noncardiac 
conditions that may produce symptoms mimicking that of myocardial 
ischemia. Aside from these changes, we made a few minor editorial 
changes (such as the insertion of commas, combining paragraphs into one 
paragraph, and the addition of the missing word ``and'' in the second 
sentence of 4.00E3f) that do not substantively affect the final rules.
    We have moved the discussion of peripheral arterial disease 
formerly in 4.00K to final 4.00E4. The final rule is the same as the 
NPRM except that, in response to a public comment, we added a phrase in 
the second paragraph clarifying that the ankle and brachial blood 
pressures are taken in the supine position. We also expanded the 
criteria for the Doppler treadmill exercise test in the third paragraph 
to permit a 10 percent grade as well as a 12 percent grade. We made a 
nonsubstantive change in the last sentence of the third paragraph to 
change the term ``contraindicated'' to ``significant risk'' to be 
consistent with terminology used in these regulations in Secs. 404.1519 
and 416.919. We explain our reasons for adopting these comments in the 
public comments section of this preamble.

4.02  Chronic Heart Failure

    This listing provides criteria for individuals with chronic heart 
failure resulting in functional restrictions that equate with NYHA 
class III or IV despite a regimen of prescribed treatment. Because 
heart enlargement is a major component of these listings, we have 
defined cardiac enlargement. We have also changed the word 
``congestive,'' which was in the title of former listing 4.02, to 
``chronic'' heart failure because the conditions covered under this 
listing are not necessarily required to be associated with congestion. 
The final rule, unlike the former rule, also requires that the 
individual be on a treatment regimen prescribed by a treating source. 
Because overt fluid retention is now often medically controlled at 
rest, even in the presence of chronic failure, we have removed the 
requirement that peripheral or pulmonary edema be present on physical 
or laboratory examination. We have also added a listing (final listing 
4.02B) that may be met with marked exercise intolerance, as 
demonstrated by inability to exercise on a treadmill at a workload 
equivalent of 5 METS or less due to symptoms of chronic heart failure, 
or the need to terminate exercise because of certain clinical findings, 
together with functional restrictions equivalent to the NYHA class III 
level.
    We have revised final listing 4.02, largely in response to public 
comments on the NPRM. In the opening paragraph of final listing 4.02, 
we have deleted the phrase, ``any specified etiology,'' in order to 
include idiopathic chronic heart failure under the listing. We also 
added a cross-reference to 4.00A for those individuals who do not have 
a regimen of prescribed treatment. As we have stated above, we removed 
the references to the NYHA functional classifications in listings 4.02A 
and B and replaced them with narrative descriptions of the criteria. We 
also reorganized the listings so that the objective medical criteria 
are presented first, followed by the functional criteria, consistent 
with other listings in appendix 1; this does not change the content of 
the listings, only their order of presentation. In listing 4.02A, we 
replaced the cross-reference to 4.00C3, which does not define 
cardiomegaly but only discusses appropriate imaging techniques, with 
more explicit examples of how cardiomegaly can be demonstrated on x-ray 
or two-dimensional echocardiography.
    In the opening paragraph of final listing 4.02B, we replaced the 
phrase, ``reduced global ejection fraction,'' with the phrase, ``left 
ventricular ejection fraction of 30 percent or less.'' The phrase 
``global ejection fraction'' is outdated medical terminology which 
means the same thing as ``left ventricular ejection fraction.'' The 
requirement of 30 percent or less removes any uncertainty about the 
criterion and is the same criterion as in listing 4.04B. In final 
listing 4.02B1, we revised the language that was in listing 4.02B2 of 
the NPRM in response to comments that demonstrated to us that it was 
unclear; the revisions are not a substantive change, but a 
clarification of the proposed rules. This includes the replacement of 
the phrase ``markedly symptomatic exercise intolerance'' with a clearer 
statement explicitly requiring the inability to exercise at the 5-MET 
level or less on an exercise test because of symptoms or specific 
clinical findings. We also added a criterion for three or more 
multiform beats in final listing 4.02B1a in response to a comment.
    Final listing 4.02C is the same as in the NPRM, except that we 
redesignated it as paragraph C of the listing. The designation as 
paragraph D in the NPRM was an error, inasmuch as there were only three 
paragraphs in the listing.

4.03  Hypertensive Cardiovascular Disease

    We have changed the title of this listing from the former, 
``Hypertensive vascular disease,'' but have made no change in the 
content. There is no change from the NPRM.

4.04  Ischemic Heart Disease

    The listing 4.04A criteria on evaluating ischemic heart disease 
when there is a recent exercise test of record have been amended to 
exclude nonspecific rhythm/conduction disturbance and expanded to 
include blood pressure response and imaging techniques in current use. 
When an exercise test has not been performed and cannot be purchased 
because performance of the test would present a significant risk to the 
individual, final listings 4.04B and C may apply. Listings 4.04B and C 
are also descriptive of individuals who may be too impaired to perform 
exercise, but who are symptomatic on normal activity. The new listing 
4.04B and C criteria require demonstration of cardiac functional loss 
resulting from heart disease.
    In response to comments, we have made a number of changes from the 
NPRM in the final listing. In the opening paragraph of the listing, we 
deleted the phrase, ``occurring repeatedly,'' because we agreed with a 
commenter who pointed out that the frequency of chest discomfort should 
not be a decisive issue inasmuch as many people structure their lives 
so as to avoid chest discomfort. The relevant issue is their functional 
limitations; that is, the level of exertion at which they would have 
chest discomfort if they were to make the attempt. We also added a 
cross-reference to 4.00A for those individuals who do not have a 
regimen of prescribed treatment.
    As in final listing 4.02, we removed the references to the NYHA 
functional classifications in listings 4.04B and C and replaced them 
with narrative descriptions of the criteria. We also reorganized these 
listings so that the objective medical criteria are presented first, 
followed by the functional criteria, consistent with the changes in 
final listing 4.02 and other listings in appendix 1.
    There are no substantive changes in final listing 4.04A from the 
NPRM. The only change is that we corrected the word ``unsloping'' in 
listing 4.04A2, to ``upsloping.'' This was only a typographical error.
    In addition to the changes already noted, we have revised final 
listing 4.04B so that its functional criterion is at the level of NYHA 
functional class III instead of the proposed class IV. In addition, we 
revised final listing 4.04C so that its objective medical criterion is 
now only angiography, instead of the proposed angiography and an 
ejection fraction. We agreed with those commenters who pointed out that 
proposed listing 4.04C described an unusual fact pattern that would 
rarely be met and that, for individuals who are at risk for exercise 
testing, the restrictions associated with NYHA functional class III 
together with documented chest discomfort and the required objective 
medical findings should be enough to establish disability at the 
listing level. There are no other changes from the NPRM in final 
listings 4.04B and C.

4.05  Recurrent Arrhythmias

    Listing 4.05 has been expanded. The new criteria specify that 
arrhythmias related to reversible causes are excluded from the listing 
and add a requirement that resting or ambulatory (Holter) 
electrocardiography demonstrating the arrhythmia be coincident with the 
occurrence of syncope or near syncope. The only change from the 
proposed rule is that, in response to a comment, we added ``near 
syncope'' to the symptoms that may satisfy the criteria of the listing.

4.06  Symptomatic Congenital Heart Disease

    We have added a new listing 4.06 on congenital heart disease 
because more individuals with this condition are living into adulthood. 
In response to comments, and for technical reasons described in the 
public comments section of this preamble, we deleted all specific 
references to functioning from the final listing. As we explain later, 
the final criteria are sufficiently severe that the functional 
limitations are implicit in the listing and need not be stated as 
separate criteria.
    Other changes from the NPRM are in final listings 4.06A2 and 4.06B. 
To make these listings consistent with the final (and proposed) 
childhood listing 104.06A1, we have added the criterion in listing 
4.06A2 of arterial O2 saturation of less than 90 percent in room 
air. In 4.06A2 and 4.06B, we have revised the arterial PO2 to 60 
Torr or less and deleted the altitude adjustments. These criteria are 
also relevant to the evaluation of cyanotic heart disease in adults. 
Finally for consistency with the language of part B and for clarity, we 
added the word ``Secondary'' to final listing 4.06E to make it the same 
as final listing 104.06D.

4.07  Valvular Heart Disease or Other Stenotic Defects, or Valvular 
Regurgitation

    The new listing provides references to other listings to be used to 
evaluate valvular heart disease or other stenotic defects or valvular 
regurgitation. It replaces former listing 4.09. We expanded the scope 
of this listing in response to a comment about proposed listing 104.06F 
in part B (final listing 104.06E) which was also relevant to the adult 
rules. We explain our reasons for this revision in the public comments 
section of this preamble.

4.08  Cardiomyopathies

    This listing provides references to other listings to be used to 
evaluate cardiomyopathies. It replaces former listing 4.09. The final 
rule is the same as the NPRM.

4.09  Cardiac Transplantation

    This is a new listing providing for a finding of disability for 1 
year following a heart transplant. We made minor editorial revisions to 
the NPRM to conform final listings 4.09 and 104.09. These revisions do 
not substantively change the final rule from the proposed rule but only 
provide consistency between part A and part B.

4.10  Aneurysm of Aorta or Major Branches

    This listing replaces former listing 4.11, and has been broadened 
to encompass all neurological complications from aneurysm, not just 
syncopal episodes. We have also added a list of common causes of 
aneurysms. Except for a minor editorial change, the listing is 
substantively the same as the proposed rule.

4.11  Chronic Venous Insufficiency

    This final listing replaces former listing 4.12 for chronic venous 
insufficiency of a lower extremity. In response to a comment, we 
revised the final rule to provide for a finding of ``meets'' when the 
individual has chronic venous insufficiency of a lower extremity with 
incompetency or obstruction of the deep venous system, resulting in 
either extensive brawny edema (final listing 4.11A), or a combination 
of superficial varicosities, stasis dermatitis, and recurrent or 
persistent ulceration which has not healed following at least 3 months 
of prescribed medical or surgical therapy (final listing 4.11B). We 
explain our reasons for this revision in the public comments section of 
this preamble.

4.12  Peripheral Arterial Disease

    This final listing replaces former listing 4.13. The final listing 
describes listing-level disability from peripheral arterial disease as 
a result of intermittent claudication or amputation at or above the 
tarsal region. The final rule is the same as the proposed rule except 
that we added the phrase ``at the ankle'' in final listing 4.12B2 
before the word ``and'' to clarify the distinction between the tests in 
listings 4.12B1 and B2. The systolic blood pressure ratio in listing 
4.12B1 is based on a comparison of blood pressures taken at the ankle 
and the arm, whereas the ratio in listing 4.12B2 is based on two ankle 
readings. This is not a change from the NPRM but a clarification of the 
meaning of a rule that has been in our listings for many years.

Revisions to Part B (Childhood Portion) of Appendix 1

104.00  Preface

    We have revised and expanded the introductory material to improve 
and facilitate its use. In response to comments, we have made a number 
of changes in this section, detailed below. Because one general comment 
expressed a concern that we had not explained our reasons for proposing 
the changes to this section of the listings, we now provide these 
explanations. In some instances, while providing these explanations in 
response to the comments, we realized that the information in 104.00 
could be more clearly and comprehensively presented and, in a few 
instances, that it could be made more consistent with statements in 
4.00 of the adult rules; we, therefore, made appropriate revisions. We 
explain all of these revisions below or in the public comments section.
104.00A  Introduction
    We have made a number of revisions from the NPRM in final 104.00A 
in response to comments and to maintain consistency with the adult 
rules. In the first paragraph, we continue to emphasize that 
cardiovascular disorders and impairments must be substantiated on the 
basis of medical evidence. However, we have revised the paragraph to 
mirror more closely the first paragraph of final 4.00A of the adult 
rules, primarily by incorporating into the first paragraph statements 
that were in subsequent paragraphs of the NPRM. The new paragraph 
incorporates the statement that was in the second sentence of the third 
paragraph of 104.00A in the NPRM, that the criteria in the listings are 
based on medical evidence consisting of signs, symptoms, and laboratory 
test abnormalities, but that it is also important to consider the 
child's response to treatment. It also includes the second sentence of 
the fourth paragraph of the NPRM, which requires a longitudinal record 
of at least 3 months; however, as in the final adult rules, we have 
added a statement in the final rules that this is unnecessary if the 
claim can be decided favorably on the basis of the existing evidence. 
In addition, because of the importance of specialization in childhood 
cardiovascular disease, we have added a statement that reasonable 
efforts should be made to ensure review by a program physician 
specializing in the evaluation of childhood cardiovascular disease or 
by a qualified pediatrician; this language is adapted from section 5036 
of Public Law 101-508, the Omnibus Budget Reconciliation Act of 1990 
(now incorporated at section 1614(a)(3)(H) of the Act), and merely 
reflects our current policy.
    Because not all forms of cardiac disease are necessarily disabling, 
the second paragraph provides examples of congenital and acquired heart 
conditions that can frequently be associated with disabling impairments 
during childhood. The second paragraph of final 104.00A is the same as 
in the NPRM, except that we corrected typographical errors. We deleted 
the third paragraph of the NPRM because we moved its sentences to other 
paragraphs: We moved the first sentence, on evidence, to the first 
paragraph of 104.00B, the section on documentation; as we have already 
stated, we moved the second sentence, describing the medical basis of 
the listings, to the first paragraph of 104.00A.
    We revised, expanded, and clarified the fourth paragraph of the 
NPRM (the third paragraph of final 104.00A). We moved the second 
sentence of the NPRM to the first paragraph. We also expanded the 
discussion in this paragraph about the adverse effects of 
cardiovascular impairments on other body systems because it could have 
been misleading. The paragraph we proposed could have suggested that 
cardiovascular impairments may affect only growth and development in 
children or cause mental retardation, when in fact they can have other 
adverse effects involving other body systems. Our intent in providing 
this paragraph was to highlight the fact that mental retardation and 
impairments of growth and development can result from cardiovascular 
impairments to ensure that these particular manifestations are not 
overlooked. However, because we are also concerned that other 
conditions should not be overlooked, we have expanded the paragraph to 
state that cardiovascular impairments, especially chronic heart failure 
and congenital heart disease, may result in impairments in other body 
systems including, but not limited to, growth, neurological, and 
mental; therefore, evaluation should include a consideration of the 
adverse effects of cardiovascular impairment in all relevant body 
systems. We then continue to highlight the importance of considering 
the effects on a child's growth and development or mental functioning, 
as described under the growth impairment (100.00), neurological 
(111.00), and mental retardation (112.05) listings.
    In the final rules, we have also added three paragraphs to make the 
preface to the childhood listings consistent with the preface to the 
adult listings, and to minimize the need to cross-refer to the adult 
rules. The fourth and fifth paragraphs of final 104.00A emphasize the 
importance of establishing a longitudinal treatment record when 
possible, or of creating a longitudinal record when there has not been 
any treatment. In the fourth paragraph, we have used much of the same 
language that is in the second paragraph of final 4.00A of the adult 
rules, but have not included discussions of factors (such as cardiac 
insult) that are generally not relevant to children. The new final 
sixth paragraph emphasizes the importance of considering equivalence 
and, if necessary when there is a claim for SSI benefits under title 
XVI, performing an individualized functional assessment whenever a 
child does not have an impairment that meets or equals a listing. This 
paragraph replaces the fifth paragraph of the NPRM, which we deleted in 
response to a public comment.
104.00B  Documentation
    Final 104.00B addresses documentation requirements, with emphasis 
on obtaining medical evidence from sources experienced in providing 
pediatric cardiac services. Reference is made to obtaining results of 
studies which may have been performed using technologies, e.g., two-
dimensional and Doppler echocardiography, exercise testing, and 
radionuclide ventriculograms, for evaluating the presence and severity 
of cardiovascular disorders. We also state explicitly that we will not 
purchase cardiac catheterization studies; this is because such 
procedures involve risk to the claimant. However, we emphasize the 
importance of requesting data and reports of cardiac catheterization 
and other tests, when they are available.
    We revised the heading of this section from the NPRM (which said, 
``Documentation and Evaluation'') so that it is the same as the 
corresponding heading in the adult rules and because this section does 
not, in fact, discuss evaluation. For consistency with the adult rules, 
we also deleted the proposed first sentence and replaced it with the 
sentence we had proposed as the first sentence of the third paragraph 
of 104.00A, slightly revised for context and to be consistent with the 
language of the adult rules. We also added a cross-reference to part A, 
4.00C1 at the end of the first paragraph to indicate that the same 
basic evidentiary requirements apply to ECG tracings for children as 
for adults. We had inadvertently omitted this cross-reference in the 
NPRM, and its addition merely makes the paragraph consistent with the 
format we follow in the second paragraph of the section.
    In response to a comment, we have also added a new second sentence 
to the second paragraph of final 104.00B, ``Documentation,'' providing 
procedural guidance on when the purchase of an ambulatory ECG may be 
appropriate. Also in response to a comment, we have added a third 
paragraph that discusses generally when exercise testing may be useful 
in evaluating children with arrhythmias or chronic heart failure. The 
new paragraph provides that exercise testing for children, while 
increasingly used, is still less frequently indicated in children than 
in adults and can rarely be performed on children who are under 6 years 
of age. It may only be purchased if the case cannot be decided based on 
the available evidence and, if it is purchased, must be performed at a 
specialty center for pediatric cardiology or other facility qualified 
to perform exercise testing for children. We also included a paragraph 
for consistency with the adult rules that indicates that purchased 
exercise tests should be performed using a generally accepted protocol 
consistent with the prevailing state of medical knowledge and clinical 
practice; that risk should be assessed for children; and that the same 
general guidelines on risk factors for adults in 4.00C2c apply to 
children.
104.00C  Treatment and Relationship to Functional Status; 104.00D 
Congenital Heart Disease
    Final 104.00 C and D provide guidelines to be used in the 
evaluation of infants and children with congenital heart disease under 
final listing 104.06H, as well as general guidance on the relationship 
of treatment to functional status. Final listing 104.06H was proposed 
listing 104.06A. We explain our reasons for moving it to the end of the 
listing under the summary of provisions to listing 104.06, below. For 
the same reasons given under the summary of provisions for 4.00D of 
part A, we revised the heading of final 104.00C from that in the NPRM 
so that it is identical to 4.00D of the adult rules. We also 
incorporated the provisions of 4.00D1 into the first paragraph of final 
104.00C to make clear that this important information about the need to 
establish a longitudinal record also applies to children, unless a 
favorable decision is possible based on the available evidence. Because 
we added the exception for decisions favorable to the claimant, we 
deleted the word, ``however,'' from the beginning of the first sentence 
of the second paragraph for context.
    In response to comments we have received, we also expanded the 
discussions in final 104.00C and D to clarify their meaning. In the 
second paragraph of final 104.00C, we state our original intent that 
``the most life-threatening forms of congenital heart disease and 
cardiac impairment'' are exemplified by the conditions named in 
104.00D; therefore, these conditions, or conditions of equivalent 
severity, are the kinds of conditions contemplated by final listing 
104.06H. We also make clear that these are conditions for which life-
saving surgery must be performed within the first year of life. We then 
provide more detail about why these conditions are disabling under the 
listing. This is because they are so severe that, even with surgery, we 
would expect them to continue to be disabling for a period of at least 
12 months, as required by the Act, because of residual impairment after 
surgery, the recovery time after surgery, or a combination of both 
factors. We do not intend any of these revisions to be a substantive 
change from the rules we proposed.
    In the third paragraph of final 104.00C, we replaced the ambiguous 
references to the ``specified period'' of time, with the actual time 
periods set forth in final listings 104.06H and 104.09. We also revised 
the last sentence of the paragraph because it contained inaccurate and 
incorrect information. We revised the general statement about 
continuing disability evaluation because it was an inaccurate statement 
of our medical improvement review standard in Sec. 416.994a, which 
requires a finding of medical improvement based on symptoms, signs, and 
laboratory findings, and a finding whether any medical improvement is 
``related to the ability to work'' before consideration of whether the 
residual impairment is still disabling. The revised language now more 
closely follows the statement in final 4.00D4 of the adult rules that, 
``continuing disability evaluation will be based upon residual 
impairment as shown by symptoms, signs, and laboratory findings,'' and 
then cross-refers to the appropriate rules for continuing disability 
review.
    We also revised the cross-references at the end of 104.00C because 
they were incorrect. The appropriate rule for evaluating continuing 
disability in children will usually be Sec. 416.994a; moreover, there 
is no longer a Sec. 416.994(c)(1)(i) in our current rules. We have, 
therefore, revised the cross-references to refer more generally to the 
three rules that might apply: Secs. 416.994a, 404.1594, and 416.994. 
The last two rules usually apply to adults, but there will be cases in 
which our continuing disability review occurs when the person is over 
age 18. Also, even though these listings apply to all people under age 
18, the continuing disability review process in Sec. 404.1594 applies 
to children seeking child's insurance benefits based on disability 
under title II.
    We deleted the first paragraph of proposed 104.00D because it was 
redundant of the discussions in 104.00C. In the first and second 
paragraphs of final 104.00D (which were the second and third paragraphs 
in the NPRM), we made minor editorial changes to clarify that the named 
conditions are only examples of the kinds of conditions discussed in 
final 104.00C and final listing 104.06H. We also added the modifier 
``listing-level'' before the word ``impairment'' in the opening clause 
of the first paragraph. Clearly, there are less severe impairments than 
the life-threatening conditions named in 104.00D which would cause 
impairment; therefore, the addition of the phrase only corrects an 
ambiguous use of the word ``impairment.'' For the same reason, we added 
the same modifier before the word ``impairment'' in the second and 
third sentences of the third paragraph of final 104.00D (the fifth 
paragraph in the NPRM). We also broadened the scope of the final second 
paragraph of the section to encompass up-to-date treatment modalities. 
We now indicate that the conditions named may require multiple surgical 
interventions (as in the NPRM), but that they may also involve only one 
surgery but require other significant treatments after surgery, such as 
multiple cardiac catheterization procedures, which are now being used 
more frequently in infants.
    We deleted the proposed fourth paragraph, regarding patent ductus 
arteriosus, in response to a comment which pointed out that the 
condition is not of the level of severity contemplated by final listing 
104.06H; this comment also led us to clarify final 104.00C and D to 
indicate more clearly the kinds of conditions that are contemplated. 
Finally, in the third sentence of the third paragraph of the final rule 
(the fifth paragraph of the NPRM) we added a parenthetical statement 
clarifying that the guidance about lower levels of pulmonary artery 
pressure ``in the absence of such a defect'' refers to primary 
pulmonary hypertension, or to some connective tissue disorders with 
cardiopulmonary involvement and pulmonary vascular destruction, such as 
systemic lupus erythematosus.
104.00E  Chronic Heart Failure
    Final 104.00E, ``Chronic heart failure,'' emphasizes the clinical 
and laboratory findings that document the existence of chronic heart 
failure, and describes symptoms of the impairment in infants and 
children. The use of imaging techniques is cited in demonstrating the 
presence of cardiomegaly or ventricular dysfunction.
    We made a number of changes and additions in response to public 
comments. We expanded the first paragraph of the section to restore 
mention of some of the symptoms from former listing 104.02 and to 
include some of the guidance we had originally proposed for inclusion 
in listing 104.02. In the second paragraph, we also deleted our 
reference to radionuclide studies (formerly in the last sentence of the 
proposed rule), which are rarely performed on children, and replaced 
the examples with references to two-dimensional or Doppler 
echocardiography, two common imaging techniques, and added a reference 
to standards for measuring cardiomegaly using echocardiography. We also 
restored the rule that was in former 104.00C regarding the use of 
roentgenography (x-rays) to determine cardiomegaly, which we had 
proposed to delete because it is now an uncommon practice for this 
purpose and below the modern standard of care for children with chronic 
heart failure. For this reason, we also now require that findings of 
cardiomegaly on chest x-ray also be accompanied by other findings 
demonstrating chronic heart failure; we have also provided a reference 
for the evaluation of enlargement using appropriate imaging techniques 
and have made minor revisions to update the rules. We explain our 
reasons for these changes in more detail in the public comments section 
of this preamble.
    In addition, we made two nonsubstantive, editorial revisions in the 
section for clarity. In the first sentence of the section, we added the 
phrase ``in infants and children'' to make clear that the guidance in 
that sentence applies to all children, not just infants. Inasmuch as 
the other two sentences in the paragraph are about infants, we were 
concerned that people understand that we had not overlooked older 
children. In the first sentence of the second paragraph (which was the 
last sentence of the proposed section in the NPRM), we deleted the 
phrase, ``can be,'' because it was ambiguous. Our intent was to give 
examples of kinds of imaging techniques that could be used to 
demonstrate manifestations of chronic heart failure, not to state that 
imaging techniques were only one alternative for demonstrating the 
manifestations.
104.00F  Valvular Heart Disease
    Final 104.00F, ``Valvular heart disease,'' describes the 
circumstances under which a listing-level impairment is likely to be 
present and the necessary documentation requirements. In the first 
sentence, we added the phrase ``or other source of record,'' after 
``treating source.'' This is a technical correction that is required 
because of final rules we published after we published this NPRM 
(``Standards for Consultative Examinations and Existing Medical 
Evidence,'' 56 FR 36932, August 1, 1991). Under Secs. 404.1502 and 
416.902, the term ``source of record'' is a more inclusive term that 
includes both treating sources and other sources, such as hospitals, 
clinics, or other medical sources that have provided an individual with 
treatment or evaluation. If the results of angiography are available 
from such sources, we will, of course, try to get them.
    As in final 104.00C and D, we added the phrase ``listing-level'' 
before the word ``impairment'' in the second sentence of the section. 
We also added ``arrhythmias'' to the list of possible outcomes of 
valvular heart disease that could result in listing-level impairments. 
This, too, is a technical correction, inasmuch as arrhythmias are 
included in the listing that corresponds to this section, final listing 
104.07. For clarity, we deleted the qualifying reference to 
``prepubertal children, age 16 years or less'' that was at the end of 
the proposed paragraph. In fact, this guidance applies to all children, 
up to age 18. Finally, for consistency with the adult rules, we have 
added the same guidance that appears in the first sentence of final 
4.00D2, that the usual time for adequate assessment of the results of 
treatment after valvular surgery is 3 months.
104.00G  Rheumatic Heart Disease
    This section renumbers and retitles former 104.00E on rheumatic 
fever. We made two changes from the NPRM language. We deleted the 
phrase ``as used in this section,'' because it is superfluous; the 
deletion does not change the meaning of the sentence at all, and is, 
therefore, only editorial. We also deleted the specific reference to 
the 1965 Jones criteria for guidance in the diagnosis of rheumatic 
fever. This is because we have recently become aware that a new 
revision is forthcoming and we want the listings to be as up-to-date as 
possible. We replaced the reference with a general requirement to use 
the ``current'' Jones criteria.

104.01  Category of Impairments, Cardiovascular

    The revisions to the part B cardiovascular listings maintain 
structural and content comparability with part A to the extent 
applicable. As in the sections of 104.00, there were a few instances in 
which we were able to incorporate language from the adult rules into 
the listings language we proposed in the NPRM or to make the same or 
similar revisions in both parts for even greater consistency between 
parts A and B.

104.02  Chronic Heart Failure

    As in the adult rules, we have removed the term ``congestive'' from 
the heading of listing 104.02 and replaced it with ``chronic.'' Because 
of pharmacological and clinical advances in the control of overt 
pulmonary and systemic congestion (fluid retention), heart failure can 
be present despite the absence of congestive signs.
    The criteria under final listing 104.02 stress the importance of 
demonstrating functional impairment. However, we also require the 
presence of either cardiomegaly or ventricular dysfunction, as 
described in 104.00E, to demonstrate the presence of chronic heart 
failure.
    We have revised listing 104.02 of the proposed rules in response to 
public comments. Final listing 104.02A is now a criterion for 
persistent tachycardia at rest, and final listing 104.02B includes 
persistent tachypnea at rest or markedly decreased exercise tolerance, 
as defined in 104.00E of the preface; in response to the comments, we 
also included the tables for resting tachycardia and tachypnea from the 
prior rules. Final listing 104.02C now lists recurrent arrhythmias 
under listing 104.02 in lieu of the cross-reference to listing 104.02 
we had proposed to provide in the listing for arrhythmias, 104.05. The 
addition of these three rules also required us to redesignate the 
paragraphs we had proposed in the NPRM.
    In addition, in response to comments which demonstrated that the 
rules could be made clearer for lay readers, we moved some of the 
requirements of proposed listing 104.02A, which discussed 
symptomatology and severity, into the prefatory discussion in 104.00E 
and expanded that discussion. We explain all of these changes and our 
reasons for them in the public comments section of the preamble.
    Final listing 104.02D (proposed listings 104.02B and 104.02C) 
provides for the evaluation of the infant and young child whose major 
manifestation of chronic heart failure is a growth disturbance. In a 
technical correction, we revised final listings 104.02D1 and D2 because 
proposed listing 104.02B was unclear and illustrated a level of 
severity that was above listing-level. We revised final listings 
104.02D2 and D3 to provide criteria for the evaluation of involuntary 
weight loss or failure to gain weight at an appropriate rate. In this 
way, we not only include children who have stopped gaining weight or 
lose weight, but also children who do not gain enough weight. We also 
explicitly provide for the possibility that a child will actually lose 
weight. Final listing 104.02D3 (proposed listing 104.02C) provides a 
reference to the listing on growth impairment. It has been included to 
permit evaluation of height and growth failure under the cardiovascular 
listing. In a technical clarification, we changed the reference in 
final listing 104.02D3 from a specific reference to listings 100.02A 
and B, to a general reference to the listings in section 100.00. In 
this way, listing 104.02D3 will remain current when we revise the 
growth impairment listings in the future.

104.03  Hypertensive Cardiovascular Disease

    The final listing is essentially the same as the former listing 
except that we have updated final Table III, ``Elevated Blood 
Pressure,'' using values currently recommended by the American Academy 
of Pediatrics (see the definition of ``Significant Hypertension'' in 
Table 5 of the ``Guidelines of the American Academy of Pediatrics; 
Report of the 2d Task Force on Blood Pressure Control in Children--
1987,'' ``Pediatrics,'' 1987; 79:1-25). The table was unnumbered in the 
NPRM because it was the only table in the proposed rules. Now that we 
have restored the tables for tachycardia at rest and tachypnea at rest 
to final listing 104.02, we have restored the designation ``Table III'' 
in the final rules. In response to a comment, we have also added a 
statement in the opening paragraph of the listing clarifying that the 
values in the table are equal to or greater than the 95th percentile 
for age.

104.05  Recurrent Arrhythmias

    We have dropped the criteria in former listing 104.05C for exercise 
intolerance because, if present, such intolerance would most likely be 
manifested in association with chronic heart failure as described under 
final listing 104.02. In response to comments, we have revised the 
final rule to state more clearly our original intent and to be more 
consistent with final listing 4.05, the corresponding adult rule. Final 
listing 104.05 is now a single paragraph which provides that recurrent 
arrhythmias resulting in uncontrolled repeated episodes of cardiac 
syncope or near syncope and arrhythmia despite prescribed treatment, 
including electronic pacemaker, and documented by resting or ambulatory 
(Holter) electrocardiography coincident with the occurrence of syncope 
or near syncope will meet the criteria of the listing. As in the adult 
rule, we now include near syncope among the symptoms that will satisfy 
the requirements of the listing.
    As we explain in more detail in the public comments section of this 
preamble, we removed the proposed criterion in listing 104.05B for 
chronic heart failure that meets the requirements of listing 104.02 and 
instead added a criterion of arrhythmia in final listing 104.02C by 
which that listing can be met. We also moved the proposed criterion in 
listing 104.05C, for electronic pacemaker therapy, into the text of the 
rule; electronic pacemaker therapy is merely a kind of treatment and, 
therefore, is now included as an example of treatment that must be 
ineffectual instead of as a separate criterion. Our intent in making 
this revision from the NPRM is not to raise the severity of the listing 
but to clarify our original meaning and to ensure that the rule is 
medically correct.

104.06  Congenital Heart Disease

    Final listing 104.06 (former listing 104.04) is renamed 
``Congenital heart disease'' because it is to be used for both cyanotic 
and acyanotic congenital conditions. The term ``palliative'' as applied 
to cardiac impairment or surgical procedures is no longer particularly 
meaningful in evaluating impairment severity. For this reason, the 
former listing 104.04A criterion, which referred to palliative surgery, 
has been omitted from the final listing.
    In a nonsubstantive, editorial change from the NPRM, we reorganized 
the paragraphs of final listing 104.06 to move proposed listing 104.06A 
to the end of the listing, as final listing 104.06H. We did this 
because proposed listing 104.06A specifies that it is confined to 
infants under age 1, but the subsequent listings in the proposed rules 
did not state that they apply to children of all ages. Therefore, if 
the listing for infants under age 1 came first, it would not be clear 
that the subsequent listings (i.e., proposed listings 104.06B through 
H) were applicable to all children, regardless of their age. By 
redesignating proposed listings 104.06B through H as final listings 
104.06A through G in the final rules and placing the infant listing 
last, there will be no possibility of ambiguity or misunderstanding 
about the applicability of the first seven criteria in the listing.
    The criteria under final listing 104.06A (proposed listing 104.06B) 
represent criteria that were previously included under listings 104.04B 
and C. Cardiac syncope, formerly in listing 104.07, is in final 
listings 104.06A3 and 104.05. In keeping with our revisions to ensure 
comparability between the adult and childhood listings, we revised the 
NPRM nonsubstantively, to reflect more closely listing 4.06A in part A. 
We now clarify that final listing 104.06A is for the evaluation of 
cyanotic heart disease. We also reversed the order of subparagraphs 1 
and 2 so that they are in the same order as the corresponding rules in 
adult listing 4.06A. Because we now include reference to cyanosis in 
the opening statement of final listing 104.06A, and because this 
listing includes both cyanotic and noncyanotic heart disease, we 
replaced the word ``cyanosis'' with the more medically accurate 
``hypoxemia'' in final listing 104.06A4. Also, in response to a comment 
about our omission of the former listing 104.08 criterion for 
hemoptysis in proposed listing 104.06D, we have deleted the criterion 
for recurrent hemoptysis in final listing 104.06A3. We explain our 
reasons for this deletion in the public comments section of this 
preamble.
    Final listings 104.06B, C, D, and E are included to maintain 
content comparability with part A, listing 4.06. In final listing 
104.06B (proposed listing 104.06C), we added the phrase, ``with 
evidence of ventricular dysfunction,'' as in the corresponding adult 
rule, final listing 4.06C. The former listing for hemoptysis (104.08) 
has been deleted for reasons we explain in the public comments section 
of this preamble; this very severe manifestation will be evaluated 
under final listing 104.06D or other appropriate listings. We also 
explain why we no longer include the finding of bronchial collaterals 
in the listing in the public comments section. In response to a 
comment, we revised final listing 104.06E (proposed listing 104.06F) to 
include other stenotic defects as well as valvular stenosis and to make 
the listing consistent with the corresponding adult listing, final 
listing 4.07. Final listing 104.06F is a new criterion that has been 
included to evaluate manifestations of symptomatic acyanotic congenital 
heart disease with ventricular dysfunction; we did not make any changes 
from the NPRM. In final listing 104.06G, we revised the reference to 
growth impairment in listing 100.02 to a more general reference to the 
listings in 100.00 in anticipation of future revisions to those 
listings.
    The revised surgical criterion in final listing 104.06H permits 
more appropriate evaluation of life-threatening cardiac impairment. In 
response to a public comment, we have revised the criteria in this 
section to better express our original intent and to make the listing 
more equitable. The final rule provides that a child who meets this 
listing will be considered under a disability for 12 months after 
surgery; children with impairments that were expected to result in 
death before age 1, who have not had surgery, and who have, in fact, 
survived to age 1 will be considered for review at age 1. The rule is 
confined to infants under 12 months of age because the majority of 
congenital conditions described are known to result in life-threatening 
cardiac impairment or death within that time period. Twelve months is 
an appropriate period of time at which to consider continuing 
disability review because it allows most infants the necessary 
postoperative time for stabilization and resumption of expected growth 
and development patterns for age. Because of this response to the 
comment, we also made a similar change in listing 104.09, ``Cardiac 
transplantation.'' We provide a more in-depth explanation of our 
reasons for these revisions of the proposed rules in the public 
comments section of this preamble.

104.07  Valvular Heart Disease or Other Stenotic Defects, or Valvular 
Regurgitation

    This new listing provides cross-reference listings for the 
evaluation of valvular or other stenotic defects, or valvular 
regurgitation, and a criterion for critical aortic stenosis in infants. 
The criteria of former listing 104.07 have been moved to final listings 
104.05 and 104.06A3. In response to a comment about final listing 
104.06E, we expanded the scope of the proposed listing by making it 
consistent with the revisions to final listings 4.06 and 104.06E.

104.08  Cardiomyopathies

    This new category provides criteria for use of the results of 
imaging techniques and provides references to other relevant listings 
for evaluating function in cardiomyopathy. The final listing is 
unchanged from the NPRM.

104.09  Cardiac Transplantation

    Children who have had heart transplants will be considered disabled 
for 1 year after surgery. We deleted the proposed criterion ``or until 
age 24 months, whichever is the later event,'' for the same reason we 
revised final 104.06H; i.e., because of the comment which pointed out 
that the proposed rule was inequitable. We explain our reasons for 
making this revision in more detail in the public comments section of 
this preamble. We also made slight revisions to this section in order 
to parallel the adult rules. These revisions are only editorial.

104.13  Chronic Rheumatic Fever or Rheumatic Heart Disease

    This listing was former listing 104.09. We have added a provision 
for finding a child disabled for 18 months after established onset of 
the impairment when the specified medical findings are present.
    We made two revisions to proposed listing 104.13A. In response to a 
comment, we restored the example of ECG findings to the listing. We 
also added a cross-reference to the example of cardiomegaly in 104.00E. 
The former listing had included such a cross-reference, but it was to 
the section of the preface that discussed cardiomegaly on x-ray. The 
NPRM, therefore, did not include the cross-reference because we had 
proposed to delete that section of the preface. Because we have 
restored the guidance in 104.00E, we also restored the cross-reference.

104.14  Hyperlipidemia

    We have added this new listing because there are forms of this 
disorder that can result in major organ complications and cause 
disabling impairment or early death. The final rule is the same as the 
proposed rule.

104.15  Kawasaki Syndrome

    In response to a comment from a major medical organization, we have 
added a listing for Kawasaki syndrome. The final listing includes 
major, listing-level cardiovascular manifestations of the disorder.

Public Comments

    Following the publication of the NPRM in the Federal Register, we 
received 24 letters containing comments pertaining to the changes we 
proposed. Ten of these letters came from individuals and Government 
agencies, both State and Federal, whose responsibilities require them 
to make disability determinations involving cardiovascular impairments 
under titles II and XVI of the Act. Eight of the letters were from 
legal services organizations or advocacy groups, State and city legal 
departments, and individual lawyers. The remaining six letters came 
from medical associations, a hospital, and other medical professionals.
    We carefully considered all of the comments and have adopted many 
of the recommendations made by the commenters. These changes are 
identified in the following discussion of issues that were raised in 
the comments.
    A number of the comments were quite long and detailed. Of 
necessity, therefore, we have condensed, summarized, or paraphrased 
them. However, we have tried to respond adequately to the substantive 
comments we received.
    For ease of reference, we have organized the comments and responses 
as follows. We first address general comments, i.e., comments that are 
either about the rules as a whole or that apply to more than one 
section of the rules. We then address the remaining comments, which 
pertain to specific sections of the rules. The section references in 
the headings below refer to the final rules. In those instances in 
which we changed the section numbers or headings in the final rules, we 
provide both the NPRM and final references in the text of the comment 
and response.

General Comments

3-Month Guideline
    Comment: We received seven comments about the 3-month guidelines in 
proposed 4.00A, 4.00C2d, and 4.00D. Four of the commenters thought that 
this was a ``blanket rule'' which would require delay in the 
adjudication of a class of claims or unnecessary delay of many claims. 
Three of the commenters pointed out that the deferral would have no 
practical effect in many SSI claims because many SSI claimants will 
receive little or no treatment; therefore, there will be little or no 
additional evidence even if we were to wait 3 months. One commenter 
said that delay in some cases would be unwarranted because 3 months 
will have already elapsed before the date of adjudication. The same 
commenter pointed out that deferral would not be necessary in some of 
the most serious cases because it will be clear that the claimant is 
disabled. This commenter suggested that we revise the rules to provide 
that deferral of adjudication is never appropriate without a specific 
reason particular to the individual's case. One medical organization 
supported this guideline, stating that it was welcome to ``those of us 
in the field of cardiac rehabilitation.''
    Response: We have not deleted the 3-month guidelines, but we have 
clarified the rules in response to the comments. We never intended to 
create a ``class'' of claimants who would be treated differently from 
other claimants. The requirement for a longitudinal record in 
cardiovascular cases is consistent with our rules for evaluating all 
types of impairments, not only cardiovascular impairments. We need a 
longitudinal record to establish the severity and duration of most 
impairments, especially those that may be amenable to treatment.
    We provided a specific time guideline in the cardiovascular rules 
because many individuals with cardiovascular impairments have sudden 
onset of their impairments, have undergone surgery, or have impairments 
that will improve with treatment; in all three instances, they may 
recover in a relatively short time. We, therefore, must have some 
indication that the individual will be disabled for 12 months, and we 
believe that a 3-month recovery period is the minimum amount of time 
needed to demonstrate whether there will be a significant recovery. We 
also did not intend to have the 3-month guideline be an invariable rule 
and accordingly we used qualifying phrases, such as ``usually'' and 
``whenever there is such evidence,'' in the proposed rules.
    However, in response to the comments we have clarified 4.00A and 
104.00A by stating specifically that the reason we require 3 months of 
evidence is to establish a longitudinal picture of the individual's 
impairment(s), in terms of medical severity, functioning, and 
symptomatology. We also explain that, for the same reasons, a 
longitudinal record is important even when the individual has not 
received ongoing treatment. We have also added explicit statements to 
final 4.00A, 4.00C, and 4.00D in part A, and 104.00A and 104.00C in 
part B, that it is not necessary to defer issuing a determination or 
decision on a claim when the available evidence establishes that the 
claimant is disabled.
    The fact that an individual may have no treating source does not 
mean that we cannot establish a longitudinal clinical record. If 
necessary, we may purchase a consultative examination for comparison 
with earlier evidence. However, in response to the comments, we have 
added a new third paragraph in final 4.00A to address this concern.
    Finally, the commenter who noted that many claimants will have 
already established a 3-month history was correct. For exactly that 
reason, we do not believe that the 3-month guideline is especially 
onerous or that it will result in many delays in adjudication. The 
final rules do not say (nor did the NPRM) that adjudicators should 
defer claims for an additional 3 months, only that they should 
establish a 3-month record. Indeed, we expect that even when deferral 
is necessary, we will rarely have to defer the claim for a full 3 
months because some time will necessarily have passed by the time of 
adjudication.
New York Heart Association Functional Classification
    Comment: We received several comments about our use of the New York 
Heart Association (NYHA) functional criteria in proposed adult listings 
4.02, 4.04, and 4.06. One commenter said that the criteria are vague 
and believed that we would misapply them to the detriment of claimants. 
Another commenter said that, in the majority of cases, treating sources 
do not use the NYHA classifications. Another commenter was concerned 
about how to use the classifications when the individual's impairment 
fluctuates, saying that it is unrealistic to presume that some patients 
will remain in one classification. Two commenters were concerned about 
how we would develop evidence and weigh treating source opinions about 
the classification.
    One commenter said that the criteria were in a publication that has 
long been out-of-print. This commenter, and several others, thought 
that, at a minimum, we should include the definitions of the NYHA 
classification levels in the listings. One commenter, however, pointed 
out that if the NYHA alters its classification standards in the future, 
it may become necessary for us to revise the listings that employ those 
standards.
    Finally, two commenters were concerned that the proposed rules 
failed to include the NYHA ``therapeutic classifications.'' One of 
these commenters said that the final regulations must at least 
recognize the concept behind the therapeutic classifications, which is 
that some individuals must restrict their activities for reasons of 
medical safety, even though they are technically capable of performing 
more exercise than is safe or medically appropriate.
    Response: We adopted the comments by removing the references to the 
NYHA functional classifications from final listings 4.02 and 4.04 and 
replacing them with explicit, descriptive functional criteria based on 
the NYHA criteria, and by removing all functional criteria from final 
listing 4.06. In this way, we have avoided the pitfalls pointed out by 
the commenters, although we do not believe that our proposed use of the 
classifications was as problematic as the commenters did.
    Cardiologists have used the NYHA functional classifications for 
many years, and the criteria are well understood by people in the 
profession; this undoubtedly accounts for the fact that we did not 
receive any comments from physicians or medical organizations about our 
proposal to use the criteria. Nevertheless, we agree with the 
commenters that there are a number of real problems that could arise in 
an adjudicatory context and, on balance, believe that the most 
straightforward solution is to simply state exactly what we require in 
the listings themselves.
    As we have explained in the first section of this preamble, the 
deletion of the references to the NYHA functional criteria also 
necessitated a reorganization of the rules in final listings 4.02 and 
4.04. This is not a substantive change; we merely reorganized the 
criteria of the proposed rules to place the medical criteria describing 
the listed medically determinable impairments first--as in all other 
listings--followed by the explicit functional criteria. This 
organization is also consistent with the organization of the mental 
listings in 12.00 and 112.00, as well as several other listings that 
include disabling functional consequences among their criteria.
    We decided to delete the proposed functional criteria from final 
listing 4.06 for several reasons, chiefly because they were 
unnecessary. Individuals whose impairments demonstrate the findings in 
final listings 4.06A, 4.06B, and 4.06E have impairments that will cause 
at least NYHA functional class III limitations. Individuals whose 
impairments satisfy the criteria in the reference listings, final 
listings 4.06C and 4.06D, already have impairments that meet the 
requirements of other listings. Therefore, the proposed functional 
requirement for listing 4.06 was at best redundant. Also, the proposed 
requirement for functional class III ``or class IV'' was unnecessary 
for logical reasons: Since functional class III would have been 
sufficient to establish disability under proposed listing 4.06, a 
person who had functional limitations equivalent to class IV (i.e., 
symptoms at rest) would have had an impairment that was more severe 
than was necessary to meet the requirements of this listing. Finally, 
some of the provisions of final listing 4.06 are the same as provisions 
in final childhood listing 104.06, which does not include functional 
criteria; deletion of the functional criteria in the adult listing 
makes the corresponding part A and part B listings consistent with each 
other.
    We did not include the NYHA therapeutic classifications in the 
listings, or explicit criteria based on the therapeutic 
classifications, because we believe that such considerations are 
generally more suitable to the assessment of residual functional 
capacity. However, we want to assure the commenters that the principle 
illustrated by the therapeutic classifications is a basic principle in 
all of our disability evaluations. It has long been our policy that 
valid medical reasons for limiting an individual's level of exertion 
(or any functioning, including mental functioning) can be sufficient to 
establish that the individual is in fact limited in that capacity; the 
individual need not demonstrate this limitation by putting himself or 
herself at risk in order to demonstrate that there is a limitation.
    As to the comment about individuals whose functional status changes 
and who, thus, might properly be classified in more than one of the 
NYHA classes at different times, it is our policy that an individual 
need not be continuously limited to the degree specified in order to be 
found disabled. This is one of the reasons we repeatedly stress the 
need for longitudinal evidence, which will permit our adjudicators to 
draw conclusions about how the individual generally functions, over 
time. Practically speaking, the fact that an individual's ability to 
function may briefly improve, only to worsen again, is immaterial to 
the determination that the individual is unable to work.
    Although we have deleted all references to the NYHA classifications 
as listings criteria, we have retained one reference in the preface to 
the listings. This is in final 4.00C2c of the preface, in which we list 
NYHA class IV heart failure as a risk factor militating against the 
purchase of an exercise test. We believe that in this narrow context 
the use of the term is sufficiently clear.
Exercise Testing
    Comment: Ten commenters strongly opposed our proposals in the NPRM 
for using exercise testing. In particular, the commenters opposed 
proposed 4.00D2, which would have required treadmill exercise testing 
in all cases in which such testing was not medically contraindicated, 
and proposed in 4.00C2a, which stated that valid exercise tests, 
although not the exclusive means for assessing the severity of heart 
disease, should be the ``primary basis'' for evaluating functional 
capacity under the listing. One commenter said that the proposed rules 
would result in exercise testing that was unnecessary, expensive, and 
dangerous. One commenter said that our statement in proposed 4.00C2a 
that the utility of exercise testing is ``well recognized'' would 
further improperly underscore the importance of exercise testing.
    Response: We adopted the comments asking us to delete the reference 
to the primacy of exercise testing in 4.00C2a and all of proposed 
4.00D2. As we have explained above in the explanation of the final 
rules, final 4.00C2a is now confined to a discussion of when to 
purchase exercise testing, and limits the circumstances in which 
purchase will be appropriate.
    The commenters offered many arguments for removing the above-
mentioned references in proposed 4.00C2a and 4.00D2. Because we have 
adopted the comments, we have not summarized all of the comments and 
arguments here. Most of those who commented on this aspect of the final 
rule pointed out that our statement in proposed 4.00C2c could have been 
misunderstood, as indeed it was by most of these commenters. The 
commenters assumed that our statement that treadmill exercise testing 
would be the ``primary basis for evaluating functional capacity under 
this listing'' meant that treadmill testing would be the primary basis 
for assessing functioning at all steps of the sequential evaluation 
process. The plain language of the proposed rule, of course, did not 
say this, but was confined to a discussion of whether a claim could be 
allowed at the listings step. Nevertheless, the comments do demonstrate 
that there were misunderstandings, especially because we also proposed 
to require the purchase of exercise testing in most cases.
    We were also persuaded by the arguments that there will be many 
cases in which it will be evident that the claimant is disabled, 
obviating the need for--and expense of--exercise testing. We also 
agreed with the commenter who pointed out that, even though we require 
consideration of exercise testing in the context of the entire record, 
the use of the word ``primary'' in the proposed rules could have been 
interpreted as an instruction to our adjudicators to automatically give 
more weight to the results of such testing than to any other evidence; 
such an instruction would be contrary to our policy.
    In related comments, several commenters suggested that we emphasize 
in the final rules that exercise testing is merely one tool that 
physicians use to evaluate the severity of cardiac disease, and that 
the proper evaluation of a claim for Social Security or SSI benefits 
based on disability requires consideration of the test results in the 
context of all the relevant evidence. As we have explained above in the 
explanation of the final rules, we have adopted the comment by adding a 
statement to this effect in 4.00A and 4.00C2e(1) of the final rules. 
Final 4.00A emphasizes the need to consider all of the evidence; the 
fact that no claim may be denied solely because the claimant's 
impairment(s) does not meet or equal in severity the requirements of a 
listing; and the need to provide an individualized assessment in every 
case. Final 4.00C2e(1) emphasizes the need to consider exercise test 
results in the context of all of the relevant evidence. We did not add 
similar statements in 4.00C2a because we revised it to change its focus 
to the purchase of exercise testing, and there was no longer a context 
for such a discussion in that section.
    We did not agree with the commenter who said that our purchase of 
exercise tests would be dangerous. We have many safeguards to ensure 
proper review by an expert physician before we will purchase such 
testing. We have also provided a detailed methodology for performing 
the tests safely and in approved facilities, including a requirement 
for supervision of the test by a physician.
    We also did not agree with the comment about our statement of the 
utility of exercise testing in the first sentence of proposed 4.00C2a; 
although, we did revise the statement. We agreed with those commenters 
who referred to the information supplied by the American Medical 
Association and the American College of Cardiology in the joint amicus 
curiae brief these organizations filed with the U.S. Court of Appeals 
in New York in the case of the State of New York v. Sullivan, 906 F.2d 
910 (2d Cir. 1990), that treadmill exercise testing does not assess all 
types of functioning or functioning in different environments. We, 
therefore, revised 4.00C2a to state more accurately that it is well 
recognized by medical experts that exercise testing is the best tool 
currently available for estimating aerobic capacity.
    Comment: Four commenters, quoting or paraphrasing the district 
court's decision in 1989 in the State of New York case, stated that the 
exercise test is not a valuable tool for assessing disability because 
the exercise test results in a misdiagnosis of ischemic heart disease 
more than one-third of the time. They also cited the part of the 
court's decision that noted that, in certain circumstances, other 
widely used procedures (e.g., the exercise thallium test, the 
equilibrium radionuclide angiogram, and angiography) are more reliable 
in measuring the severity of ischemic heart disease.
    Three of these commenters, in identical or similar language, also 
asserted that the proposed listings authorized adjudicators to deny 
benefits based on the results of suboptimal exercise tests. Another 
commenter stated that many exercise tests purchased on a consultative 
basis are terminated due to detoning or hyperventilation before 
cardiovascular challenge can occur, thus rendering the exercise test 
useless for evaluation of cardiac functioning.
    Response: We do not use treadmill exercise tests to diagnose the 
existence of impairments; we use them to estimate the aerobic capacity 
of individuals and, hence, as a test of a certain kind of functional 
capacity. Indeed, in the preamble in the NPRM, we stated that we 
proposed to remove the requirement for a targeted heart rate because 
``the proposed listings do not use the exercise test for diagnosis but 
rather for functional evaluation.'' We made this statement in part 
because we agree with the commenters that the failure to demonstrate 
ischemia on an ECG does not by itself establish that the individual's 
heart is normal; however, it is evidence of the exercise test level 
that can be safely performed without triggering ischemic ST changes 
and, hence, is some evidence of aerobic capacity. Even when an exercise 
test is negative, it still provides some information about the 
individual's ability to exercise to the level achieved on the test.
    Although we agree with the commenters' opinion that the exercise 
thallium test is the best method for diagnosing the existence of 
ischemic heart disease, we repeat that the issue in these listings is 
not one of diagnosis but of functioning. We also agree that the other 
tests may be useful for evaluating the severity of ischemic heart 
disease in some circumstances; however, they are not generally more 
reliable than the exercise test for this purpose. For this reason, we 
do not agree that they belong in our listings as alternative criteria. 
The listings are only examples. We do not attempt to state every 
possible fact pattern in the listings; that is the province of our 
findings that claimants may have an impairment that is equal in 
severity to a listed impairment.
    In response to the three commenters who asserted that the proposed 
rules would permit adjudicators to deny benefits based on suboptimal 
exercise tests, we have stated above that the failure to meet or equal 
the requirements of a listing is never a basis for denying a claim. We 
can find nothing in the proposed rules, or in the preamble to the 
proposed rules, that could have led the commenters to draw this 
conclusion. The results of suboptimal exercise testing are only one 
piece of evidence that we must consider in the context of the entire 
record.
    Comment: Another commenter was concerned that the proposed rules 
would ``no longer prohibit adjudicators from relying upon suboptimal 
exercise tests in determining that the listing for ischemic heart 
disease is not met.'' The commenter asserted that the use of suboptimal 
tests would allow adjudicators to conclude ``erroneously'' that a 
listing was not met, and that our statement in the preamble that 
suboptimal tests are a useful measure of function (56 FR 31267) is 
contradicted by listing 4.04, which does not permit adjudicators to 
consider the test as a sign of the claimant's limitations. The 
commenter said that the listings should be changed to specifically 
allow adjudicators to consider the inability to complete a test as a 
ground for establishing that an impairment is of listing-level 
severity, as is done in listing 4.02, which recognizes that markedly 
symptomatic exercise intolerance may meet that listing. Alternatively, 
the commenter recommended that the listing should explicitly state that 
the inability to complete a test is not evidence that a person is free 
from ischemic heart disease. A similar comment added that our assurance 
that those cases that do not meet the listings could be allowed under 
the rules for equivalence was not persuasive because the commenter had 
seen many cases in which this had not occurred.
    Response: We believe that the commenter who made the first comment 
misunderstood our prior rules and the statement we made in the preamble 
to the proposed rules about why we deleted the requirement for 
attainment of a targeted heart rate. An impairment meets a listing in 
only one way: by matching exactly the criteria in that listing. Because 
the results of suboptimal exercise tests have never been criteria in 
our listings, they cannot be--and never could have been--a per se 
reason for finding whether a person's impairment met or did not meet a 
listing. Thus, the listings did not ``prohibit'' us from using the 
results of suboptimal exercise tests to find that an impairment did not 
meet a listing. The only question in any ``meets'' finding is whether 
the person has presented evidence that satisfies the specific criteria 
of the listing.
    As we have explained in the preceding response, the failure of a 
suboptimal test to diagnose coronary disease does not mean that the 
test results are without use; and it is not a ``false negative'' in the 
scientific sense, because we are not using the exercise test for 
diagnosis. This is not a new policy. We have always required our 
adjudicators to consider at all steps in the sequential evaluation 
process all relevant evidence of an individual's functioning, which 
includes failure to complete an exercise test.
    Nor does listing 4.04 prohibit adjudicators from using the results 
of suboptimal or negative exercise tests as evidence of a person's 
aerobic functional capacity. It is faulty reasoning to conclude that we 
do not consider the implications of a person's ability to function on a 
suboptimal or negative exercise test because we do not include a 
criterion within the listing for a test that is not ``positive.'' All 
this means is that a claimant with a suboptimal or negative exercise 
test cannot have, on the basis of that test, an impairment that 
``meets'' the requirements of those listings that include exercise 
tests among their criteria.
    We did not adopt the suggestion to add a criterion to final listing 
4.04 which would permit a finding of ``meets'' the listing based on 
markedly symptomatic exercise intolerance. The commenter's comparison 
with listing 4.02 was inapt. In proposed listing 4.02B, the requirement 
for ``markedly symptomatic exercise intolerance'' (the language of 
which we have now deleted in our clarification of the final rule) had 
to be associated with objective, demonstrable chronic heart failure, 
with cardiac enlargement or ventricular dysfunction. In final listing 
4.04A, the listing for angina demonstrated by exercise testing, the 
objective requirement is the ECG abnormality or inability to maintain 
adequate blood pressure on exercise to support the inference that 
symptoms alleged at a low level of exercise may be attributed to 
ischemia; if we were to replace it with an allegation of exercise 
intolerance, the listing would not have any objective criteria. Again, 
this does not mean that an individual who is actually limited by his or 
her ischemic heart disease despite inability to ``meet'' this listing 
cannot be found disabled. The person may present evidence demonstrating 
equivalence to a listing, or by establishing a residual functional 
capacity which prevents him or her from working at past work and other 
work. Also, exercise intolerance may be indicative of a noncardiac 
condition that may alone or in conjunction with a cardiac impairment 
render a person disabled.
    Nevertheless, we find reasonable and helpful the commenter's 
suggestion that we add a statement to the listing that the ability or 
inability to complete an exercise test is not evidence that a person is 
free from ischemic heart disease. We believe, however, that the 
statement is more appropriate in 4.00C2e(1) of the preface, where we 
discuss evaluation of the results of exercise testing. We have, 
therefore, added the statement as a new second sentence in final 
4.00C2e.
    Comment: Four commenters referred to the amicus curiae brief filed 
in the case of State of New York v. Sullivan by the American Medical 
Association and the American College of Cardiology, in which it was 
argued that the treadmill exercise test is not a fair measure of stress 
from heat, cold, humidity, pollution, altitude, psychological 
pressures, or other work-related stresses in the workplace. The brief 
also pointed out that in the workplace a person may, for example, be 
required to perform tasks that require substantial use of the arms, 
isometric exertion and physical effort that is sudden or prolonged, and 
these things are not measured by the treadmill exercise test. In 
identical language, two of the commenters said that the proposed rules 
demonstrated that we ``continue to ignore this medical reality.''
    Response: These final rules are consistent with the facts presented 
in the amicus curiae brief. As the commenters noted in their quotes 
from the brief, the argument presented by the two medical associations 
discussed the efficacy of the treadmill stress test as the sole or 
primary basis for establishing a person's ability to work, not the 
propriety of our use of the treadmill exercise test in our listings. 
Indeed, we received comments on the NPRM from both medical 
organizations, and neither of them objected to the proposed rules in 
this regard--in fact, no physician advised us that he or she objected 
to this aspect of the proposed rules.
    However, as we have explained above, we have revised the final 
rules to lessen further the likelihood that they can be misinterpreted 
to mean that the treadmill exercise test is the ``primary'' basis for 
assessing ``functioning'' throughout the sequential evaluation process. 
We have also revised the final rules to underscore our longstanding 
policy that when an individual's impairment does not meet a listing, 
all relevant evidence must be considered to determine whether the 
individual is disabled. It is in the residual functional capacity 
assessment that factors are considered such as the effect of heat, 
cold, humidity, pollution, altitude, psychological pressures, and other 
work-related stresses.
    Comment: Four commenters also opposed the rule that listing 4.04, 
for cardiac ischemia, could not be ``met'' without the results of 
exercise testing unless the individual was at risk for exercise. The 
commenters said that this rule prevents consideration of other test 
results, such as echocardiograms and angiograms, under the listings. 
One of the commenters said that the listings must state that the 
criteria in listings 4.04B and C (the listings that include 
echocardiography and angiography among their criteria) can be met even 
if the claimant can exercise on a treadmill or even if an exercise 
treadmill test is negative. The same commenter said that 4.00A 
``instructs adjudicators to disregard'' evidence of other tests, the 
opinions of treating physicians and the claimant's pain, and that the 
listings do not permit signs or symptoms (such as pain) to be 
considered as the basis for proving that an impairment actually meets a 
listing.
    Response: We did not adopt the comments, except to the extent that 
we included language in the new fourth paragraph of final 4.00A which 
reminds adjudicators that the listings are only examples of common 
cardiovascular disorders that are disabling. The listings are intended 
to be a screening device by which we can identify and allow claims 
filed by the most obviously disabled individuals; they are not an all-
inclusive list of disabilities under which all individuals must be 
found disabled. Even though echocardiography can demonstrate 
abnormality of cardiac functioning, and coronary angiography is the 
``gold standard'' for ascertaining the presence of coronary 
atherosclerosis, neither of these tests provides information about 
whether any discovered disease is directly related to or predictive of 
functional status in the way that a positive exercise test does.
    This does not mean, however, that individuals who are unable to 
exercise for the requisite time on a treadmill, or individuals who have 
negative exercise tests will be found not disabled. All it means is 
that we cannot find that they have impairments that ``meet'' the 
listings. We believe that our revisions to the preface have clarified 
that such individuals will always receive the benefit of an 
individualized assessment that considers all of the evidence--including 
the results of any other tests they may have undergone, their symptoms, 
and their treating sources' opinions.
    With respect to the last comment, we could not find any text in the 
NPRM in which we instructed our adjudicators to disregard any evidence, 
nor would we have intentionally written such a rule because it would 
have been contrary to our policy. Indeed, subsequent to the publication 
of the NPRM, we published final rules that explicitly address the 
importance of the role of pain and treating source opinions in our 
adjudications (see ``Standards for Consultative Examinations and 
Existing Medical Evidence,'' 56 FR 36932, August 1, 1991; and 
``Evaluation of Symptoms, Including Pain,'' 56 FR 57928, November 14, 
1991). Again, if the evidence of record, including evidence from a 
treating physician, and including evidence of pain or other symptoms, 
establishes that an individual is disabled, the fact that the evidence 
fails to show that a listing is met or equaled will not prevent a 
favorable determination. Moreover, listing 4.04 is a listing for 
cardiac ischemia, which ordinarily describes a kind of chest pain; we 
do not even consider using this listing unless the individual has the 
symptom of ``chest discomfort,'' as we state in the opening sentence of 
the listing.
    Comment: One commenter criticized the example of equivalence we had 
provided in the preamble to the proposed rules (56 FR 31267). The 
commenter also said that the listings as proposed did not set forth any 
substantive method for considering nonexercise test evidence in 
determining equivalence, and the general language in the preface about 
equaling any listing was insufficient.
    Response: We disagree. We did not include the example in the final 
rules because it is virtually impossible to devise a single example, or 
even two or three examples, that would illustrate such complex concepts 
as, for instance, equivalence. Moreover, any example we devised would 
have to be so clear and unambiguous that it would not provide useful 
guidance; indeed, this could be a valid criticism of the example we 
provided in the preamble to the NPRM.
    We do not provide methods for determining equivalence in any of the 
listings sections in part A or part B. We have separate rules in 
Secs. 404.1526, 416.926, and 416.926a for that purpose.
    Comment: In addition to those commenters who mentioned that an 
evaluation of pain should be included as part of the listings, two 
commenters thought that the proposed rules allowed exercise testing to 
be used to overcome an individual's allegations of pain. One commenter 
said that lack of adequate consideration of the limitations imposed by 
pain associated with ischemia will result in unrealistic functional 
assessments. The commenter referred to the decision in Avery v. 
Secretary of HHS, 797 F.2d 19 (1st Cir., 1987), which requires an 
individualized assessment of pain in the event of a medically 
determinable impairment that could cause pain.
    Response: We did not intend to give the impression that a single 
exercise test in and of itself would be sufficient evidence to overcome 
an allegation of pain. As noted above, in November 1991, we published 
final rules regarding the evaluation of pain (56 FR 57928). Among other 
things, these rules require consideration of all relevant evidence, 
including a number of specific factors that must be considered whenever 
we consider an allegation of a symptom, such as pain. Our rules also 
require an individualized assessment of each person's allegations of 
pain, consistent with the Avery decision.
    Comment: Several commenters raised issues of treating physician 
opinion and its relationship to the rules on exercise testing. A number 
of the commenters questioned the proposed rule in 4.00C2c that 
permitted a program physician to override a treating physician's 
opinion as to the advisability of conducting an exercise test under 
some circumstances. Most stated that we should never permit a program 
physician to require a claimant to submit to an exercise test against 
the recommendation of a treating physician. Several commenters thought 
that the program physician's decision to override a treating source's 
opinion would be limited to a review of the medical evidence in the 
claim file without giving proper weight to the treating source's 
opinion. Three commenters, citing the proposed rule in 4.00C2f (final 
4.00C2e(1)) that required us to obtain statements from treating sources 
before we would decide whether to override their opinions, felt that 
the rule would create professional relations problems which will be 
magnified when the treating physician's opinion is overridden. One 
person suggested that the rules should require the program physician to 
ask the treating physician his or her opinion on whether the exercise 
test should be performed and to ask whether an exercise test had been 
performed in the past year. One commenter felt this policy could 
interfere with patient/doctor relationships. Another questioned if a 
statement should be made in the regulations that the facility 
performing the test should decide whether the test is contraindicated 
prior to its performance. Three commenters wanted to know what we would 
do if there is no treating source. One commenter asked whether our use 
of the term ``examining source'' meant that we would contact every 
physician who had examined the claimant, even if the physician was not 
a treating physician.
    Several commenters stated that we were not giving proper weight to 
the opinion of treating physicians because proposed 4.00D2 required the 
purchase of an exercise test if a timely exercise test was not 
available and posed no significant risk to the individual.
    Response: In response to the comments, we have made a number of 
revisions in the preface; however, we did not adopt the comments that 
asked us to prohibit program physicians from ever overriding the 
opinions of treating sources. In 4.00C2c of the NPRM we stated that we 
``will generally give great weight to the treating physicians' opinions 
and will generally not override them.'' We also characterized as 
``limited'' the situations in which we would override such an opinion 
and require a written statement of explanation whenever a source's 
opinion was overridden. Clearly, therefore, we did not expect program 
physicians to override the opinions of treating sources except in the 
rarest of cases.
    To make clear what those cases are, and in response to the 
comments, we first deleted proposed 4.00D2, which required exercise 
tests in all cases in which such testing posed no significant risk to 
the individual, thereby sharply limiting the number of cases in which 
the issue of whether to purchase an exercise test will even arise. 
Second, we revised final 4.00C2a to provide guidance for when purchase 
of an exercise test may be appropriate: when there is a question 
whether an impairment meets or is equivalent in severity to one of the 
listings, or when there is insufficient evidence in the record to 
evaluate aerobic capacity, and the claim cannot otherwise be favorably 
decided. Third, we revised 4.00C2c to state plainly that it will be a 
rare case in which the program physician would override the opinion of 
a treating source with respect to the purchase of an exercise test. If 
the treating source's opinion is overridden, the program physician must 
document the file with a rationale setting forth his or her reasons for 
doing so. We also expanded final 4.00C2c to indicate that an exercise 
test will only be purchased if it poses no significant risk to the 
individual and the claim cannot otherwise be favorably decided. Fourth, 
we revised final 4.00C2e(1) to state even more clearly the rule from 
the NPRM that, if purchase of an exercise test is contemplated, and the 
claimant has a treating physician who has not ordered an exercise test, 
and there is no apparent reason why the person would be at risk, 
contact must be made with the source to determine the source's opinion 
whether exercise testing poses a significant risk, or whether there is 
some other reason why the treating physician did not have the test 
performed, such as because of a financial bar.
    We do not believe that there will be significant professional 
relations problems from these rules. We believe that physicians will 
understand that we are contacting them out of concern for their 
patients' well-being, inasmuch as we will have to contact them when we 
need to purchase an exercise test and it is not clear why they have not 
ordered one, or why they think an exercise test poses a significant 
risk. Also, again, it should be a rare instance when the treating 
physician's opinion will be overridden.
    It has always been the responsibility of the provider performing 
the exercise test to determine whether there is any risk to the 
claimant. Also, if there is no treating source, the program physician 
will be responsible for assessing the risk of exercise to the claimant, 
as has always been the case in the past. Neither of these policies is 
new, nor are they unique to the cardiovascular listings. However, in 
response to the comment that asked whether all ``examining'' but 
nontreating sources should be contacted, we have revised the section by 
deleting the phrase ``examining physician.'' Under the aforementioned 
``Standards for Consultative Examinations and Existing Medical 
Evidence,'' we no longer use the term ``examining physician,'' and our 
rules requiring recontact with physicians are clearly set out. Whether 
it will be necessary to contact other sources who may have examined the 
claimant but who are not treating sources will vary from case to case.
    Comment: With regard to general policy on treating source opinions, 
several commenters said that the rules demonstrated that we do not give 
appropriate weight to such opinions. One commenter said that we did not 
provide any instruction in the listings on how to evaluate treating 
physician opinions about the nature and severity of the claimant's 
impairment, or which demonstrate that particular exercise test results 
are questionable, or provide some other basis for establishing 
disability. Another commenter said that the final rule must make clear 
that the results of an exercise test do not constitute substantial 
evidence to override a treating physician's opinion on the severity of 
the claimant's impairment. One commenter from a State located within 
the Second Circuit said that an exercise test should not be purchased 
if the treating physician can determine disability without it. The 
commenter said that, by purchasing an exercise test, we would not be 
according proper weight to the opinion of the claimant's treating 
physician if the treating physician has determined that the test is not 
necessary to evaluate disability.
    Response: We added the clause, ``in addition to information about 
the nature and severity of the impairment,'' to the end of the second 
sentence of the second paragraph of final 4.00A (which was the third 
sentence in proposed 4.00A). This revision reflects Secs. 404.1527 and 
416.927 of our rules. Otherwise, we did not adopt the comments. We 
would not provide rules for evaluating treating source opinions in this 
listing or any other because there are separate rules (Secs. 404.1527 
and 416.927) for considering the opinions of treating physicians. (See 
56 FR 36932, ``Standards for Consultative Examinations and Existing 
Medical Evidence,'' August 1, 1991.) The rules apply to all 
impairments.
    When a treating physician provides us with evidence that 
demonstrates that the results of a particular exercise test are 
questionable, or a treating source provides evidence upon which we 
could base an allowance, our general rules provide that we will have to 
determine whether there is a conflict in the evidence. If there is a 
conflict in the evidence, we are required to resolve it, giving more 
weight to the treating source's opinion than we would if the opinion 
had come from a source who was not a treating physician (see 
Secs. 404.1527(d) and 416.927(d)). If necessary to resolve the 
conflict, we may recontact the treating physician for additional 
information. Of course, we may be able to resolve the conflict based on 
the existing evidence, such as when we agree with the treating source 
that the results of a test are questionable or that the claimant is 
disabled. Our rules also explain how we decide whether there is 
substantial evidence to overcome a treating source's opinion. If the 
treating physician's opinion that a claimant is disabled is well 
supported by the evidence of record and is not contradicted by the 
other substantial evidence, we will give it controlling weight.
    In response to the last comment, the Act provides that we must 
determine whether a claimant is disabled, not the treating physician. 
We are not aware of any decision of the Second Circuit, or any other 
Federal court, that holds that a treating source may make the 
determination of disability or decide the sufficiency of the evidence 
for SSA under the Act.
    Comment: Several attorneys opposed the proposed rules, asserting 
that they were in conflict with the Social Security Act and the 
decision in the State of New York case in which the United States Court 
of Appeals for the Second Circuit ruled that all relevant evidence must 
be considered in determining disability and that the results of 
exercise tests cannot be used to the exclusion of other evidence to 
deny disability benefits. The commenters offered a number of arguments 
addressing the proposed policies both in general and as they pertained 
specifically to the ``meets,'' ``equals,'' and residual functional 
capacity aspects of our decisions. Two of the commenters cited the 
United States Supreme Court's decision in Sullivan v. Zebley, 493 U.S. 
521 (1990), in support of the notion that all disability determinations 
must be based on an individualized consideration of each claim and 
reflect all relevant medical evidence.
    Response: We believe that the revisions we have already described 
address most of the commenters' concerns, and that these rules are not 
contrary to the Second Circuit's holding in State of New York. We also 
believe that we have the statutory authority under section 205(a) of 
the Act (made applicable to title XVI by section 1631(d)(1)) to issue 
these rules, which have national applicability and which the Second 
Circuit (or any other court) has not reviewed or found to be invalid.
    We also want to emphasize that we consider all of the medical 
evidence of record at step 3 of the sequential evaluation process, 
i.e., the listings step. We do this to be certain that the evidence is 
consistent, to resolve any apparent conflicts in the record, and, if we 
ultimately rely on a treadmill exercise test to find a claimant 
disabled under the listings, to be certain that it is a valid test and 
not a ``false-positive'' test. Our policy does not permit a finding of 
``meets'' or ``equals'' in the face of a record that casts doubt on the 
validity of the test results; plainly, we must consider the entire 
record to determine whether the test raises such a doubt. Moreover, at 
the equivalence stage of step 3, evidence that may not play a role in a 
finding of ``meets'' may be decisive in our determination that the 
claimant is disabled; and, as we have repeatedly stated throughout this 
preamble, if a claimant is unable to establish that he or she has an 
impairment that meets or equals the severity of a listing, we will 
continue the sequential evaluation to determine whether, based on all 
the evidence of record, the claimant is disabled.
    Beyond that, we believe that the Supreme Court ruled in Zebley that 
it is not possible to provide individualized assessments to all 
claimants under the listings, and that the listings could never be 
revised to adequately provide for such assessments. For example, in its 
detailed analysis of the listings as a sole test of disability for 
children claiming benefits under the SSI program, the Court stated that 
``the listings obviously do not cover all illnesses and abnormalities 
that actually can be disabling,'' that ``the listings * * * exclude any 
claimant whose impairment would not prevent any and all persons from 
doing any kind of work, but which actually precludes the particular 
claimant from working, given its actual effects on him--such as pain, 
consequences of medication, and other symptoms that vary greatly with 
the individual,'' that ``the equivalence analysis excludes'' many 
claimants, and that ``there are several obvious categories of claimants 
who would not qualify under the listings but who nonetheless would meet 
the statutory standard.'' 493 U.S. at 533-34. Even more to the point, 
the Court stated:


    Even if the listings were set at the same level of severity as 
the statute, and expanded to cover many more * * * impairments, no 
set of listings could ensure that * * * claimants would receive 
benefits * * * under the individualized, functional analysis 
contemplated by the statute * * *. No decision process restricted to 
comparing claimants' medical evidence to a fixed, finite set of 
medical criteria can respond adequately to the infinite variety of 
medical conditions and combinations thereof, the varying impact of 
such conditions due to the claimant's individual characteristics and 
the constant evolution of medical diagnostic techniques.


Id. at 539, emphasis in original. The Court's holding in Zebley, 
therefore, was to require an additional step beyond the listings step, 
at which we would be required to satisfy the statutory criterion in 
title XVI of the Act of providing an individualized analysis for 
children based on all the evidence on a case-by-case basis; it was not 
that we must expand the listings to provide individualized assessments 
at the listings step. Therefore, we believe that, far from supporting 
the commenters' contentions, the Zebley decision refutes them.
    Again, all claimants who are not found disabled at the listings 
step receive the kind of individualized assessment which considers the 
effects of pain, the side effects of medication, and the myriad other 
factors that describe their particular responses to their particular 
impairments, as required by the law. Much of the new text we have added 
to the prefaces to Parts A and B of these final rules is intended to 
underscore these important, longstanding policy principles.
    Moreover, the listings step, which is only an allowance mechanism, 
is not required by the Social Security Act. If we did not have a 
listings step or if we were precluded from using our listings, it would 
only work to the detriment of claimants in terms of delaying some 
allowances, not to their advantage.
Information in the Preamble
    Comment: One commenter expressed the view that in a number of 
instances, and especially in the childhood listings, we had proposed 
significant changes to our rules that were not mentioned or explained 
in the preamble. The commenter suggested that this would preclude the 
public from having a meaningful opportunity to comment on proposed rule 
changes.
    Response: We believe the NPRM did give the public a meaningful 
opportunity to comment on the proposed rules, and that the detailed 
nature of the comments we received, which are discussed in this 
preamble to our final rules, attests to this fact.
The New Listings Will Result in More Denials
    Comment: Three commenters thought that the proposed listings would 
result in our denying more claims for benefits. One commenter said that 
the new listings would result in denials of benefits to ``tens of 
thousands of seriously impaired adult cardiac disease claimants.'' The 
other two commenters said that the proposed listings would result in 
more denials by the State agencies, and that this would result in more 
requests for hearings before an administrative law judge. One of these 
commenters also thought that the proposed listings provided less 
discretion to the State agencies than the former listings did.
    Response: We use the listings only to find people disabled. Failure 
to have an impairment that meets or equals in severity a listing is 
never a basis for denying any claim, whether the claim is filed for an 
adult or a child.
    However, it is true that some people whose impairments would have 
met the criteria of our prior listings will not be found to have 
impairments that meet these final listings. It is also true that some 
of these people will not be found disabled. For example, under 4.04B of 
the prior listings, individuals with chest pain of cardiac origin and 
an ejection fraction of 30 percent had impairments that met the 
listing. We now know that an ejection fraction of 30 percent does not 
reliably establish the severity or functional effects of an impairment; 
indeed, many people with ejection fractions of 30 percent function 
normally.
    It is, of course, not true that the listings will result in the 
denial of individuals who are seriously impaired, nor was that our 
intent. Our only aim in these revisions was to fairly and accurately 
update the listings consistent with current medical knowledge.
    Nevertheless, in response to these and several other comments 
discussed below, we have revised 4.00A and 104.00A to clarify that the 
guidance in the prefaces to the listings is primarily for the purpose 
of deciding whether an impairment meets one of the listings. We have 
also added new paragraphs to 4.00A and 104.00A (already described 
above, in the summary of provisions) which remind adjudicators that 
individuals whose impairments do not meet any listing may still have 
impairments that are equivalent in severity to a listed impairment, or 
that may result in a finding of disability as a result of the adult or 
child sequential evaluation processes based on an individualized 
assessment of their functioning. We have also added language in several 
places in the prefaces to state that all evidence in the case must be 
considered, and that, if the evidence already obtained establishes a 
favorable decision, the adjudicator should not continue development 
just to determine whether an impairment meets a listing.
    Finally, we do not agree that the rules provide less discretion to 
the State agencies than the former listings. As the former listings 
were based almost exclusively on objective, diagnostic medical 
findings, the revised listings which provide functional criteria as 
well as medical criteria arguably provide the decisionmaker with more 
discretion.

4.00  Preface to Part A

4.00A  Introduction
    Comment: A number of commenters were concerned about the statement 
in the NPRM that ``most individuals usually have received the benefit 
of a medically prescribed program of progressive physical activity 
accompanied by relevant educational and psychological support.'' They 
said that many of the people who file for title II and SSI disability 
benefits are outside the mainstream of health care and cannot afford 
such treatment or do not live where it is available. Three commenters 
thought that we would deny claims in which the claimants did not 
undergo treatment; one of these commenters said that the listings 
indicated that a claimant who did not undergo treatment should be 
denied. Two commenters were concerned that the rules were related to 
the rules on failure to follow prescribed treatment. One medical 
association suggested that the language in 4.00A should be, ``Whenever 
there is such evidence, the clinical record must include a description 
of the therapy and positive evidence of rehabilitation measures 
prescribed by the treating source and response.'' One attorney 
commenter pointed out that listing 4.04 requires a regimen of 
prescribed treatment as a prerequisite under that listing, so that a 
claimant whose impairment satisfies the criteria in listing 4.04A but 
does not receive treatment still could not be found disabled under that 
listing.
    Response: We adopted the comments by clarifying the final rules. In 
the second paragraph of final 4.00A, we now indicate that ``many'' 
individuals who have ``listing-level'' impairments will have received 
the benefit of such treatment. Although we agree that people with 
impairments of lesser severity than those in the listings may not 
necessarily receive this kind of therapy, we believe that the listing-
level impairments are so severe that many, if not most, individuals 
with such serious--and in the case of cardiovascular impairments, 
potentially life-threatening--impairments will be placed on some sort 
of rehabilitative and supportive program.
    However, we also state that, ``[w]henever there is such evidence * 
* *, [i]t is important to document any prescribed therapy and response 
because this medical management may have improved the individual's 
functional status,'' recognizing that there will be cases in which no 
such treatment was prescribed or instituted. This does not mean that we 
will find individuals who have not received treatment to be not 
disabled, only that we cannot find them to have impairments that 
``meet'' the requirements of those listings that require a regimen of 
therapy, as noted by the last commenter. For instance, an individual 
whose impairment meets the requirements of final listing 4.04A but does 
not have any treatment available to him or her, and who can establish 
duration of 12 months (prospectively or retrospectively) could be found 
to have an impairment that is equivalent in severity to the listing.
    To underscore our policy that we never deny claims because of 
failure to meet the listings, we added new paragraphs in final 4.00A 
and 104.00A which stress the necessity for making an equivalence 
determination when a claimant's impairment(s) does not meet a listing, 
and for assessing residual functional capacity (or performing an 
individualized functional assessment) when a claimant's severe 
impairment(s) neither meets nor equals in severity any listing. We have 
also added reminders in final 4.00A and 104.00A to consider all 
relevant evidence whenever there is no evidence of therapy.
    Finally, the rules on failure to follow prescribed treatment are 
quite complex, but have one simple underpinning: They do not come into 
play unless an individual's treating source has prescribed treatment 
for the individual which the individual is not following. If the 
individual's treating source has not prescribed treatment, or the 
individual does not have a treating source, the principle does not 
apply.
    Comment: Two legal services organizations commented on the 
statement in 4.00A of the NPRM that individuals who are 
``deconditioned'' may do poorly on exercise testing. The commenters 
thought that this meant our adjudicators would disregard deconditioning 
in the determination of disability. One commenter said that the section 
should instruct adjudicators to consider the effects of deconditioning 
as a basis for a finding of disability because it is a consequence of a 
cardiovascular impairment or a side effect of treatment (e.g., 
bedrest). The same commenter also said that we should include 
deconditioning as a listing criterion in listing 4.04.
    Response: We did not adopt the specific comments, but we have 
clarified the rules. By ``deconditioned,'' we meant the reversible 
exercise intolerance that comes from a lack of activity, such as from 
prolonged hospitalization, but which resolves with therapy. Thus, one 
of our reasons for requiring a longitudinal clinical record of at least 
3 months was to allow time for the individual to become reconditioned; 
in this way our evaluation would consider the individual in the most 
stable condition and provide a more accurate picture of expected long-
term functioning. We have, therefore, revised the proposed language 
(now in the second paragraph of final 4.00A) to clarify our original 
intent that inactivity or bedrest may result in a reversible 
deconditioned state, and that individuals in such a deconditioned state 
may do poorly on exercise testing if the testing is performed before 
they have been reconditioned.
    We do not agree that the factor of deconditioning should be added 
to listing 4.04. Listing 4.04 already includes functional criteria that 
result from the listed impairment.
4.00B  Cardiovascular Impairment
    Comment: A national medical association noted that we had included 
arterial desaturation as one of the consequences of heart disease in 
4.00B4. They noted, however, that we had not provided a methodology for 
quantifying this condition, and suggested that purchase of exercise 
pulse oximetry in conjunction with exercise testing may be an objective 
measure of degree of impairment.
    Response: We did not adopt the comment. The documentation for 
arterial desaturation is found in the criteria in 4.06A or B and the 
methodology can be easily inferred from the criteria. We do not provide 
for the purchase of pulse oximetry to determine oxygen saturation 
because it is not as sensitive a measure as arterial blood oxygen 
tension. Of course, if we obtain it from a treating source, we will 
consider the results along with all other evidence.
4.00C  Documentation
    Comment: One commenter thought that the requirement in 4.00C1 for a 
12-lead resting ECG rather than a 3-lead test might pose a problem 
because the 3-lead test is used in smaller facilities and rural areas. 
Another commenter thought that the statement in 4.00C1 that the 
tracings of a resting ECG ``must be submitted,'' might be 
misinterpreted by adjudicators to mean that a resting ECG must be 
purchased even if the documentation is sufficient to meet a listing 
that does not require an ECG.
    Response: We did not adopt the comments. The provision is 
substantively the same as the provision in 4.00F of the prior listings, 
and has not been a problem in the past. We will, of course, accept a 3-
lead ECG which is submitted to us by a treating or examining source, 
and use it to decide the case if the remaining information in the 
record is sufficient. If this information is not sufficient for a 
favorable decision, we may purchase the needed evidence. When we 
purchase an ECG, it will be a 12-lead test. With regard to the second 
commenter's point, inasmuch as the language in the final rule is nearly 
identical to the language in former 4.00F and there was no 
misunderstanding of that language, we do not believe that these rules 
will be misinterpreted either.
    Comment: Two attorney commenters said that detailed descriptions of 
ECG findings without the original or legible copies of the tracing 
should be sufficient documentation, at least in the Second Circuit. The 
commenters, in nearly identical language, said that this regulatory 
requirement ``frequently results in purchase of a CE with [its] 
attendant problems.''
    Response: We require ECG tracings to minimize error in adjudication 
and to ensure that standardized criteria for interpretation are applied 
to all claimants. Having the actual tracings, or a copy, also permits 
us to compare the findings with other evidence in the record--such as 
ECGs from emergency rooms and other medical sources--which the treating 
source might not have seen, and permits us to create a longitudinal 
picture through serial tests. Furthermore, our requirement for the 
actual tracings or a copy is only consistent with standard medical 
practice; it is routine to send the tracings along with other medical 
records whenever referring a patient to another physician, and for this 
reason it is rare for treating sources to provide the kind of detailed 
description of the tracing and the methodology in their narrative 
reports needed to understand the interpretation and methodology. 
Although it is possible that the physician could provide us with a 
narrative showing all of the information we would need to resolve any 
questions we might have, we have found it much simpler and more 
straightforward to review the tracings.
    Under our recently published standards for obtaining consultative 
examinations, we do not anticipate the purchase of many consultative 
examinations merely because a treating source failed to submit actual 
tracings. The rules which are applicable nationwide, including in the 
Second Circuit, require us to make every reasonable effort to recontact 
the doctor to try to obtain the missing evidence. If the tracing is for 
some reason not available, the rules also provide that the treating 
source is the preferred source for a consultative examination, and 
that, furthermore, we may restrict the examination only to the evidence 
we need. Therefore, if it is necessary to complete the record, we may 
purchase a new ECG from the treating source, without the need for a 
full-blown, independent consultative examination. We will only purchase 
examinations from other physicians when we have good reason to do so, 
primarily because the treating source has been unable or unwilling to 
provide us with adequate information and is unable or unwilling to 
perform the consultation for us.
    The commenters seemed to have assumed that our rule for obtaining 
tracings is one that only works to the detriment of the claimant. In 
fact, because we have specialists in cardiovascular disorders working 
in our State agencies, and because treating sources are not always 
specialists, we often find abnormalities on ECGs that were not noted in 
the treating sources' readings. Without review of the actual tracings 
by a knowledgeable program physician applying nationally acceptable 
standards, erroneous decisions could result which adversely affect 
claimants. The rule can, and does, work both ways.
    Comment: A national medical association suggested that we should 
include a requirement for routine documentation of the effects of 
hyperventilation under 4.00C1b(4) because it is a necessary part of the 
examination.
    Response: We partially adopted the comment. We could not add a rule 
requiring documentation of the effects of hyperventilation under 
4.00C1b(4) because it is a general section that includes existing 
medical evidence. Inasmuch as this existing evidence often will not 
include documentation of the effects of hyperventilation, such a 
requirement would have little practical effect. However, we can require 
such testing when we purchase exercise testing on a consultative basis. 
Therefore, we have added this requirement under final 4.00C2b(1), the 
methodology section under ``Purchasing Exercise Tests.'' The new 
language is an almost exact copy of the third and fourth sentences of 
the first paragraph of 4.00F2 of the former rules.
    In addition, the comment made us realize that the statement in 
proposed 4.00C1b(4) was an inadvertent error. It required 
posthyperventilation tracings to be deferred for at least 10 minutes 
after hyperventilation, which is manifestly so long a recovery time 
that it would defeat the purpose of the hyperventilation study. We 
have, therefore, corrected the sentence to state what we originally 
intended--that the exercise test should be deferred for at least 10 
minutes after hyperventilation.
    Comment: A medical association provided an updated reference for 
exercise standards to be used in 4.00C1b(5).
    Response: Instead of updating the literature citations, we decided 
to delete the references entirely in favor of a more general 
requirement to use generally accepted protocols for post-exercise 
electrocardiograms. In this way, we ensure that the criteria will not 
again become out-of-date in the future.
    Comment: Several commenters asserted that the use of handrails and 
the Borg scale intensity rating of perceived exertion are not usually 
reported in the existing medical evidence. The commenters pointed out 
that, if this information is required for a program acceptable exercise 
test, it will require frequent recontact with the attending physician. 
They also said that this information will usually not be available from 
hospitals.
    Response: In response to the comment, we deleted the language on 
the Borg scale and the use of handrails. We agree with the commenters 
that these are not usually reported or available.
    Comment: One commenter referred to our statement in the third 
sentence of proposed 4.00C2a that ``[e]xercise test reports, when 
available, must be included in the file.'' The commenter said that 
there are occasions when certain exercise tests would not be germane to 
a decision, e.g., an exercise test performed only a week after an acute 
event or when chest discomfort is clearly not of coronary ischemic 
origin.
    Response: Although we do not agree with the commenter, we have 
deleted the sentence in our clarification of final 4.00C2a. Inasmuch as 
we have revised 4.00C2 to focus only on the purchase of exercise tests, 
it is no longer relevant to discuss available (i.e., existing) 
evidence.
    Even though we have not retained the sentence, it is still our 
policy that all relevant, existing evidence must be requested. In the 
case of a person alleging chest pain, this will include reports of 
exercise testing. We do not believe that it would be fair, or accurate, 
to automatically conclude that a person's chest pain is ``clearly'' not 
of cardiac origin when a physician has seen fit to order a treadmill 
test for the individual. Moreover, as we have stated several times in 
this preamble, the treadmill test provides valuable information about 
an individual's aerobic capacity which will always be relevant to 
determining functional capacity.
4.00C2b  Methodology
    Comment: An attorney said that we had acknowledged in 4.00C2b(1) 
that ``there is more than a little uncertainty about what constitutes a 
MET or 5 METS'' on an exercise test, and said that there was no 
universally accepted guideline in the medical community for how many 
METS a person has completed at a given level of a treadmill test. The 
commenter suggested, therefore, that we provide a definition in the 
listings that would serve as the standard for these listings.
    Response: We did not adopt the comment. It is universally 
understood by physicians that the metabolic equivalent, or MET, is a 
unit of sitting, resting oxygen uptake equal to approximately 3.5 ml of 
oxygen per kilogram of body weight per minute. Exercise protocols, such 
as the Bruce protocol, are standardized and have been developed so that 
each level approximates a workload in multiples of METS; hence, stage I 
of the Bruce protocol approximates 5 METS. (See, e.g., The American 
Heart Association Special Report, ``Exercise Standards: A Statement for 
Health Professionals From the American Heart Association,'' 
``Circulation,'' Vol. 82, No. 6, December, 1990, p. 2286; and Schlant, 
et al., ``Guidelines for Exercise Testing: A Report of the Joint 
American College of Cardiology/American Heart Association Task Force on 
Assessment of Cardiovascular Procedures (Subcommittee on Exercise 
Testing),'' printed in the ``Journal of the American College of 
Cardiology,'' Vol. 8, No. 3, September, 1986, p. 725, and 
``Circulation,'' Vol. 74, No. 3, September, 1986, p. 653A.) An exact 
MET level can be determined only by measuring the volume of oxygen 
uptake directly, but this is rarely done in conjunction with the 
clinical use of the ECG treadmill exercise test.
    We do not believe that there is any reason for us to define the 
criteria of these listings beyond what is universally understood by 
physicians. Inasmuch as we use the listings only to find people 
disabled, the fact that there is some variation from person to person 
in the actual workload imposed at a given level of an exercise protocol 
will not disadvantage any claimant. Those claimants who demonstrate the 
requisite findings of the listings on exercise will be found to have 
impairments that meet the listings; those who do not will still have 
the opportunity to demonstrate disability by showing equivalence to the 
listings or by an individualized assessment of their residual 
functional capacities.
    Comment: A commenter from a national medical association suggested 
the use of measured oxygen consumption to quantify functional capacity 
objectively. The commenter said that use of measured oxygen consumption 
may enhance objectivity of data obtained during exercise testing and 
strengthen interpretation of exercise test results in cases where 
effort on the part of the claimant is questionable; while measurement 
of oxygen consumption is not frequently used in clinical practice, the 
additional data gathered from this relatively simple, noninvasive 
technique may allow for more objective interpretation of respiratory or 
cardiovascular limitation.
    Response: Although we agree that exercise testing with measurement 
of maximal oxygen uptake provides an accurate determination of aerobic 
capacity, this procedure is not widely utilized and would also increase 
the cost of impairment evaluation prohibitively. Therefore, we are 
unable to adopt the comment. However, in response to the comment, we 
have added a discussion of the efficacy of such testing to final 
4.00C3, ``Other studies,'' and the importance of obtaining test results 
when the test has been performed by a treating source or other medical 
source.
    Comment: A national medical association suggested the 5 METS or 
less criterion should apply to leg exercise only and that we should 
provide a threshold for arm exercises. The commenter recommended that 
the arm threshold should be 60 to 70 percent of the leg criterion. The 
commenter also said that some work does not require much leg work and, 
therefore, thought the treadmill test could be inappropriate. The 
commenter said that some people without the use of legs may still be 
capable of performing many jobs.
    Response: The primary focus of the preface to the listings is on 
providing guidance for the evaluation of disability under the listings, 
although the guidance may also be applicable for disability evaluation 
at other steps of the sequence. The listings are only examples of 
impairments that are so severe that they preclude the performance of 
gainful activity. We believe that the exercise threshold levels we have 
provided in these listings are so limited as to render the issue of arm 
exercise at the listing level moot; even sedentary work requires some 
ability to walk, stand, lift, and carry, as well as the ability to 
travel to and from a job. We also want to make clear that, under our 
longstanding rules, people who do not have the use of their legs are 
always deemed to be disabled.
    We do agree with the commenter that arm exercise testing is less 
sensitive than treadmill testing in the detection of myocardial 
ischemia because the large muscle groups are not exercised. We believe 
that the maximal aerobic capacity is approximately 80 percent of a 
treadmill exercise test. A MET level on arm exercise that would be 
comparable to the 5-MET level of the treadmill test would be 4 METs, 
and in such a circumstance, an equivalence determination would be 
appropriate.
    Comment: Another commenter wondered whether the increase in 
systolic blood pressure before and early into exercise discussed in 
4.00C2b(4) and the diastolic rise in blood pressure discussed in 
4.00C2b(5) occur with sufficient frequency that they warrant inclusion 
in the listings. The commenter also suggested that we add a statement 
requiring review by a program physician to ensure the validity of the 
test interpretation. Another commenter recommended that we add the 
phrase, ``at peak level,'' to the third sentence of proposed 
4.00C2b(5), so that the sentence would begin: ``Conversely, an increase 
in diastolic blood pressure with low workloads, together with a 
decrease in systolic blood pressure at peak level and decrease in pulse 
pressure * * *.'' Another commenter also asked if the significance of 
severe systolic hypertension during exercise needs to be mentioned.
    Response: We believe that the guidance we provide in final 
4.00C2b(4) is important and that the phenomenon does occur frequently 
enough to warrant inclusion in the methodology section of the preface. 
It is well known that there is a degree of apprehension in the patient-
physician encounter which can result in a transient elevation of blood 
pressure, and this occurs commonly. As the test progresses, the 
transient elevation in blood pressure settles down. This degree of 
apprehension must be taken into consideration when blood pressure is 
measured early in an exercise test in order to distinguish between 
tests that are truly sign-or symptom-limited and those that register 
transient elevations in blood pressure early in the test as a result of 
apprehension.
    We agree with the first commenter that the changes in diastolic 
blood pressure discussed in proposed 4.00C2b(5) were not as important; 
it is the decrease in systolic blood pressure which is associated with 
significant coronary disease and which increases the risk of further 
treadmill testing. Moreover, the paragraph could have been misleading; 
it is, in fact, not invariably true that an increase in diastolic blood 
pressure with low workloads, together with a decrease in systolic blood 
pressure and decrease in pulse pressure, indicates a response to 
inadequate cardiac output. There are other reasons why this may occur. 
Therefore, we have deleted the paragraph and redesignated proposed 
4.00C2b(6) (with modifications to delete redundancies and cross-
references not in the final regulation) as final 4.00C2b(5). We did not 
adopt the comment to add ``at peak level.''
    We did not adopt the comment that asked us to add a sentence 
ensuring review by a program physician because the results of an 
exercise test must be considered in the context of all of the other 
evidence in the individual's case record, and we did not wish to 
mandate a physician review of only one specific type of evidence. We 
did not single out the issue of blood pressure because we believe that 
it is well understood by experienced program physicians that 
interpretation of exercise tests takes into account all relevant 
factors, including the individual's exercise time, stage of exercise, 
electrocardiographic manifestations, and blood pressure changes. Blood 
pressure changes as an isolated finding are not the basis of an 
exercise test interpretation.
    Finally, we adopted the comment that asked us to add information 
about the significance of systolic hypertension on exercise. We added a 
sentence to the end of final 4.00C2b(4) that states: ``In addition, 
isolated systolic hypertension may be a manifestation of 
arteriosclerosis.''
    Comment: A physician, noting that 4.00C2b(4) discusses the 
significance of a decrease in systolic blood pressure during exercise 
to below the usual resting level, wondered whether it would be helpful 
if we added a discussion of the significance of a decrease in systolic 
blood pressure during exercise that did not fall to below the resting 
level. The commenter noted similar passages in listings 4.02B2 and 
4.04A4.
    Response: As the commenter is undoubtedly aware, a decrease in 
systolic blood pressure that does not fall below the resting level may 
have no special significance. It is merely one of many factors the 
doctor must consider when interpreting the test results. Because we do 
not, and could not, list all of the factors that must be considered in 
exercise test interpretation, we do not believe that it would be 
helpful to address this particular one, either in the preface or the 
listings.
    Comment: We received two general comments about the examples of 
risk factors in proposed 4.00C2c. One commenter noted that there were 
fewer risk factors named in the proposed rules than in 4.00G3 of the 
former rules, and that we had recategorized some of the risk factors 
militating against the purchase of exercise testing in the former rules 
to factors that would limit interpretation of exercise test results in 
the proposed rules. This commenter, who believed that the rules 
``contemplate[  ] large numbers of exercise tests purchased by State 
agencies notwithstanding the judgments of treating physicians,'' said 
that we would also be exposing claimants to additional risk ``by 
abandoning many of the factors which have heretofore restrained the 
discretion of SSA decisionmakers.'' In a similar vein, the second 
commenter indicated a belief that, by changing some of the factors in 
the prior rules (such as musculoskeletal and neurological impairments) 
from risk factors to factors that would limit the usefulness of 
exercise testing, we were indicating that we would purchase exercise 
tests in which the results are likely to be of limited or no value 
because of the presence of other diseases.
    Response: In response to the comments, we have restored to final 
4.00C2c several of the examples from the former rules of conditions 
that would preclude purchase of exercise testing; specifically, Wolff-
Parkinson-White syndrome (which we have deleted from final 4.00C2e(2)), 
marked aortic stenosis, marked pulmonary hypertension, and limiting 
musculoskeletal and neurological impairments. We had proposed to delete 
the examples of second-and third-degree heart blocks because they are 
subsumed under the category of arrhythmias; moreover, second-degree 
heart blocks are not always significant risks to exercise testing, as 
in the case of Wenckebach periods. However, in response to the 
comments, we have included third-degree heart blocks and Mobitz II, a 
kind of second degree heart block that would preclude the purchase of 
testing, as examples of arrhythmias. We deleted the example of 
ventricular aneurysms because we now know that they do not rupture; 
therefore, we no longer consider them to be a significant risk to 
exercise testing for that reason. Arrhythmias that result from 
ventricular aneurysms would already be covered. However, in response to 
the comments, we have revised the example of aneurysms that was in the 
former rules to include chronic or dissecting aortic aneurysms and have 
added it to the list of examples. We deleted the example from the 
former rules of recent onset angina because it is too imprecise. Unless 
the angina is unstable or progressive, there is no significant risk to 
the purchase of testing.
    In some cases, the examples in the NPRM were the same as the 
examples in the former rules, but with more up-to-date or clearer 
language; for instance, ``atrial'' fibrillation instead of 
``auricular'' fibrillation and the example of cardiac drug toxicity 
instead of the less precise ``individuals on medication where 
performance of stress testing may constitute a significant risk.'' In 
the final rules, we have also clarified an example we proposed in the 
NPRM. Because ``recent'' aortic dissection was unclear, we have revised 
the example to ``unrepaired'' aortic dissection; thus, we will not 
require recency. The proposed rules contained the example of arterial 
dissection after coronary angioplasty; however, we eliminated this 
example because it was medically inappropriate. Coronary arterial 
dissection usually occurs during coronary angioplasty and is the major 
means by which arterial blood flow is reestablished. Therefore, it is 
not a risk factor for exercise testing. In addition, we have added 
language to 4.00C2c to indicate that the risk factors listed are only 
examples, not an all-inclusive list. Based upon the facts of each 
individual's case, the program physician can decide if other factors 
would pose a risk to the claimant.
    With regard to the first commenter's remark that we intend to 
purchase many exercise tests despite the judgments of treating 
physicians, we believe that the commenter misread the NPRM. As we have 
already explained, the proposed rules clearly stated that the program 
physician would ``give great weight to the treating physicians' 
opinions and will generally not override them,'' in this regard.
    Comment: One commenter from a national medical association 
recommended that the unstable progressive angina in 4.00C2c should be 
well-documented as it could be attributable to anxiety, especially 
following coronary artery bypass graft surgery.
    Response: We always try to document any impairment as well as 
possible. However, we believe that unstable angina due to any cause 
raises a sufficient question of risk that we will not purchase exercise 
testing.
    Comment: One national cardiological association said that our 
proposal in 4.00C2d to wait 3 months following an acute myocardial 
infarction or angioplasty before purchasing an exercise test was too 
long. They indicated an exercise test can be safely done within 1 to 2 
months. Conversely, a lay commenter suggested that we might extend the 
deferral period to 4 months.
    Response: We did not adopt the comments. We chose a 3-month 
deferral period not for reasons of safety, but to provide a reasonable 
period of time for recovery so that we could realistically evaluate the 
benefits achieved from medical management. We also believe that 3 
months is long enough to wait before purchasing the test, and that 
there is no need to extend the requirement to 4 months.
    Comment: One commenter asked whether the factors in proposed 
4.00C2e (final 4.00C2e(2)) could be used to rule out exercise testing, 
inasmuch as they limit test interpretation.
    Response: The factors in this section may, on an individual case 
basis, be used for this purpose, although they generally will not 
preclude exercise testing. We have included this section to alert 
program physicians that these things sometimes make interpretation of 
an exercise test more difficult. They do not necessarily pose a risk to 
the individual, nor do they make interpretation impossible.
    Comment: The same commenter asked us to define ``prolonged periods 
of physical inactivity.''
    Response: We have added a parenthetical statement indicating that 
an example of a prolonged period of physical inactivity could be two 
weeks of bedrest.
    Comment: Two attorneys, using identical language, said that they 
were concerned about the ``requirement'' for an exercise test for 
individuals with chronic heart failure, who generally are treated with 
digitalis. The commenters said that the medication alters the ECGs and 
can result in misinterpretation of the exercise test results, thereby 
denying benefits to disabled individuals. Two other attorneys, also 
using identical language, said that the rules authorized reliance on 
exercise tests where medication conceals the underlying impairment. One 
of these commenters said that we should instruct adjudicators to 
disregard results of exercise tests that are unfavorable to individuals 
when the individuals were on medication at the time of the test. 
Another commenter thought that we should not use treadmills or Holter 
monitors to evaluate ischemia when the individual takes medication that 
controls symptoms because a negative test result does not give a 
reliable picture of the person's ability to function on a sustained 
basis. This commenter also said that we routinely invalidate positive 
test findings when they are supportive of listing criteria because the 
claimant is on digitalis.
    Response: The final rules do not require exercise testing for 
people with chronic heart failure. They provide a listing under which 
people who have chronic heart failure can show listing-level severity 
through exercise testing, in addition to two other listings for the 
impairment that do not include exercise testing among their criteria. 
Moreover, the exercise test criteria in listing 4.02 do not include 
ischemic findings; they require only an inability to exercise at 5 METS 
or less or a need to stop exercising because of certain abnormalities 
that require the cessation of the test. Therefore, any effect of 
medication on the ECG will have no relevance to whether the person 
meets this particular listing. However, because these commenters 
misunderstood the language we had proposed, we have revised listing 
4.02B1 to make it clearer. We also repeat that the results of exercise 
tests alone will not result in denials of benefits.
    With regard to the issue of the value of exercise testing when 
medication controls symptoms, a so-called medication-affected test in 
an asymptomatic individual indicates the effectiveness of the 
treatment. The absence of ST changes on an exercise test does not mean 
that the person is free of coronary disease; however, the absence of 
ischemic ECG changes or symptoms at a given level of exercise indicates 
what can safely be done in terms of aerobic capacity. As we have 
already stated, we do not use the exercise test to determine the 
existence of an impairment, but the aerobic functional limitations 
associated with the impairment. If medication sufficiently controls an 
impairment, the impairment is not disabling.
    With regard to digitalis, we agree with the commenters that the 
presence of baseline segment abnormalities makes interpretation of an 
abnormal exercise test difficult and reduces the usefulness of such a 
piece of medical evidence in the evaluation of ischemic heart disease. 
This is because digitalis may induce or accentuate ST segment 
displacements on exercise, resulting in a false-positive test; for this 
reason, an abnormal exercise test of an individual taking digitalis 
could not in itself establish that the individual has met the criteria 
of the ischemia listing. However, this is not the case for normal 
tests, and a normal exercise test is valid. In any event, inasmuch as 
we have deleted the requirement to purchase exercise tests we believe 
that we have responded to the commenters' concerns.
4.00C3  Other Studies
    Comment: A national medical association suggested that we should 
place less reliance on imaging techniques such as echocardiography and 
radionuclide tests in determining function. The commenter said that too 
much emphasis is placed on ejection fraction when tests must be related 
to aerobic capacity, and that the effective cardiac output and 
effective stroke volume are more important.
    Response: We did not adopt the comment. It was not clear to us what 
the commenter meant by ``effective'' cardiac output and stroke volume. 
However, both cardiac output and stroke volume are measures of 
ventricular function that do not in themselves provide enough 
information from which one could infer reduced functional capacity. The 
implications of both depend on variables that we could not easily 
define in these listings. For instance, cardiac output depends on 
stroke volume and heart rate--which can vary through a wide range--as 
well as according to the individual's size. Therefore, neither test is 
amenable to a simple cutoff for listings purposes as the ejection 
fraction is. Moreover, stroke volume is one of the components used to 
calculate the ejection fraction. Even if we provide a threshold level 
for stroke volume in these listings, it would likely be redundant 
because it would also appear as a reduced ejection fraction.
    Comment: One commenter felt that stricter guidelines need to be 
given as to when studies described in 4.00C3 may be purchased to ensure 
that the treating physician's opinion is carefully considered in order 
to protect the safety of claimants.
    Response: We have clarified section 4.00C3 to indicate that these 
tests may only be purchased if the claim cannot be favorably decided on 
another basis, and the program physician, preferably one experienced in 
the care of patients with cardiovascular disease, has concluded that 
the test does not pose a significant risk to the individual.
    Comment: One commenter asked us to provide criteria for purchase of 
two-dimensional echocardiograms and radionuclide studies in 4.00C3.
    Response: We did not adopt the comment. We believe that this is a 
procedure that is more appropriate to our operational manual and other 
subinstructions than to the regulations.
4.00D  Treatment and Relationship to Functional Status
    Comment: One commenter suggested that the requirement in proposed 
4.00D4 to obtain results of biopsies during the first few months after 
transplant was unnecessary because the listing directs an allowance 
once we have documented that a transplant has been performed.
    Response: We adopted the comment by deleting the sentence. As the 
commenter correctly noted, under listing 4.09 there is a presumption of 
disability for 1 year following cardiac transplantation so that there 
is no need for requiring biopsy results within that period.
4.00E  Clinical Syndromes
    Comment: A physician noted the statement in proposed 4.00E1a that 
chronic heart failure may manifest itself as pulmonary congestion 
``or'' systemic congestion could be clarified to indicate that it might 
also result in both pulmonary and systemic congestion.
    Response: We adopted the comment.
    Comment: One national medical association suggested that we add 
``chest wall syndrome'' to the list of noncardiac conditions that may 
also produce symptoms mimicking myocardial ischemia.
    Response: We adopted the comment in final 4.00E3f.
    Comment: A national medical organization suggested three clarifying 
language additions for the second and third paragraphs of 4.00E4.
    Response: We adopted two of the suggestions but not the third. In 
the first sentence of the second paragraph of 4.00E4, we added the 
phrase, ``in the supine position,'' following the word, ``determined,'' 
as suggested because this is standard practice for determining the 
ankle/brachial blood pressure ratio. Similarly, in the fifth sentence 
of the third paragraph of the section, we now indicate that the 
treadmill for Doppler exercise may be on a ``10 or 12 percent'' grade, 
instead of 12 percent alone, in recognition of variations in standard 
practice; for the purposes of this listing, the difference in the 
grading is so small as to be immaterial. We did not adopt the third 
suggestion, to indicate in the same sentence that the exercise should 
be for ``up to'' 5 minutes, because we already include the possibility 
that the individual will not complete the test in the fourth sentence 
of the paragraph.
    Comment: Under 4.00E4 there is a requirement for a resting ankle/
brachial systolic blood pressure ratio of less than 0.50. A national 
medical association commented that this is an arbitrary ratio and that 
pain and pallor must also be taken into account, even though the 
individual may register 0.45.
    Response: We disagree. The requirement for a resting ankle/brachial 
systolic blood pressure ratio of less than 0.50 is not arbitrary, but 
is confirmed by the literature and clinical experience. (See, e.g., 
Carter, S.A., ``Rate of Pressure Measurements in Vascular Disease,'' in 
Bernstein, E.F., ``Non-invasive Diagnostic Techniques in Valvular 
Disease,'' The C.B. Mosby Company, Saint Louis, 1987.)
    Furthermore, the existence of pain and pallor is implicit in the 
requirements. Listing 4.12 requires ``intermittent claudication,'' 
which is a description of a kind of pain. People with the severely 
reduced ankle/brachial systolic pressure of 0.50 will have both pallor 
and rubor, among other clinical findings, and the symptom of pain 
associated with the degree of marked peripheral arterial disease 
described in the listings. We do not believe that it would be helpful 
to mention only one such common finding.
    Comment: One commenter suggested that we add text to 4.00E4 
explaining that varicose veins by themselves do not generally cause 
disability at the listing level, and that the listing concerns chronic 
venous insufficiency, not isolated varicosities.
    Response: Although the commenter was correct, we do not believe 
that it is necessary to add this much detail to the listings, since it 
is evident from the listed criteria that a mere finding of varicose 
veins could not meet or equal the criteria of the listing.

Listings

4.02  Chronic Heart Failure

    Comment: Several commenters believed a definition of cardiac 
enlargement should be provided.
    Response: We agree and have added two common examples of cardiac 
enlargement to listing 4.02A.
    Comment: Two attorney commenters noted that proposed listing 4.02 
introduced an exercise test criterion. One of these commenters said 
that we had presented no justification to support our conclusion that 
exercise testing is an appropriate measure of disability arising from 
chronic heart disease. The other commenter noted that in our former 
rules (in 4.00G3), we had listed congestive heart failure as a reason 
for not purchasing exercise tests. A third commenter asked us to define 
``markedly symptomatic exercise intolerance'' as used in proposed 
listing 4.02B1.
    Response: Experts in the field of cardiology indicate that exercise 
testing of individuals with chronic cardiac failure that is compensated 
is both safe and useful. Among the objectives for subjecting these 
individuals to exercise testing are the sensitive and specific 
detection of impaired cardiac performance, a gradation of the severity 
of chronic cardiac failure, the assessment of aerobic capacity of the 
patient, and the monitoring of the therapeutic response (see, e.g., 
``Cardiopulmonary Exercise Testing,'' Karl T. Weber, Joseph S. Janicki, 
1986, W. B. Saunders Company, Philadelphia). Nevertheless, we still 
exclude individuals who have chronic heart failure resulting in NYHA 
functional class IV limitations in 4.00C2c.
    Our proposal to use the term ``markedly symptomatic exercise 
intolerance'' was unclear. We have, therefore, clarified final listing 
4.02B1 by deleting the phrase and revising the final clause to state 
clearly what we intended; i.e., that the person is unable to exercise 
in an exercise test at a workload equivalent to 5 METS or less because 
of symptoms of chronic heart failure. In this way, the criteria for 
symptoms will be evaluated only under B2 of final listing 4.02.
    Comment: One commenter asked if in listing 4.02B the ``recurrent 
fatigue and dyspnea'' must occur at rest or with exercise, or either.
    Response: NYHA functional class III connotes that the individual is 
comfortable at rest but symptomatic on ordinary physical activity. 
Similarly, NYHA functional class IV connotes that the individual is 
symptomatic at rest. We believe that we have clarified these principles 
by deleting the references to the functional classes and instead 
incorporating into the listings narrative descriptions of the 
functional criteria.
    Comment: One commenter suggested that we include multiform 
ventricular premature contractions in listing 4.02B2a.
    Response: We adopted the comment. We have rephrased this listing 
section to indicate, ``Three or more consecutive ventricular premature 
beats or three or more multiform beats * * *.''

4.04  Ischemic Heart Disease

    Comment: Three commenters addressed the requirement in the opening 
sentence of proposed listing 4.04 for chest discomfort ``occurring 
repeatedly.'' One commenter asked us to define the phrase. Two 
commenters pointed out that individuals may restrict their activities 
so as to avoid discomfort or lessen its frequency. One of the 
commenters said that we should delete the phrase, arguing that the 
issue is not how often chest discomfort of ischemic origin occurs, but 
whether it occurs consistently with exertion.
    Response: We adopted the comment that asked us to delete the 
phrase. We agree with the last commenter that the issue is not how 
often the chest pain occurs, because the individual may have structured 
his or her activities so as to avoid chest pain, but whether the 
individual would have symptoms with a given level of exertion. 
Moreover, the specific listings criteria in the subparagraphs establish 
the level of functional severity for the listings.
    Comment: One commenter thought that the requirement in listing 
4.04A1 for findings in at least 3 consecutive complexes would make it 
too difficult to meet this listing. Another commenter asked if the 
requirement in listing 4.04A1 for depression in 3 complexes rather than 
2 is correct.
    Response: The requirement for findings in at least 3 consecutive 
complexes is correct. We changed the requirement from the prior 
listing's 2 complexes to bring these listings into line with standard 
medical practice. We do not intend to raise the severity level of the 
listing, but to make it more accurate and up-to-date by minimizing the 
effect of wandering baseline or artifacts.
    Comment: One commenter requested that we consider expanding 
proposed listing 4.04A5 to include abnormal findings on stress 
echocardiogram and IV Persantine tests. The commenter said that these 
tests could improve sensitivity in assessing claimants with coronary 
artery disease who have negative routine exercise tests.
    Response: We did not accept the comment because these are new 
technologies that are still being developed. Moreover, they are not 
widely available and there is currently no correlation indicating the 
sensitivity or specificity of these tests relative to more accepted 
technologies, such as thallium stress testing. For these reasons, it 
would not be appropriate at this time to include such modalities in our 
listings.
    Comment: Two attorney commenters thought that proposed listings 
4.04B and C tightened the criteria in the nonexercise test listings for 
ischemic heart disease and could not be met. One of these commenters 
said that this constriction of the listings was unfair and unwarranted, 
and that the former listing did not have these requirements. Another 
commenter asked why listing 4.04C requires both angiographic evidence 
of severe vessel occlusion and an ejection fraction of 30 percent or 
less.
    Response: In response to the comments, we revised the rules so that 
final listing 4.04C requires only angiographic evidence instead of both 
angiographic evidence and a decreased ejection fraction. We also 
revised final listing 4.04B so that it has the same functional severity 
level as listing 4.04C, i.e., the condition must result in symptoms on 
ordinary physical activity even though the individual is comfortable at 
rest; this equates with NYHA functional class III. Proposed listing 
4.04B had required NYHA functional class IV, which requires symptoms at 
rest.
    We revised the listings employing ejection fraction criteria from 
the prior rules, which permitted a finding of ``meets'' based on the 
ejection fraction alone, because it is well supported in the medical 
literature that individuals with even markedly decreased ejection 
fractions can functionally live perfectly normal lives; the ejection 
fraction alone does not indicate functional capacity. This is also the 
reason we require functional limitations associated with angiographic 
evidence of coronary atherosclerosis; as we have already explained, 
this test does not provide information about whether any discovered 
disease is directly related to or predictive of functional status.
    Comment: Two commenters noted that we deleted the criteria of 
former listings 4.04B1 through B5, which provided criteria for 
evaluating chest pain following myocardial infarctions based on resting 
ECG findings. One commenter said that resting ECGs should not be 
entirely omitted; another said that we should retain the prior 
listings.
    Response: We did not adopt the comments. We deleted the criteria 
because the presence of a previous myocardial infarction does not, of 
itself, guarantee that subsequent chest pain is of cardiac origin. 
Therefore, we need other information to establish that the individual 
is experiencing ischemia resulting in chest discomfort. An exercise 
test may be useful for this purpose. Additionally, a resting and 
exercise two-dimensional echocardiogram or radionuclide ventriculogram 
may also demonstrate cardiac dysfunction, including ischemia. Again, 
the fact that we have deleted the criteria does not mean that 
individuals who demonstrate these findings will be found not disabled. 
It only means that they do not have impairments that ``meet'' the 
criteria of the listing.
    Comment: Two commenters asked whether we deliberately deleted the 
word ``proximal'' in proposed listing 4.04C2b (final listing 4.04C1b), 
``70 percent or more narrowing of another nonbypassed coronary 
artery''; former listing 4.04B7b, the corresponding listing that was in 
effect at the time of the NPRM, had specified that the artery must be a 
proximal artery.
    Response: We did delete the word intentionally to recognize that 
lesions in other than a proximal portion of the major arteries may 
result in significant impairment.
    Comment: One commenter noted that we had deleted the criterion in 
former listing 4.04A5 for development of second-or third-degree heart 
block. The commenter thought that heart blocks and conductive defects 
with exercise are an important part of the ischemic heart disease 
listings and asked us to consider reinstating the criterion.
    Response: We did not adopt the comment. We deleted the criterion 
because it is unnecessary. Individuals with significant heart block or 
conductive defects will have either blood pressure changes or exercise 
intolerance and would be considered under the standards for those 
consequences of the impairment.

4.05  Recurrent Arrhythmias

    Comment: Four commenters believed that the requirement in listing 
4.05 that cardiac syncope be coincident with findings on a Holter 
monitor is an unduly restrictive requirement. They said that medical 
care is unavailable to many claimants, particularly in the SSI claimant 
population, who may only have the benefit of a Holter evaluation 
because of a consultative examination. The commenters also said that it 
was unreasonable to require that the claimant sustain an episode during 
an isolated twenty-four hour period.
    Response: We did not adopt the comments. There are many causes for 
syncope, including noncardiac causes and, except for the use of a 
Holter monitor with the individual lying on a tilt table or by 
ambulatory monitoring there is no other way to determine whether there 
is true cardiac syncope. There has to be an association between the 
symptom and medically determinable arrhythmia to satisfy the 
requirements of this listing.
    Even though we agree with the commenters that there will be some 
individuals who have true cardiac syncope but who have not been 
appropriately tested by treating sources, we do not believe that this 
will occur as often as the commenters do. Cardiac syncope is such a 
severe symptom that it is unlikely that many people who experience it 
will not be appropriately tested. In any case, and as we have stated 
throughout this preamble, those individuals who cannot present evidence 
showing that their impairments meet this listing may still establish 
disability in other ways.
    Comment: A national medical association suggested that the addition 
of the phrase ``near syncope'' would make the listing description of 
symptoms associated with uncontrolled arrhythmias more accurate.
    Response: We agree, and have added the phrase in final 4.00B3 and 
listings 4.05 and 104.05.

4.06  Symptomatic Congenital Heart Disease

    Comment: One commenter noted that the vast majority of claimants 
suffering from symptomatic congenital heart disease who suffer cyanosis 
at rest will meet either the requirements of listing 4.06A1 or A2. 
However, the commenter was concerned that we might deny claimants whose 
impairments do not quite meet the requirements of those listings. The 
commenter believed that cyanosis at rest should in itself be sufficient 
to meet listing-level severity.
    Response: We did not adopt the comment. The commenter was correct 
that the vast majority of people who are cyanotic at rest will have 
impairments that meet this listing. However, we did not list cyanosis 
by itself because it can be too subjective a finding on which to base a 
listing. The listings are not intended to be all-inclusive. Those 
individuals who have cyanosis at rest but who do not demonstrate the 
criteria of the listing may still demonstrate disability through the 
equivalence concept and, if necessary, through an individualized 
assessment of their residual functional capacities. However, as we 
explained earlier in this preamble, and in response to this comment, we 
deleted the proposed functional criteria from final listing 4.06 partly 
in recognition of the fact that individuals who are cyanotic at rest 
will necessarily have functional limitations of at least NYHA 
functional class III.
    Comment: One commenter suggested that we provide specific 
guidelines in the listings regarding when to purchase blood gas studies 
because these types of tests constitute an increased risk for 
claimants.
    Response: We did not adopt the comment. It would be very unusual 
for a person with congenital cyanotic heart disease not to have at 
least one blood gas test available; therefore, we do not expect to 
purchase many of these tests. We also believe that the guidelines for 
the purchase of such tests more appropriately belong in our operational 
manual instructions. These tests do not pose a risk to the claimant.

4.08  Cardiomyopathies

    Comment: One commenter was apparently concerned about the 
references to listings 4.02 and 4.04 in listing 4.08. The commenter 
noted that individuals with hypertrophic cardiomyopathy are high risk 
individuals and should not be subjected to exercise tests.
    Response: Listing 4.08 refers to criteria in other listings for the 
evaluation of cardiomyopathy because the consequences of this 
impairment are so varied (e.g., heart failure, ischemia, arrhythmia) 
that it is more efficient to refer to other listings to evaluate the 
consequences rather than repeating all the criteria under listing 4.08. 
We agree that individuals with hypertrophic cardiomyopathy are at risk 
for sudden death due to arrhythmias and physical exertion and they 
should not be required to do an exercise test. We have added this 
condition to the risk factors in final 4.00C2c to make it clear that we 
will not purchase exercise tests in these cases. We did not revise the 
cross-references in final listing 4.08 because some individuals may 
have undergone exercise testing and may be able to show that their 
impairments meet final listings 4.02B or 4.04A. Those who have not may 
still be evaluated under final listings 4.02A and C, and 4.04B and C, 
the listings that do not include exercise tests among their criteria.

4.09  Cardiac Transplantation

    Comment: One national medical association indicated that some 
individuals undergoing cardiac transplantation have recurring 
disability beyond 1 year. The commenter pointed out that serial 
observations and retesting must be continued.
    Response: Final listing 4.09 does not conflict with these facts. 
The listing provides that, after the year of disability, the individual 
must be reevaluated under whatever listing is appropriate to his or her 
residual cardiac impairment, where a finding of ``meets'' or ``equals'' 
may be possible.
    Moreover, under the medical improvement disability review standard 
that we are required to follow under Secs. 404.1594 and 416.994 (and 
Sec. 416.994a for children) we may not find that an individual's 
disability has ended ``automatically'' after a certain period of time. 
We are generally required to show that there has been medical 
improvement in the individual's disabling impairment, that the 
improvement is related to the ability to work, and that the individual 
is not currently under a disability. This means that, even if the 
individual's impairment has improved, we may still find that the 
individual continues to be disabled based on an assessment of residual 
functional capacity and consideration of vocational factors.

4.11  Chronic Venous Insufficiency

    Comment: One commenter thought that there were too many criteria 
required to meet listing 4.11. The commenter said that it should be 
unnecessary to require all five of the listed criteria in order to find 
a claimant disabled and that brawny edema alone should be sufficient 
for a finding of disability. Conversely, another commenter questioned 
whether this listing is too liberal, but did not say why.
    Response: We adopted the first comment. Extensive brawny edema is 
the most severe, end-stage manifestation of chronic venous 
insufficiency. However, we believe that because there can be 
variability in the severity of each of the other findings we proposed 
in listing 4.11, they must all be present together in order to 
establish listing-level severity based on chronic venous insufficiency 
that has not resulted in extensive brawny edema. We have, therefore, 
divided final listing 4.11 into two paragraphs. Final listing 4.11A 
will be met when there is extensive brawny edema resulting from deep 
venous insufficiency, while final listing 4.11B will be met with deep 
venous insufficiency with associated superficial varicosities, stasis 
dermatitis, and recurrent or persistent ulceration which has not healed 
following at least 3 months of prescribed medical or surgical therapy. 
We did not include a 3-month treatment requirement in final listing 
4.11A because extensive brawny edema is a sufficiently severe finding 
in itself that treatment is not an issue in establishing duration.
    We also reiterate for the first commenter that an individual does 
not have to demonstrate an impairment that ``meets'' a listing to be 
found disabled.
    Comment: One commenter suggested that we consider adding a 
statement about the evaluation of lower extremity pain without evidence 
of chronic venous insufficiency.
    Response: We did not adopt the comment. It was not clear to us from 
the comment what information the commenter was suggesting we provide. 
However, we have regulations for the evaluation of pain that we believe 
adequately cover the issue for all impairments.

4.12  Peripheral Arterial Disease

    Comment: One commenter suggested that we also require failure to 
visualize superficial arteries in listing 4.12A.
    Response: We did not adopt the comment. When the common femoral or 
deep femoral artery cannot be visualized, superficial arteries will not 
be visualized either. Therefore, such a requirement would be 
superfluous.
    Comment: Another commenter suggested that we add a third criterion, 
``ischemic pain at rest,'' to listing 4.12B.
    Response: We did not adopt the comment. Individuals with peripheral 
vascular disease who have ischemic pain at rest will have more severe 
impairments than are already described in final listing 4.12B1. Such 
individuals have resting ankle/brachial systolic blood pressures in the 
range of 0.30 to 0.40, which is even more severe than the listing 
requirement of 0.50.

104.00  Preface to Part B

104.00A  Introduction
    Comment: One commenter pointed out that the statement in the fifth 
paragraph of proposed 104.00A about age-appropriate activities for 
individuals age 14\1/2\ to 18 (i.e., that they ``are not unlike those 
of young adults'') was inconsistent with the interim final rules for 
evaluating childhood disability we had issued on February 11, 1991 
(``Determining Disability for a Child Under Age 18,'' 56 FR 5534).
    Response: We have deleted the entire paragraph. The rules for 
evaluating childhood function in Secs. 416.924 through 416.924e provide 
information on the evaluation of age-appropriate activities for 
children of all ages irrespective of the nature of their impairments. 
They are, therefore, also applicable to children with cardiovascular 
impairments.
104.00B  Documentation
    Comment: One commenter thought that the requirement in the second 
sentence of proposed 104.00B, which provided in part that a copy of 
relevant echocardiographic views ``must'' be included in the record, 
was unrealistic. The commenter pointed out that the State agency is not 
always able to obtain these copies. Therefore, the commenter 
recommended that we change the word ``must'' to ``should.''
    Response: We adopted the comment.
104.00D  Congenital Heart Disease
    Comment: A physician recommended that we eliminate the reference to 
patent ductus arteriosus in the fourth paragraph of proposed 104.00D 
because this condition is almost always amenable to surgical or medical 
therapy with very low morbidity.
    Response: We adopted the comment. Because of advances in the 
medical and surgical treatment of patent ductus arteriosus, infants 
born with this condition would not ordinarily be expected to experience 
chronic cardiopulmonary impairment. We have, therefore, deleted the 
paragraph. We have also clarified final 104.00 C and D so that they 
clearly indicate the kinds of conditions and the level of severity 
intended by the discussion. In addition, we have included guidance in 
final 104.00D for those conditions which, even though not named, 
nevertheless result in listing-level impairment in individual cases.
104.00E  Chronic Heart Failure
    Comment: A national medical association, noting our reference to 
``imaging techniques such as echocardiography and radionuclide 
studies'' in the last sentence of proposed 104.00E, pointed out that 
pediatric cardiologists almost never do radionuclide studies. The 
commenter suggested that we revise the last sentence to refer to other 
tests, such as two-dimensional and Doppler echocardiography.
    Response: We adopted the comment.

Listings

104.02  Chronic Heart Failure

    Comment: One attorney was concerned that our proposal to remove the 
tables for tachycardia and tachypnea from listing 104.02 would increase 
the severity level of the listing. The commenter thought that the 
exercise intolerance criterion was more restrictive in proposed listing 
104.02 than in former listing 104.02G. The commenter also thought that 
there was no justification for removal of chest x-ray evidence 
demonstrating cardiomegaly. The commenter was also concerned that 
hepatomegaly and edema were not included as criteria for this listing, 
even though they were in the prior listing. This commenter and another 
commenter noted that there were no criteria for fatigue and dyspnea in 
proposed listing 104.02.
    Response: Although the commenter was not correct in the belief that 
we had proposed to make the listing more stringent, we adopted most of 
the comments. Final listing 104.02A is now a criterion for persistent 
tachycardia at rest; final listing 104.02B is now a criterion for 
persistent tachypnea at rest or markedly decreased exercise tolerance; 
and we have included the corresponding tables from our former listings 
as Tables I and II at the end of the section. We moved the other 
criteria we had proposed in listing 104.02A into final 104.00E of the 
preface, and also added chronic dyspnea, tachypnea, and orthopnea to 
the list of symptoms (including fatigue and weakness) we had already 
included; we did not specifically mention all of them in the proposed 
listings because they are symptoms of chronic heart failure with which 
all physicians are familiar. Our proposed addition of the phrase 
``markedly reduced'' (now ``markedly decreased'' in the final rules) to 
modify the requirement for exercise tolerance was only a clarification; 
infants with labored respiration on mild exertion have markedly 
decreased exercise tolerance.
    The opinion of the commenter that there was no justification for 
our deletion of the rule for cardiomegaly demonstrated by x-ray was not 
correct. Our prior listing could be met only with the demonstration of 
two criteria associated with chronic heart failure. The proposed (and 
final) listing is the same, in that it requires as an initial matter 
the demonstration of findings--such as cardiomegaly--that confirm the 
existence of the impairment, and one other of the criteria in the 
subparagraphs. Our proposal to delete the requirement that cardiomegaly 
be demonstrated by x-ray was consistent with contemporary standards of 
care for infants and children with chronic heart failure, in which 
other, more accurate, imaging techniques, including echocardiography 
and other radionuclide studies, are generally used. Heart failure in 
children must be properly assessed; like cancer, it is not a diagnosis 
to be lightly entertained, and should not be based on an x-ray in a 
physician's office.
    However, we concede that not all claimants receive the proper care 
and that there will still occasionally be x-rays in the evidence 
without appropriate imaging techniques. Therefore, in response to the 
comment, we have restored our prior rules on x-ray evidence of heart 
enlargement demonstrated by cardiothoracic ratio in final 104.00E, with 
the provision that the findings must also be accompanied by other signs 
of chronic heart failure or ventricular dysfunction. Because we 
restored these provisions, we were also required to clarify how 
enlargement is evaluated using appropriate imaging techniques, such as 
echocardiography. We have not included the former requirements for 
enlargement on serial x-rays or for measurement of chamber size based 
on x-rays because these are outdated methods of assessment.
    The foregoing also explains why we removed the listing criteria for 
hepatomegaly and dependent edema. Like cardiomegaly, these are examples 
of medical findings that are used to confirm the existence of chronic 
heart failure, as described in 4.00E.
    With regard to the comments on dyspnea, we also added two new 
paragraphs to final 104.00B providing for the possibility of exercise 
testing in children. Exercise testing has been increasingly used in 
children age 6 and older for the assessment of some arrhythmias, for 
the assessment of the severity of chronic heart failure, and for the 
assessment of recovery of function following cardiac surgery or other 
therapy; however, it is still less frequently indicated for children 
than for adults and can rarely be successfully performed on children 
under age 6. We do not expect to purchase many such tests for children, 
but they may be useful in those few cases in which we are unable to 
obtain sufficient evidence about a child's functioning (for instance, 
from treating sources and schools) to make a decision. The first 
paragraph, therefore, provides that we may purchase the test only if we 
cannot decide the case based on the available evidence; therefore, it 
cannot be used to disadvantage any child claimant. The paragraph also 
requires that the testing must be performed in a specialty center for 
pediatric cardiology or other facility qualified to perform exercise 
testing for children.
    The second paragraph parallels the adult rules by requiring that 
such testing be performed using a generally accepted protocol 
consistent with the prevailing state of medical knowledge and clinical 
practice. In addition, it prohibits the purchase of the test for a 
child for whom the performance of the test constitutes a significant 
risk and adopts by reference the risk provisions in the adult rules, 
which are also applicable to children.

104.03  Hypertensive Cardiovascular Disease

    Comment: The same attorney commenter thought that the table for 
elevated blood pressure in listing 104.03 established a higher level of 
severity in the listings for children. The commenter also noted that we 
had not provided any explanation in the preamble to the NPRM of our 
reasons for making the change.
    Response: As we explained in the preamble to the NPRM, Table III in 
final listing 104.03 comprises up-to-date values that are recommended 
by the American Academy of Pediatrics (56 FR 31269). The table is taken 
from the definition of ``Significant Hypertension'' in Table 5 of the 
``Guidelines of the American Academy of Pediatrics; Report of the 2d 
Task Force on Blood Pressure Control in Children--1987,'' 
``Pediatrics,'' 1987; 79:1-25. These guidelines should be in every 
pediatrician's office.
    The levels in Table III are equal to or greater than the 95th 
percentile for age. In response to the comment, we have added this 
explanation to the opening of the final listing.
    In most instances, the readings in final Table III are not more 
severe than those on the former table. We also provide more refined age 
category breaks. In any event, no child will be disadvantaged by our 
updating of the rule inasmuch as, to meet this listing, a child must 
also show another, consequent listing-level impairment, as in the 
former listing. Children whose blood pressures do not rise to the 
levels on the table but who have listing-level kidney, cerebrovascular, 
or heart impairment will still be found disabled at the listing level 
by virtue of their secondary impairments.

104.05  Recurrent Arrhythmias

    Comment: The same commenter thought that our proposal in 104.05A to 
require that any episode of cardiac syncope be coincident with the 
documented arrhythmia, was a new requirement.
    Response: As we explained under the comments about the 
corresponding adult rule, listing 4.05, the proposed language merely 
clarified what is standard medical practice. In order for cardiac 
syncope to be related to the arrhythmia, it must be coincident with the 
arrhythmia. In response to the comment, we have also added a new second 
sentence to the second paragraph of final 4.00B, ``Documentation,'' 
providing procedural guidance on when the purchase of an ambulatory ECG 
may be appropriate.
    Comment: The same commenter thought that by cross-referencing the 
chronic heart failure listing in proposed listing 104.05B while 
omitting the exercise intolerance criteria in former listing 104.05C, 
we were suggesting that even a claimant meeting the more exacting 
stricture of the listing 104.02A exercise intolerance test would still 
be found not to meet the arrhythmia listing unless he or she also 
suffered from chronic heart failure. The commenter felt that an 
explicit exercise criterion should be included in the arrhythmia 
listing.
    Response: We do not believe it is necessary to add the explicit 
exercise criterion to 104.05. Cardiac arrhythmias are common in 
children, and most childhood arrhythmias are essentially benign. When a 
child with a cardiac arrhythmia develops exercise intolerance with 
labored respirations on mild exertion, this is usually indicative of 
chronic heart failure. This is why we provided the cross-reference to 
listing 104.02 instead of incorporating the criteria from listing 
104.02 into the listing on arrhythmia, which is what the prior listing 
essentially did. In addition, there are other criteria associated with 
arrhythmias in childhood, such as syncope or near syncope, which are 
more frequent manifestations of severe cardiac arrhythmias and which 
may not be necessarily associated with congestive heart failure.
    However, in response to the comment, and because we have tried in 
these final rules to maintain consistency between part A and part B 
wherever possible and appropriate, we have revised listings 104.02 and 
104.05. We have removed the reference to listing 104.02 from the 
arrhythmia listing and have instead added a criterion for arrhythmia 
under final listing 104.02C, by which that listing can now be met; this 
better describes our original intent than did proposed listing 104.05B. 
In addition, we revised the remaining language in final listing 104.05 
into a single paragraph that is more consistent with the adult rules, 
as explained in the summary of provisions.
    Comment: A medical association recommended that we add nonsyncopal 
arrhythmia to listing 104.05. Another commenter questioned why we did 
not mention A-V dissociation in the proposed listing; it was a 
criterion in the heading of the former listing.
    Response: In response to the comment, we have revised final listing 
104.05 to include near syncopal episodes clearly documented to be 
associated with arrhythmia. This is also consistent with our addition 
of the term to final listing 4.05.
    We did not actually delete A-V dissociation from the listing. A-V 
dissociation is a kind of heart block and is, therefore, implicit in 
the term ``heart block.'' We, therefore, proposed to delete the term 
because the prior listing language was ambiguous: The use of the 
conjunctive, ``or,'' in the former rules did not mean that A-V 
dissociation was a separate medical entity from heart block; it meant 
that A-V dissociation was another way of describing a heart block. 
However, so that there is no misunderstanding, we have restored the 
term parenthetically after ``heart block'' in the final listing.

104.06  Congenital Heart Disease

    Comment: A physician questioned our approach in proposed listing 
104.06A (final listing 104.06H). The commenter pointed out that the 
proposed rule to consider all children who meet the requirements of the 
listing to be disabled until age 24 months would result in inequitable 
outcomes. For instance, a child who had life-saving surgery at age 3 
months would in effect be found disabled for at least 21 months, 
whereas a child who was found disabled under this listing at age 11 
months would be found disabled for only 13 months. The commenter 
suggested that a more equitable approach would be to provide a recovery 
time period instead of an age cutoff.
    Response: We adopted the comment, which actually conveyed our 
original intent. We have revised the final rule to state that we will 
consider an infant who meets the requirements of the listing to be 
disabled until the attainment of age 1, or for 12 months after surgery, 
whichever is later, and then reevaluate. We believe that 12 months is a 
sufficient period for stabilization and developmental recovery and, in 
this manner, all infants will be treated in the same way under the 
listing. We tied the recovery time period to surgery because the 
listing is for children who are expected to die within the first year 
of life without surgical intervention. If by age 1 an infant has not 
had surgery and has survived, the child should be reevaluated because 
the impairment may no longer be as severe as it originally appeared. 
Even if it is of listing-level severity at age 1, we should be able to 
evaluate it under one of the other listings; if it is less severe than 
listing-level, we will perform an individualized functional assessment 
and determine whether the child is still disabled.
    In addition, we believe that the comment also required us to revise 
the period described in proposed listing 104.09, ``Cardiac 
transplantation,'' because it used the same language. Indeed, proposed 
listing 104.09 could have been viewed as being even more inequitable. 
This is because, under the proposed rule, all children over age 1 would 
have received a period of 12 months (because a 12-month period for a 
child over 12 months old would always end later than age 24 months), 
whereas all children under age 1 would always receive a period of more 
than 12 months, some for nearly 2 years, others for closer to 1 year. 
In sum, we believe that the revisions to final listings 104.06H and 
104.09 are simply fairer.
    Comment: A commenter indicated that squatting, which was a 
criterion in former listing 104.04B, was not included in listing 
104.06, the corresponding listing in these rules, and questioned its 
absence.
    Response: We proposed to delete squatting because it is now a rare 
finding. In the past, squatting was usually seen in older surviving 
children with uncorrected cardiac defects associated with dyspnea and 
cyanosis at rest. Because the majority of children with cyanotic forms 
of congenital heart disease are now undergoing corrective surgery 
within the first year of life to restore oxygenation at more 
physiological levels, squatting is rarely seen. Nonetheless, to 
encompass the rare situation in which squatting may be present, we have 
restored it as a criterion under final listing 104.06A3.
    Comment: A national medical association thought that the four 
manifestations of persistent, chronic hypoxemia we proposed in listing 
104.06B might be too rigid. As an example, it said that a hematocrit of 
55 percent or greater could create a problem for children who are 
anemic, and suggested that the qualifier ``in the absence of documented 
anemia'' should be added to the criterion. An attorney noted that 
former listing 104.04C required a ``chronic'' hematocrit of 55 percent 
or greater, whereas proposed listing 104.06B2 specified a hematocrit of 
55 percent or greater ``on two or more evaluations within a 3-month 
period.'' The commenter thought that this was a more stringent 
requirement than the one in the former listings, called it a ``hidden'' 
change in the listings, and strongly objected to it.
    Response: We did not adopt the comments. Other than providing the 
example of anemia, the first commenter did not provide any other 
explanation for the belief that the four criteria in final listing 
104.06A are too rigid. We believe that anemic and hypoxic children will 
be covered by final listing 104.06A1 because the arterial oxygen 
saturation should be significantly reduced as a result of anemia. The 
requirement that hematocrit results at 55 percent or greater be 
obtained twice or more within a 3-month period is consistent with 
medical practice in children, is manifestly a more precise programmatic 
definition of what is meant by the term ``chronic'' hematocrit of 55 
percent or greater, and is not intended as a change in severity level. 
Moreover, we proposed the change through proper notice-and-comment 
rulemaking procedures and received no negative comments about the 
proposal aside from this lay comment.
    Comment: The same attorney commenter from the preceding comment 
noted our statement in the preamble of the NPRM that we proposed to 
delete former listing 104.08, ``Recurrent hemoptysis,'' because the 
condition could be evaluated under proposed listing 104.06E (56 FR 
31269). The commenter said that, in fact, we had not mentioned 
hemoptysis (or bronchial collaterals, another criterion in the former 
listing) in proposed listing 104.06E. Another commenter asked whether 
nonvalvular stenosis should have been included in proposed listing 
104.06F.
    Response: We did not adopt the first comment. Hemoptysis is an 
uncommon manifestation of cardiovascular disease which occurs only in 
the presence of cyanotic heart disease or pulmonary hypertension of 
such seriousness that the primary condition would already have met or 
equaled the listings. Under final listing 104.06D, a child does not 
have to have an impairment so severe that it results in hemoptysis. 
Because of this fact, we have also deleted the criterion for recurrent 
hemoptysis in final listing 104.06A3; this, too, describes an 
impairment that exceeds listing-level severity.
    We deleted the reference to bronchial collaterals for the same 
reason. Major collaterals (which generally are shown by cardiac 
catheterization) are only present in infants and children with severe 
cyanosis, occasionally combined with congestive failure, and such 
children will already have evidence that their impairments meet or are 
equivalent to one of the listings.
    We adopted the second comment because we did intend to include 
nonvalvular stenotic lesions under this listing. We, therefore, revised 
final listing 104.06E (which was proposed listing 104.06F) to include 
congenital valvular or other stenotic defects, or valvular 
regurgitation. For consistency, we made similar revisions in final 
listings 4.07 and 104.07.

104.13  Chronic Rheumatic Fever or Rheumatic Heart Disease

    Comment: Another commenter noted that proposed listing 104.13 was 
essentially the same as prior listing 104.09A but that we had not 
included ECG findings as an example of an abnormal laboratory finding, 
as in the former listing.
    Response: We have restored the example of abnormal ECG findings to 
final listing 104.13A. We had proposed to delete the example only 
because it is a minor criterion; it is not a specific requirement that 
abnormal ECG findings be provided in order to meet or equal the 
listing. The elevated sedimentation rate and abnormal ECG findings are 
only two of several minor criteria that can be used in establishing the 
diagnosis of continuing rheumatic activity. The use of elevated 
sedimentation rate to the exclusion of the ECG and other minor criteria 
was not intended to preclude the use or substitution of these other 
criteria for meeting the requirement that rheumatic activity be 
manifested by abnormal laboratory findings.

104.15  Kawasaki Syndrome

    Comment: A national medical association suggested that we include a 
separate listing for Kawasaki syndrome. The commenter said that we 
should mention specifically the anatomical and arterial findings 
associated with the disorder and the chronic sequelae, including 
coronary lesions or valvular involvement.
    Response: We have added a new listing 104.15 for Kawasaki syndrome. 
The listing will be met with Kawasaki syndrome associated with a major 
coronary artery aneurysm or with chronic heart failure of the level 
described in listing 104.02. The current treatment of Kawasaki syndrome 
with immunoglobulin therapy is thought to reduce the risk of the two 
major cardiac sequelae, coronary artery aneurysms and ventricular 
dysfunction. Listing-level impairment from either of these causes is 
rare, probably on the order of less than 5 percent of all infants 
affected, and it is unusual for the syndrome to be a cause of chronic 
cardiovascular impairment. However, in those instances in which such 
sequelae occur, other listings (such as listing 104.07, for valvular 
and other stenotic lesions) may be used for evaluation.
    Accordingly, with the aforementioned revisions, the regulations are 
adopted.

Regulatory Procedures

Executive Order 12291

    The Secretary has determined that this is not a major rule under 
Executive Order 12291 because implementation will not result in 
additional costs of $100 million and other threshold criteria for a 
major rule are not met. Therefore, a regulatory impact analysis is not 
required.

Paperwork Reduction Act

    These final regulations will impose no new reporting or 
recordkeeping requirements subject to clearance by the Office of 
Management and Budget.

Regulatory Flexibility Act

    We certify that these final regulations, if promulgated, will not 
have a significant economic impact on a substantial number of small 
entities because they primarily affect only disability claimants and 
beneficiaries under title II and title XVI of the Social Security Act.

(Catalog of Federal Domestic Assistance Program No. 93.802, 
Disability Insurance; No. 93.807)

List of Subjects in 20 CFR Part 404

    Administrative practice and procedure, Blind, Disability benefits, 
Old-Age, Survivors and Disability Insurance, Reporting and 
recordkeeping requirements, Social Security.


    Dated: April 8, 1993.

Louis D. Enoff,
Principal Deputy Commissioner of Social Security.

    Approved: October 22, 1993.

Donna E. Shalala,
Secretary of Health and Human Services.

    For the reasons set out in the preamble, part 404 of Chapter III of 
title 20 of the Code of Federal Regulations is amended as follows:

PART 404--FEDERAL OLD-AGE, SURVIVORS AND DISABILITY INSURANCE 
(1950-________)

Subpart P--[Amended]

    1. The authority citation for subpart P continues to read as 
follows:

    Authority: Secs. 202, 205 (a), (b), and (d) through (h), 216(i), 
221 (a) and (i), 222(c), 223, 225, and 1102 of the Social Security 
Act; 42 U.S.C. 402, 405 (a), (b), and (d) through (h), 416(i), 421 
(a) and (i), 422(c), 423, 425, and 1302.

    2. Appendix 1 to subpart P is amended by revising item 5 of the 
introductory text before part A to read as follows:

Appendix 1 to Subpart P--Listing of Impairments

* * * * *
    5. The cardiovascular system listings (4.00 and 104.00) within 4 
years. Consequently, the listings in this body system will no longer be 
effective on February 10, 1994.
* * * * *
    3. Listing 4.00, Cardiovascular System, of part A of appendix 1 to 
subpart P of part 404 is revised to read as follows:

4.00  Cardiovascular System

    A. Introduction. The listings in this section describe 
impairments resulting from cardiovascular disease based on symptoms, 
physical signs, laboratory test abnormalities, and response to a 
regimen of therapy prescribed by a treating source. A longitudinal 
clinical record covering a period of not less than 3 months of 
observations and therapy is usually necessary for the assessment of 
severity and expected duration of cardiovascular impairment, unless 
the claim can be decided favorably on the basis of the current 
evidence. All relevant evidence must be considered in assessing 
disability.
    Many individuals, especially those who have listing-level 
impairments, will have received the benefit of medically prescribed 
treatment. Whenever there is evidence of such treatment, the 
longitudinal clinical record must include a description of the 
therapy prescribed by the treating source and response, in addition 
to information about the nature and severity of the impairment. It 
is important to document any prescribed therapy and response because 
this medical management may have improved the individual's 
functional status. The longitudinal record should provide 
information regarding functional recovery, if any.
    Some individuals will not have received ongoing treatment or 
have an ongoing relationship with the medical community despite the 
existence of a severe impairment(s). Unless the claim can be decided 
favorably on the basis of the current evidence, a longitudinal 
record is still important because it will provide information about 
such things as the ongoing medical severity of the impairment, the 
degree of recovery from cardiac insult, the level of the 
individual's functioning, and the frequency, severity, and duration 
of symptoms. Also, several listings include a requirement for 
continuing signs and symptoms despite a regimen of prescribed 
treatment. Even though an individual who does not receive treatment 
may not be able to show an impairment that meets the criteria of 
these listings, the individual may have an impairment(s) equivalent 
in severity to one of the listed impairments or be disabled because 
of a limited residual functional capacity.
    Indeed, it must be remembered that these listings are only 
examples of common cardiovascular disorders that are severe enough 
to prevent a person from engaging in gainful activity. Therefore, in 
any case in which an individual has a medically determinable 
impairment that is not listed, or a combination of impairments no 
one of which meets a listing, we will make a medical equivalence 
determination. Individuals who have an impairment(s) with a level of 
severity which does not meet or equal the criteria of the 
cardiovascular listings may or may not have the residual functional 
capacity (RFC) which would enable them to engage in substantial 
gainful activity. Evaluation of the impairment(s) of these 
individuals should proceed through the final steps of the sequential 
evaluation process (or, as appropriate, the steps in the medical 
improvement review standard).
    B. Cardiovascular impairment results from one or more of four 
consequences of heart disease:
    1. Chronic heart failure or ventricular dysfunction.
    2. Discomfort or pain due to myocardial ischemia, with or 
without necrosis of heart muscle.
    3. Syncope, or near syncope, due to inadequate cerebral 
perfusion from any cardiac cause such as obstruction of flow or 
disturbance in rhythm or conduction resulting in inadequate cardiac 
output.
    4. Central cyanosis due to right-to-left shunt, arterial 
desaturation, or pulmonary vascular disease.
    Impairment from diseases of arteries and veins may result from 
disorders of the vasculature in the central nervous system (11.04A, 
B), eyes (2.02-2.04), kidney (6.02), and other organs.
    C. Documentation. Each individual's file must include 
sufficiently detailed reports on history, physical examinations, 
laboratory studies, and any prescribed therapy and response to allow 
an independent reviewer to assess the severity and duration of the 
cardiovascular impairment.

1. Electrocardiography

    a. An original or legible copy of the 12-lead electrocardiogram 
(ECG) obtained at rest must be submitted, appropriately dated and 
labeled, with the standardization inscribed on the tracing. 
Alteration in standardization of specific leads (such as to 
accommodate large QRS amplitudes) must be identified on those leads.
    (1) Detailed descriptions or computer-averaged signals without 
original or legible copies of the ECG as described in subsection 
4.00Cla are not acceptable.
    (2) The effects of drugs or electrolyte abnormalities must be 
considered as possible noncoronary causes of ECG abnormalities of 
ventricular repolarization, i.e., those involving the ST segment and 
T wave. If available, the predrug (especially digitalis glycoside) 
ECG should be submitted.
    (3) The term ``ischemic'' is used in 4.04A to describe an 
abnormal ST segment deviation. Nonspecific repolarization 
abnormalities should not be confused with ``ischemic'' changes.
    b. ECGs obtained in conjunction with treadmill, bicycle, or arm 
exercise tests should meet the following specifications:
    (1) ECGs must include the original calibrated ECG tracings or a 
legible copy.
    (2) A 12-lead baseline ECG must be recorded in the upright 
position before exercise.
    (3) A 12-lead ECG should be recorded at the end of each minute 
of exercise, including at the time the ST segment abnormalities 
reach or exceed the criteria for abnormality described in 4.04A or 
the individual experiences chest discomfort or other abnormalities, 
and also when the exercise test is terminated.
    (4) If ECG documentation of the effects of hyperventilation is 
obtained, the exercise test should be deferred for at least 10 
minutes because metabolic changes of hyperventilation may alter the 
physiologic and ECG response to exercise.
    (5) Post-exercise ECGs should be recorded using a generally 
accepted protocol consistent with the prevailing state of medical 
knowledge and clinical practice.
    (6) All resting, exercise, and recovery ECG strips must have a 
standardization inscribed on the tracing. The ECG strips should be 
labeled to indicate the times recorded and the relationship to the 
stage of the exercise protocol. The speed and grade (treadmill test) 
or work rate (bicycle or arm ergometric test) should be recorded. 
The highest level of exercise achieved, blood pressure levels during 
testing, and the reason(s) for terminating the test (including 
limiting signs or symptoms) must be recorded.

2. Purchasing Exercise Tests

    a. It is well recognized by medical experts that exercise 
testing is the best tool currently available for estimating maximal 
aerobic capacity in individuals with cardiovascular impairments. 
Purchase of an exercise test may be appropriate when there is a 
question whether an impairment meets or is equivalent in severity to 
one of the listings, or when there is insufficient evidence in the 
record to evaluate aerobic capacity, and the claim cannot otherwise 
be favorably decided. Before purchasing an exercise test, a program 
physician, preferably one with experience in the care of patients 
with cardiovascular disease, must review the pertinent history, 
physical examinations, and laboratory tests to determine whether 
obtaining the test would present a significant risk to the 
individual (see 4.00C2c). Purchase may be indicated when there is no 
significant risk to exercise testing and there is no timely test of 
record. An exercise test is generally considered timely for 12 
months after the date performed, provided there has been no change 
in clinical status that may alter the severity of the cardiac 
impairment.
    b. Methodology.
    (1) When an exercise test is purchased, it should be a ``sign-or 
symptom-limited'' test characterized by a progressive multistage 
regimen. A purchased exercise test must be performed using a 
generally accepted protocol consistent with the prevailing state of 
medical knowledge and clinical practice. A description of the 
protocol that was followed must be provided, and the test must meet 
the requirements of 4.00C1b and this section. A pre-exercise 
posthyperventilation tracing may be essential for the proper 
evaluation of an ``abnormal'' test in certain circumstances, such as 
in women with evidence of mitral valve prolapse.
    (2) The exercise test should be paced to the capabilities of the 
individual and be supervised by a physician. With a treadmill test, 
the speed, grade (incline) and duration of exercise must be recorded 
for each exercise test stage performed. Other exercise test 
protocols or techniques that are used should utilize similar 
workloads.
    (3) Levels of exercise should be described in terms of workload 
and duration of each stage, e.g., treadmill speed and grade, or 
bicycle ergometer work rate in kpm/min or watts.
    (4) Normally, systolic blood pressure and heart rate increase 
gradually with exercise. A decrease in systolic blood pressure 
during exercise below the usual resting level is often associated 
with ischemia-induced left ventricular dysfunction resulting in 
decreased cardiac output. Some individuals (because of 
deconditioning or apprehension) with increased sympathetic responses 
may increase their systolic blood pressure and heart rate above 
their usual resting level just before and early into exercise. This 
occurrence may limit the ability to assess the significance of an 
early decrease in systolic blood pressure and heart rate if exercise 
is discontinued shortly after initiation. In addition, isolated 
systolic hypertension may be a manifestation of arteriosclerosis.
    (5) The exercise laboratory's physical environment, staffing, 
and equipment should meet the generally accepted standards for adult 
exercise test laboratories.
    c. Risk factors in exercise testing. The following are examples 
of situations in which exercise testing will not be purchased: 
unstable progressive angina pectoris, a history of acute myocardial 
infarction within the past 3 months, New York Heart Association 
(NYHA) class IV heart failure, cardiac drug toxicity, uncontrolled 
serious arrhythmia (including uncontrolled atrial fibrillation, 
Mobitz II, and third-degree block), Wolff-Parkinson-White syndrome, 
uncontrolled severe systemic arterial hypertension, marked pulmonary 
hypertension, unrepaired aortic dissection, left main stenosis of 50 
percent or greater, marked aortic stenosis, chronic or dissecting 
aortic aneurysm, recent pulmonary embolism, hypertrophic 
cardiomyopathy, limiting neurological or musculoskeletal 
impairments, or an acute illness. In addition, an exercise test 
should not be purchased for individuals for whom the performance of 
the test is considered to constitute a significant risk by a program 
physician, preferably one experienced in the care of patients with 
cardiovascular disease, even in the absence of any of the above risk 
factors. In defining risk, the program physician, in accordance with 
the regulations and other instructions on consultative examinations, 
will generally give great weight to the treating physicians' 
opinions and will generally not override them. In the rare situation 
in which the program physician does override the treating source's 
opinion, a written rationale must be prepared documenting the 
reasons for overriding the opinion.
    d. In order to permit maximal, attainable restoration of 
functional capacity, exercise testing should not be purchased until 
3 months after an acute myocardial infarction, surgical myocardial 
revascularization, or other open-heart surgical procedures. Purchase 
of an exercise test should also be deferred for 3 months after 
percutaneous transluminal coronary angioplasty because restenosis 
with ischemic symptoms may occur within a few months of angioplasty 
(see 4.00D). Also, individuals who have had a period of bedrest or 
inactivity (e.g., 2 weeks) that results in a reversible 
deconditioned state may do poorly if exercise testing is performed 
at that time.
    e. Evaluation.
    (1) Exercise testing is evaluated on the basis of the work level 
at which the test becomes abnormal, as documented by onset of signs 
and symptoms and any ECG abnormalities listed in 4.04A. The ability 
or inability to complete an exercise test is not, by itself, 
evidence that a person is free from ischemic heart disease. The 
results of an exercise test must be considered in the context of all 
of the other evidence in the individual's case record. If the 
individual is under the care of a treating physician for a cardiac 
impairment, and this physician has not performed an exercise test 
and there are no reported significant risks to testing (see 
4.00C2c), a statement should be requested from the treating 
physician explaining why it was not done or should not be done 
before deciding whether an exercise test should be purchased. In 
those rare situations in which the treating source's opinion is 
overridden, follow 4.00C2c. If there is no treating physician, the 
program physician will be responsible for assessing the risk to 
exercise testing.
    (2) Limitations to exercise test interpretation include the 
presence of noncoronary or nonischemic factors that may influence 
the hemodynamic and ECG response to exercise, such as hypokalemia or 
other electrolyte abnormality, hyperventilation, vasoregulatory 
deconditioning, prolonged periods of physical inactivity (e.g., 2 
weeks of bedrest), significant anemia, left bundle branch block 
pattern on the ECG (and other conduction abnormalities that do not 
preclude the purchase of exercise testing), and other heart diseases 
or abnormalities (particularly valvular heart disease). Digitalis 
glycosides may cause ST segment abnormalities at rest, during, and 
after exercise. Digitalis or other drug-related ST segment 
displacement, present at rest, may become accentuated with exercise 
and make ECG interpretation difficult, but such drugs do not 
invalidate an otherwise normal exercise test. Diuretic-induced 
hypokalemia and left ventricular hypertrophy may also be associated 
with repolarization changes and behave similarly. Finally, treatment 
with beta blockers slows the heart rate more at near-maximal 
exertion than at rest; this limits apparent chronotropic capacity.

3. Other Studies

    Information from two-dimensional and Doppler echocardiographic 
studies of ventricular size and function as well as radionuclide 
(thallium201) myocardial ``perfusion'' or radionuclide 
(technetium 99m) ventriculograms (RVG or MUGA) may be useful. These 
techniques can provide a reliable estimate of ejection fraction. In 
selected cases, these tests may be purchased after a medical history 
and physical examination, report of chest x-rays, ECGs, and other 
appropriate tests have been evaluated, preferably by a program 
physician with experience in the care of patients with 
cardiovascular disease. Purchase should be considered when other 
information available is not adequate to assess whether the 
individual may have severe ventricular dysfunction or myocardial 
ischemia and there is no significant risk involved (follow 4.00C2a 
guides), and the claim cannot be favorably decided on any other 
basis.
    Exercise testing with measurement of maximal oxygen uptake 
(VO2) provides an accurate determination of aerobic capacity. 
An exercise test without measurement of oxygen uptake provides an 
estimate of aerobic capacity. When the results of tests with 
measurement of oxygen uptake are available, every reasonable effort 
should be made to obtain them.
    The recording of properly calibrated ambulatory ECGs for 
analysis of ST segment signals with a concomitantly recorded symptom 
and treatment log may permit more adequate evaluation of chest 
discomfort during activities of daily living, but the significance 
of these data for disability evaluation has not been established in 
the absence of symptoms (e.g., silent ischemia). This information 
(including selected segments of both the ECG recording and summary 
report of the patient diary) may be submitted for the record.
    4. Cardiac catheterization will not be purchased by the Social 
Security Administration.
    a. Coronary arteriography. If results of such testing are 
available, the report should be obtained and considered as to the 
quality and type of data provided and its relevance to the 
evaluation of the impairment. A copy of the report of the cardiac 
catheterization and ancillary studies should also be obtained. The 
report should provide information citing the method of assessing 
coronary arterial lumen diameter and the nature and location of 
obstructive lesions. Drug treatment at baseline and during the 
procedure should be reported. Coronary artery spasm induced by 
intracoronary catheterization is not to be considered evidence of 
ischemic disease. Some individuals with significant coronary 
atherosclerotic obstruction have collateral vessels that supply the 
myocardium distal to the arterial obstruction so that there is no 
evidence of myocardial damage or ischemia, even with exercise. When 
available, quantitative computer measurements and analyses should be 
considered in the interpretation of severity of stenotic lesions.
    b. Left ventriculography (by angiography). The report should 
describe the wall motion of the myocardium with regard to any areas 
of hypokinesis, akinesis, or dyskinesis, and the overall contraction 
of the ventricle as measured by the ejection fraction. Measurement 
of chamber volumes and pressures may be useful. When available, 
quantitative computer analysis provides precise measurement of 
segmental left ventricular wall thickness and motion. There is often 
a poor correlation between left ventricular function at rest and 
functional capacity for physical activity.
    D. Treatment and relationship to functional status.
    1. In general, conclusions about the severity of a 
cardiovascular impairment cannot be made on the basis of type of 
treatment rendered or anticipated. The overall clinical and 
laboratory evidence, including the treatment plan(s) or results, 
should be persuasive that a listing-level impairment exists. The 
amount of function restored and the time required for improvement 
after treatment (medical, surgical, or a prescribed program of 
progressive physical activity) vary with the nature and extent of 
the disorder, the type of treatment, and other factors. Depending 
upon the timing of this treatment in relation to the alleged onset 
date of disability, impairment evaluation may need to be deferred 
for a period of up to 3 months from the date of treatment to permit 
consideration of treatment effects. Evaluation should not be 
deferred if the claim can be favorably decided based upon the 
available evidence.
    2. The usual time after myocardial infarction, valvular and/or 
revascularization surgery for adequate assessment of the results of 
treatment is considered to be 3 months. If an exercise test is 
performed by a treating source within a week or two after 
angioplasty, and there is no significant change in clinical status 
during the 3-month period after the angioplasty that would 
invalidate the implications of the exercise test results, the 
exercise test results may be used to reflect functional capacity 
during the period in question. However, if the test was done 
immediately following an acute myocardial infarction or during a 
period of protracted inactivity, the results should not be projected 
to 3 months even if there is no change in clinical status.
    3. An individual who has undergone cardiac transplantation will 
be considered under a disability for 1 year following the surgery 
because, during the first year, there is a greater likelihood of 
rejection of the organ and recurrent infection. After the first year 
posttransplantation, continuing disability evaluation will be based 
upon residual impairment as shown by symptoms, signs, and laboratory 
findings. Absence of symptoms, signs, and laboratory findings 
indicative of cardiac dysfunction will be included in the 
consideration of whether medical improvement (as defined in 
Secs. 404.1579(b)(1) and (c)(1), 404.1594(b)(1) and (c)(1), or 
416.994(b)(1)(i) and (b)(2)(i), as appropriate) has occurred.
    E. Clinical syndromes.
    1. Chronic heart failure (ventricular dysfunction) is considered 
in these listings as one category whatever its etiology, i.e., 
atherosclerotic, hypertensive, rheumatic, pulmonary, congenital or 
other organic heart disease. Chronic heart failure may manifest 
itself by:
    a. Pulmonary or systemic congestion, or both; or
    b. Symptoms of limited cardiac output, such as weakness, 
fatigue, or intolerance of physical activity.
    For the purpose of 4.02A, pulmonary and systemic congestion are 
not considered to have been established unless there is or has been 
evidence of fluid retention, such as hepatomegaly or ascites, or 
peripheral or pulmonary edema of cardiac origin. The findings of 
fluid retention need not be present at the time of adjudication 
because congestion may be controlled with medication. Chronic heart 
failure due to limited cardiac output is not considered to have been 
established for the purpose of 4.02B unless symptoms occur with 
ordinary daily activities, i.e., activity restriction as manifested 
by a need to decrease activity or pace, or to rest intermittently, 
and are associated with one or more physical signs or abnormal 
laboratory studies listed in 4.02B. These studies include exercise 
testing with ECG and blood pressure recording and/or appropriate 
imaging techniques, such as two-dimensional echocardiography or 
radionuclide or contrast ventriculography. The exercise criteria are 
outlined in 4.02B1. In addition, other abnormal symptoms, signs, or 
laboratory test results that lend credence to the impression of 
ventricular dysfunction should be considered.
    2. For the purposes of 4.03, hypertensive cardiovascular disease 
is evaluated by reference to the specific organ system involved 
(heart, brain, kidneys, or eyes). The presence of organic impairment 
must be established by appropriate physical signs and laboratory 
test abnormalities as specified in 4.02 or 4.04, or for the body 
system involved.
    3. Ischemic (coronary) heart disease may result in an impairment 
due to myocardial ischemia and/or ventricular dysfunction or 
infarction. For the purposes of 4.04, the clinical determination 
that discomfort of myocardial ischemic origin (angina pectoris) is 
present must be supported by objective evidence as described under 
4.00Cl, 2, 3, or 4.
    a. Discomfort of myocardial ischemic origin (angina pectoris) is 
discomfort that is precipitated by effort and/or emotion and 
promptly relieved by sublingual nitroglycerin, other rapidly acting 
nitrates, or rest. Typically the discomfort is located in the chest 
(usually substernal) and described as crushing, squeezing, burning, 
aching, or oppressive. Sharp, sticking, or cramping discomfort is 
considered less common or atypical. Discomfort occurring with 
activity or emotion should be described specifically as to timing 
and usual inciting factors (type and intensity), character, 
location, radiation, duration, and response to nitrate therapy or 
rest.
    b. So-called anginal equivalent may be localized to the neck, 
jaw(s), or hand(s) and has the same precipitating and relieving 
factors as typical chest discomfort. Isolated shortness of breath 
(dyspnea) is not considered an anginal equivalent for purposes of 
adjudication.
    c. Variant angina of the Prinzmetal type, i.e., rest angina with 
transitory ST segment elevation on ECG, may have the same 
significance as typical angina, described in 4.00E3a.
    d. If there is documented evidence of silent ischemia or 
restricted activity to prevent chest discomfort, this information 
must be considered along with all available evidence to determine if 
an equivalence decision is appropriate.
    e. Chest discomfort of myocardial ischemic origin is usually 
caused by coronary artery disease. However, ischemic discomfort may 
be caused by noncoronary artery conditions, such as critical aortic 
stenosis, hypertrophic cardiomyopathy, pulmonary hypertension, or 
anemia. These conditions should be distinguished from coronary 
artery disease, because the evaluation criteria, management, and 
prognosis (duration) may differ from that of coronary artery 
disease.
    f. Chest discomfort of nonischemic origin may result from other 
cardiac conditions such as pericarditis and mitral valve prolapse. 
Noncardiac conditions may also produce symptoms mimicking that of 
myocardial ischemia. These conditions include gastrointestinal tract 
disorders, such as esophageal spasm, esophagitis, hiatal hernia, 
biliary tract disease, gastritis, peptic ulcer, and pancreatitis, 
and musculoskeletal syndromes, such as chest wall muscle spasm, 
chest wall syndrome (especially after coronary bypass surgery), 
costochondritis, and cervical or dorsal arthritis. Hyperventilation 
may also mimic ischemic discomfort. Such disorders should be 
considered before concluding that chest discomfort is of myocardial 
ischemic origin.

4. Peripheral Arterial Disease

    The level of impairment is based on the symptomatology, physical 
findings, Doppler studies before and after a standard exercise test, 
or angiographic findings.
    The requirements for evaluating peripheral arterial disease in 
4.12B are based on the ratio of the systolic blood pressure at the 
ankle to the systolic blood pressure at the brachial artery, 
determined in the supine position at the same time. Techniques for 
obtaining ankle systolic blood pressures include Doppler, 
plethysmographic studies, or other techniques.
    Listing 4.12B1 is met when the resting ankle/brachial systolic 
blood pressure ratio is less than 0.50. Listing 4.12B2 provides 
additional criteria for evaluating peripheral arterial impairment on 
the basis of exercise studies when the resting ankle/brachial 
systolic blood pressure ratio is 0.50 or above. The decision to 
obtain exercise studies should be based on an evaluation of the 
existing clinical evidence, but exercise studies are rarely 
warranted when the resting ankle-over-brachial systolic blood 
pressure ratio is 0.80 or above. The results of exercise studies 
should describe the level of exercise, e.g., speed and grade of the 
treadmill settings, the duration of exercise, symptoms during 
exercise, the reasons for stopping exercise if the expected level of 
exercise was not attained, blood pressures at the ankle and other 
pertinent sites measured after exercise, and the time required to 
return the systolic blood pressure toward or to the pre-exercise 
level. When an exercise Doppler study is purchased by the Social 
Security Administration, the requested exercise must be on a 
treadmill at 2 mph on a 10 or 12 percent grade for 5 minutes. 
Exercise studies should not be performed on individuals for whom 
exercise poses a significant risk.
    Application of the criteria in 4.12B may be limited in 
individuals who have marked calcific (Monckeberg's) sclerosis of the 
peripheral arteries or marked small vessel disease associated with 
diabetes mellitus.

4.01  Category of Impairments, Cardiovascular System

    4.02  Chronic heart failure while on a regimen of prescribed 
treatment (see 4.00A if there is no regimen of prescribed 
treatment). With one of the following:
    A. Documented cardiac enlargement by appropriate imaging 
techniques (e.g., a cardiothoracic ratio of greater than 0.50 on a 
PA chest x-ray with good inspiratory effort or left ventricular 
diastolic diameter of greater than 5.5 cm on two-dimensional 
echocardiography), resulting in inability to carry on any physical 
activity, and with symptoms of inadequate cardiac output, pulmonary 
congestion, systemic congestion, or anginal syndrome at rest (e.g., 
recurrent or persistent fatigue, dyspnea, orthopnea, anginal 
discomfort);
      OR
    B. Documented cardiac enlargement by appropriate imaging 
techniques (see 4.02A) or ventricular dysfunction manifested by S3, 
abnormal wall motion, or left ventricular ejection fraction of 30 
percent or less by appropriate imaging techniques; and
    1. Inability to perform on an exercise test at a workload 
equivalent to 5 METs or less due to symptoms of chronic heart 
failure, or, in rare instances, a need to stop exercise testing at 
less than this level of work because of:
    a. Three or more consecutive ventricular premature beats or 
three or more multiform beats; or
    b. Failure to increase systolic blood pressure by 10 mmHg, or 
decrease in systolic pressure below the usual resting level (see 
4.00C2b); or
    c. Signs attributable to inadequate cerebral perfusion, such as 
ataxic gait or mental confusion; and
    2. Resulting in marked limitation of physical activity, as 
demonstrated by fatigue, palpitation, dyspnea, or anginal discomfort 
on ordinary physical activity, even though the individual is 
comfortable at rest;
    OR
    C. Cor pulmonale fulfilling the criteria in 4.02A or B.
    4.03  Hypertensive cardiovascular disease. Evaluate under 4.02 
or 4.04, or under the criteria for the affected body system (2.02 
through 2.04, 6.02, or 11.04A or B).
    4.04  Ischemic heart disease, with chest discomfort associated 
with myocardial ischemia, as described in 4.00E3, while on a regimen 
of prescribed treatment (see 4.00A if there is no regimen of 
prescribed treatment). With one of the following:
    A. Symptom-and sign-limited exercise test demonstrating at least 
one of the following manifestations at a workload equivalent to 5 
METs or less:
    1. Horizontal or downsloping depression, in the absence of 
digitalis glycoside therapy and/or hypokalemia, of the ST segment of 
at least -0.10 millivolts (-1.0 mm) in at least 3 consecutive 
complexes that are on a level baseline in any lead (other than aVR) 
and that have a typical ischemic time course of development and 
resolution (progression of horizontal or downsloping ST depression 
with exercise, and persistence of depression of at least -0.10 
millivolts for at least 1 minute of recovery); or
    2. An upsloping ST junction depression, in the absence of 
digitalis glycoside therapy and/or hypokalemia, in any lead (except 
aVR) of at least -0.2 millivolts or more for at least 0.08 seconds 
after the J junction and persisting for at least 1 minute of 
recovery; or
    3. At least 0.1 millivolt (1 mm) ST elevation above resting 
baseline during both exercise and 3 or more minutes of recovery in 
ECG leads with low R and T waves in the leads demonstrating the ST 
segment displacement; or
    4. Failure to increase systolic pressure by 10 mmHg, or decrease 
in systolic pressure below usual clinical resting level (see 
4.00C2b); or
    5. Documented reversible radionuclide ``perfusion'' 
(thallium201) defect at an exercise level equivalent to 5 METs 
or less;
    OR
    B. Impaired myocardial function, documented by evidence (as 
outlined under 4.00C3 or 4.00C4b) of hypokinetic, akinetic, or 
dyskinetic myocardial free wall or septal wall motion with left 
ventricular ejection fraction of 30 percent or less, and an 
evaluating program physician, preferably one experienced in the care 
of patients with cardiovascular disease, has concluded that 
performance of exercise testing would present a significant risk to 
the individual, and resulting in marked limitation of physical 
activity, as demonstrated by fatigue, palpitation, dyspnea, or 
anginal discomfort on ordinary physical activity, even though the 
individual is comfortable at rest;
    OR
    C. Coronary artery disease, demonstrated by angiography 
(obtained independent of Social Security disability evaluation), and 
an evaluating program physician, preferably one experienced in the 
care of patients with cardiovascular disease, has concluded that 
performance of exercise testing would present a significant risk to 
the individual, with both 1 and 2:
    1. Angiographic evidence revealing:
    a. 50 percent or more narrowing of a nonbypassed left main 
coronary artery; or
    b. 70 percent or more narrowing of another nonbypassed coronary 
artery; or
    c. 50 percent or more narrowing involving a long (greater than 1 
cm) segment of a nonbypassed coronary artery; or
    d. 50 percent or more narrowing of at least 2 nonbypassed 
coronary arteries; or
    e. Total obstruction of a bypass graft vessel; and
    2. Resulting in marked limitation of physical activity, as 
demonstrated by fatigue, palpitation, dyspnea, or anginal discomfort 
on ordinary physical activity, even though the individual is 
comfortable at rest.
    4.05  Recurrent arrhythmias, not related to reversible causes 
such as electrolyte abnormalities or digitalis glycoside or 
antiarrhythmic drug toxicity, resulting in uncontrolled repeated 
episodes of cardiac syncope or near syncope and arrhythmia despite 
prescribed treatment (see 4.00A if there is no prescribed 
treatment), documented by resting or ambulatory (Holter) 
electrocardiography coincident with the occurrence of syncope or 
near syncope.
    4.06  Symptomatic congenital heart disease (cyanotic or 
acyanotic), documented by appropriate imaging techniques (as 
outlined under 4.00C3) or cardiac catheterization. With one of the 
following:
    A. Cyanosis at rest, and:
    1. Hematocrit of 55 percent or greater, or
    2. Arterial O2 saturation of less than 90 percent in room 
air, or resting arterial PO2 of 60 Torr or less;
    OR
    B. Intermittent right-to-left shunting resulting in cyanosis on 
exertion (e.g., Eisenmenger's physiology) and with arterial PO2 
of 60 Torr or less at a workload equivalent to 5 METs or less;
    OR
    C. Chronic heart failure with evidence of ventricular 
dysfunction, as described in 4.02;
    OR
    D. Recurrent arrhythmias as described in 4.05;
    OR
    E. Secondary pulmonary vascular obstructive disease with a mean 
pulmonary arterial pressure elevated to at least 70 percent of the 
mean systemic arterial pressure.
    4.07  Valvular heart disease or other stenotic defects, or 
valvular regurgitation, documented by appropriate imaging techniques 
or cardiac catheterization. Evaluate under the criteria in 4.02, 
4.04, 4.05, or 11.04.
    4.08  Cardiomyopathies, documented by appropriate imaging 
techniques or cardiac catheterization. Evaluate under the criteria 
in 4.02, 4.04, 4.05, or 11.04.
    4.09  Cardiac transplantation. Consider under a disability for 1 
year following surgery; thereafter, reevaluate residual impairment 
under 4.02 to 4.08.
    4.10  Aneurysm of aorta or major branches, due to any cause 
(e.g., atherosclerosis, cystic medial necrosis, Marfan syndrome, 
trauma), demonstrated by an appropriate imaging technique. With one 
of the following:
    A. Acute or chronic dissection not controlled by prescribed 
medical or surgical treatment;
    OR
    B. Chronic heart failure as described under 4.02;
    OR
    C. Renal failure as described under 6.02;
    OR
    D. Neurological complications as described under 11.04.
    4.11  Chronic venous insufficiency of a lower extremity. With 
incompetency or obstruction of the deep venous system and one of the 
following:
    A. Extensive brawny edema;
    OR
    B. Superficial varicosities, stasis dermatitis, and recurrent or 
persistent ulceration which has not healed following at least 3 
months of prescribed medical or surgical therapy.
    4.12  Peripheral arterial disease. With one of the following:
    A. Intermittent claudication with failure to visualize (on 
arteriogram obtained independent of Social Security disability 
evaluation) the common femoral or deep femoral artery in one 
extremity;
    OR
    B. Intermittent claudication with marked impairment of 
peripheral arterial circulation as determined by Doppler studies 
showing:
    1. Resting ankle/brachial systolic blood pressure ratio of less 
than 0.50; or
    2. Decrease in systolic blood pressure at the ankle on exercise 
(see 4.00E4) of 50 percent or more of pre-exercise level at the 
ankle, and requiring 10 minutes or more to return to pre-exercise 
level;
    OR
    C. Amputation at or above the tarsal region due to peripheral 
vascular disease.

    4. Listing 104.00, Cardiovascular System, of part B of appendix 1 
to subpart P of part 404 is revised to read as follows:

104.00  Cardiovascular System

A. Introduction

    The listings in this section describe childhood impairments 
resulting from congenital or acquired cardiovascular disease based 
on symptoms, physical signs, laboratory test abnormalities, and 
response to a regimen of therapy prescribed by a treating source. A 
longitudinal clinical record covering a period of not less than 3 
months of observations and therapy is usually necessary for the 
assessment of severity and expected duration unless the child is a 
neonate or the claim can be decided favorably on the basis of the 
current evidence. All relevant evidence must be considered in 
assessing a child's disability. Reasonable efforts should be made to 
ensure evaluation by a program physician specializing in childhood 
cardiovascular impairments or a qualified pediatrician.
    Examples of congenital defects include: abnormalities of cardiac 
septation, such as ventricular septal defect or atrioventricular 
(AV) canal; abnormalities resulting in cyanotic heart disease, such 
as tetralogy of Fallot or transposition of the vessels; valvular 
defects or obstructions to ventricular outflow, including pulmonary 
or aortic stenosis and/or coarctation of the aorta; and major 
abnormalities of ventricular development, including hypoplastic left 
heart syndrome or pulmonary tricuspid atresia with hypoplastic right 
ventricle. Acquired heart disease may be due to cardiomyopathy, 
rheumatic heart disease, Kawasaki syndrome, or other etiologies. 
Recurrent arrhythmias, severe enough to cause functional impairment, 
may be seen with congenital or acquired heart disease or, more 
rarely, in children with structurally normal hearts.
    Cardiovascular impairments, especially chronic heart failure and 
congenital heart disease, may result in impairments in other body 
systems including, but not limited to, growth, neurological, and 
mental. Therefore, evaluation should include consideration of the 
adverse effects of cardiovascular impairment in all relevant body 
systems, and especially on the child's growth and development, or 
mental functioning, as described under the Growth impairment 
(100.00), Neurological (111.00), and Mental retardation (112.05) 
listings.
    Many children, especially those who have listing-level 
impairments, will have received the benefit of medically prescribed 
treatment. Whenever there is evidence of such treatment, the 
longitudinal clinical record must include a description of the 
therapy prescribed by the treating source and response, in addition 
to information about the nature and severity of the impairment. It 
is important to document any prescribed therapy and response because 
this medical management may have improved the child's functional 
status. The longitudinal record should provide information regarding 
functional recovery, if any.
    Some children will not have received ongoing treatment or have 
an ongoing relationship with the medical community despite the 
existence of a severe impairment(s). Unless the claim can be decided 
favorably on the basis of the current evidence, a longitudinal 
record is still important because it will provide information about 
such things as the ongoing medical severity of the impairment, the 
level of the child's functioning, and the frequency, severity, and 
duration of symptoms. Also, several listings include a requirement 
for continuing signs and symptoms despite a regimen of prescribed 
treatment. Even though a child who does not receive treatment may 
not be able to show an impairment that meets the criteria of these 
listings, the child may have an impairment(s) equivalent in severity 
to one of the listed impairments or be disabled because of a 
substantial reduction in the ability to function independently, 
appropriately, and effectively in an age-appropriate manner.
    Indeed, it must be remembered that these listings are only 
examples of common cardiovascular disorders that are severe enough 
to prevent a child from functioning independently, appropriately, 
and effectively in an age-appropriate manner. When a child has a 
medically determinable impairment that is not listed, or a 
combination of impairments no one of which meets a listing, we will 
make an equivalence determination. Also, with respect to children 
claiming SSI benefits under title XVI of the Act who have an 
impairment(s) with a level of severity which does not meet or equal 
the criteria of the cardiovascular listings, we will determine 
whether the impairment(s) is of comparable severity to one that 
would disable an adult. In these cases, an individualized functional 
assessment is crucial to the evaluation of a child's ability to 
function independently, appropriately, and effectively in an age-
appropriate manner when the impairment(s) is severe but the criteria 
of these listings are not met or equaled.

B. Documentation

    Each child's file must include sufficiently detailed reports on 
history, physical examinations, laboratory studies, and any 
prescribed therapy and response to allow an independent reviewer to 
assess the severity and duration of the cardiovascular impairment. 
Data should be obtained preferably from an office or center 
experienced in pediatric cardiac assessment. The actual 
electrocardiographic tracing (or adequately marked photocopy) and 
echocardiogram report with a copy of relevant echocardiographic 
views should be included (see part A, 4.00C1).
    Results of additional studies necessary to substantiate the 
diagnosis or to document the severity of the impairment, including 
two-dimensional and Doppler echocardiography, and radionuclide 
ventriculograms, should be obtained as appropriate according to part 
A, 4.00C3. Ambulatory electrocardiographic monitoring may also be 
obtained if necessary to document the presence or severity of an 
arrhythmia.
    Exercise testing, though increasingly used, is still less 
frequently indicated in children than in adults, and can rarely be 
successfully performed in children under 6 years of age. It may be 
of value in the assessment of some arrhythmias, in the assessment of 
the severity of chronic heart failure, and in the assessment of 
recovery of function following cardiac surgery or other therapy. It 
will only be purchased by the Social Security Administration if the 
case cannot be decided based on the available evidence and, if 
purchased, must be performed in a specialty center for pediatric 
cardiology or other facility qualified to perform exercise testing 
for children.
    Purchased exercise tests should be performed using a generally 
accepted protocol consistent with the prevailing state of medical 
knowledge and clinical practice. An exercise test should not be 
purchased for a child for whom the performance of the test is 
considered to constitute a significant risk by a program physician. 
See 4.00C2c.
    Cardiac catheterization will not be purchased by the Social 
Security Administration. If the results of catheterization are 
otherwise available, they should be obtained.

C. Treatment and Relationship to Functional Status

    In general, conclusions about the severity of a cardiovascular 
impairment cannot be made on the basis of type of treatment rendered 
or anticipated. The overall clinical and laboratory evidence, 
including the treatment plan(s) or results, should be persuasive 
that a listing-level impairment exists. The amount of function 
restored and the time required for improvement after treatment 
(medical, surgical, or a prescribed program of progressive physical 
activity) vary with the nature and extent of the disorder, the type 
of treatment, and other factors. Depending upon the timing of this 
treatment in relation to the alleged onset date of disability, 
impairment evaluation may need to be deferred for a period of up to 
3 months from the date of treatment to permit consideration of 
treatment effects.
    Evaluation should not be deferred if the claim can be favorably 
decided based upon the available evidence.
    The most life-threatening forms of congenital heart disease and 
cardiac impairments, such as those listed in 104.00D, almost always 
require surgical treatment within the first year of life to prevent 
early death. Even with surgery, these impairments are so severe that 
it is likely that the impairment will continue to be disabling long 
enough to meet the duration requirement because of significant 
residual impairment post-surgery, or the recovery time from surgery, 
or a combination of both factors. Therefore, when the impairment is 
one of those named in 104.00D, or is as severe as one of those 
impairments, the presence of a listing-level impairment can usually 
be found on the basis of planned or actual cardiac surgery.
    A child who has undergone surgical treatment for life-
threatening heart disease will be found under a disability for 12 
months following the date of surgery under 104.06H (for infants with 
life-threatening cardiac disease) or 104.09 (for a child of any age 
who undergoes cardiac transplantation) because of the uncertainty 
during that period concerning outcome or long-term results. After 12 
months, continuing disability evaluation will be based upon residual 
impairment, which will consider the clinical course following 
treatment and comparison of symptoms, signs, and laboratory findings 
preoperatively and after the specified period. (See Secs. 416.994a, 
404.1594, or 416.994, as appropriate, for our rules on medical 
improvement and whether an individual is no longer disabled.)

D. Congenital Heart Disease

    Some congenital defects usually lead to listing-level impairment 
in the first year of life and require surgery within the first year 
as a life-saving measure. Examples of impairments that in most 
instances will require life-saving surgery before age 1, include, 
but are not limited to, the following: hypoplastic left heart 
syndrome; critical aortic stenosis with neonatal heart failure; 
critical coarctation of the aorta, with or without associated 
anomalies; complete AV canal defects; transposition of the great 
arteries; tetralogy of Fallot; and pulmonary atresia with intact 
ventricular septum.
    In addition, there are rarer defects which may lead to early 
mortality and that may require multiple surgical interventions or a 
combination of surgery and other major interventional procedures 
(e.g., multiple ``balloon'' catheter procedures). Examples of such 
defects include single ventricle, tricuspid atresia, and multiple 
ventricular septal defects.
    Pulmonary vascular obstructive disease can cause cardiac 
impairment in young children. When a large or nonrestrictive septal 
defect or ductus is present, pulmonary artery mean pressures of at 
least 70 percent of mean systemic levels are used as a criterion of 
listing-level impairment. In the absence of such a defect (i.e., 
with primary pulmonary hypertension, or in some connective tissue 
disorders with cardiopulmonary involvement and pulmonary vascular 
destruction), listing-level impairment may be present at lower 
levels of pulmonary artery pressure, in the range of at least 50 
percent of mean systemic levels.

E. Chronic Heart Failure

    Chronic heart failure in infants and children may manifest 
itself by pulmonary or systemic venous congestion, including 
cardiomegaly, chronic dyspnea, tachypnea, orthopnea, or 
hepatomegaly; or symptoms of limited cardiac output, such as 
weakness or fatigue; or a need for cardiotonic drugs. Fatigue or 
exercise intolerance in an infant may be manifested by prolonged 
feeding time associated with signs of cardiac impairment, including 
excessive respiratory effort and sweating. Other manifestations of 
chronic heart failure during infancy may include failure to gain 
weight or involuntary loss of weight and repeated lower respiratory 
tract infections.
    Cardiomegaly or ventricular dysfunction must be present and 
demonstrated by imaging techniques, such as two-dimensional and 
Doppler echocardiography. (Reference: Feigenbaum, Harvey, 
``Echocardiography,'' 4th Edition, Lea and Febiger, 1986, Appendix, 
pp. 621-639.) Chest x-ray (6 ft. PA film) will be considered 
indicative of cardiomegaly if the cardiothoracic ratio is over 60 
percent at age 1 year or less, or 55 percent at more than 1 year of 
age.
    Findings of cardiomegaly on chest x-ray must be accompanied by 
other evidence of chronic heart failure or ventricular dysfunction. 
This evidence may include clinical evidence, such as hepatomegaly, 
edema, or pulmonary venous congestion; or echocardiographic 
evidence, such as marked ventricular dilatation above established 
normals for age, or markedly reduced ejection fraction or shortening 
fraction.

F. Valvular Heart Disease

    Valvular heart disease requires documentation by appropriate 
imaging techniques, including Doppler echocardiogram studies or 
cardiac catheterization if catheterization results are available 
from a treating source or other source of record. Listing-level 
impairment is usually associated with critical aortic stenosis in a 
newborn child, persistent heart failure, arrhythmias, or valve 
replacement and ongoing anticoagulant therapy. The usual time after 
valvular surgery for adequate assessment of the results of treatment 
is considered to be 3 months.

G. Rheumatic Heart Disease

    The diagnosis should be made in accordance with the current 
revised Jones criteria for guidance in the diagnosis of rheumatic 
fever.
    104.01  Category of Impairments, Cardiovascular System
    104.02  Chronic heart failure. Documented by clinical and 
laboratory findings as described in 104.00E, and with one of the 
following:
    A. Persistent tachycardia at rest (see Table I);
      OR
    B. Persistent tachypnea at rest (see Table II), or markedly 
decreased exercise tolerance (see 104.00E);
      OR
    C. Recurrent arrhythmias, as described in 104.05;
      OR
    D. Growth disturbance, with:
    1. An involuntary weight loss (or failure to gain weight at an 
appropriate rate for age) resulting in a fall of 15 percentiles from 
established growth curve (on standard growth charts) which persists 
for 2 months or longer; or
    2. An involuntary weight loss (or failure to gain weight at an 
appropriate rate for age) resulting in a fall to below the third 
percentile from established growth curve (on standard growth charts) 
which persists for 2 months or longer; or
    3. Growth impairment as described under the criteria in 100.00. 

                      Table I--Tachycardia at Rest                      
------------------------------------------------------------------------
                                                                 Apical 
                                                                 heart  
                             Age                                 (beats 
                                                                  per   
                                                                minute) 
------------------------------------------------------------------------
Under 1 yr...................................................        150
1 through 3 yrs..............................................        130
4 through 9 yrs..............................................        120
10 through 15 yrs............................................        110
Over 15 yrs..................................................       100 
------------------------------------------------------------------------


                      Table II--Tachypnea at Rest                       
------------------------------------------------------------------------
                                                             Respiratory
                                                              rate over 
                            Age                                  (per   
                                                               minute)  
------------------------------------------------------------------------
Under 1 yr.................................................          40 
1 through 5 yrs............................................          35 
6 through 9 yrs............................................          30 
Over 9 yrs.................................................         25  
------------------------------------------------------------------------

    104.03  Hypertensive cardiovascular disease. With persistently 
elevated blood pressure equal to or greater than the 95th percentile 
for age (see Table III), and one of the following:
    A. Impaired renal function, as described in 106.02;
      OR
    B. Cerebrovascular damage, as described in 111.06;
      OR
    C. Chronic heart failure as described in 104.02. 

                   Table III--Elevated Blood Pressure                   
------------------------------------------------------------------------
                                      Systolic over       Diastolic over
                Age                      (mmHg)      OR       (mmHg)    
------------------------------------------------------------------------
Under 1 month......................              95                   --
1 month through 2 yrs..............             112                   74
3 through 5 yrs....................             116                   76
6 through 9 yrs....................             122                   78
10 through 12 yrs..................             126                   82
13 through 15 yrs..................             136                   86
16 to 18 yrs.......................             142                  92 
------------------------------------------------------------------------

    104.05  Recurrent arrhythmias, such as persistent or recurrent 
heart block (A-V dissociation), repeated symptomatic 
tachyarrhythmias or bradyarrhythmias or long QT syndrome 
arrhythmias, not related to reversible causes such as electrolyte 
abnormalities or digitalis glycoside or antiarrhythmic drug 
toxicity, resulting in uncontrolled repeated episodes of cardiac 
syncope or near syncope and arrhythmia despite prescribed treatment, 
including electronic pacemaker (see 104.00A if there is no 
prescribed treatment), and documented by resting or ambulatory 
(Holter) electrocardiography coincident with the occurrence of 
syncope or near syncope.
    104.06  Congenital heart disease. With one of the following:
    A. Cyanotic heart disease, with persistent, chronic hypoxemia as 
manifested by:
    1. Hematocrit of 55 percent or greater on two or more 
evaluations within a 3-month period; or
    2. Arterial O2 saturation of less than 90 percent in room 
air, or resting PO2 of 60 Torr or less; or
    3. Hypercyanotic spells, syncope, characteristic squatting, or 
other incapacitating symptoms directly related to documented 
cyanotic heart disease; or
    4. Exercise intolerance with increased hypoxemia on exertion;
      OR
    B. Chronic heart failure with evidence of ventricular 
dysfunction, as described in 104.02;
      OR
    C. Recurrent arrhythmias as described in 104.05;
      OR
    D. Secondary pulmonary vascular obstructive disease with a mean 
pulmonary arterial pressure elevated to at least 70 percent of the 
mean systemic arterial pressure;
      OR
    E. Congenital valvular or other stenotic defects, or valvular 
regurgitation, as described in 104.00F and 104.07;
      OR
    F. Symptomatic acyanotic heart disease, with ventricular 
dysfunction resulting in significant restriction of age-appropriate 
activities or inability to complete age-appropriate tasks (see 
104.00A);
      OR
    G. Growth failure, as described in 100.00;
      OR
    H. For infants under 12 months of age at the time of filing, 
with life-threatening congenital heart impairment that will or has 
required surgical treatment in the first year of life, consider the 
infant to be under a disability until the attainment of age 1 or for 
12 months after surgery, whichever is the later event; thereafter, 
evaluate impairment severity with reference to 104.02 to 104.08.
    104.07  Valvular heart disease or other stenotic defects, or 
valvular regurgitation, documented by appropriate imaging techniques 
or cardiac catheterization.
    A. Evaluate according to criteria in 104.02, 104.05, 111.06, or 
11.04;
      OR
    B. Critical aortic stenosis in newborn.
    104.08  Cardiomyopathies, documented by appropriate imaging 
techniques, including echocardiography or cardiac catheterization, 
if catheterization results are available from a treating source. 
Impairment must be associated with an ejection fraction of 50 
percent or less and significant left ventricular dilatation using 
standardized age-appropriate echocardiographic ventricular cavity 
measurements. Evaluate under the criteria in 104.02, 104.05, or 
111.06.
    104.09  Cardiac transplantation. Consider under a disability for 
1 year following surgery; thereafter, evaluate residual impairment 
under 104.02 to 104.08.
    104.13  Chronic rheumatic fever or rheumatic heart disease. 
Consider under a disability for 18 months from the established onset 
of impairment with one of the following:
    A. Persistence of rheumatic fever activity for 6 months or more 
which is manifested by significant murmur(s), cardiac enlargement 
(see 104.00E) or ventricular dysfunction, and other abnormal 
laboratory findings, as for example, an elevated sedimentation rate 
or ECG findings;
      OR
    B. Evidence of chronic heart failure, as described under 104.02;
      OR
    C. Recurrent arrhythmias, as described under 104.05.
    104.14  Hyperlipidemia. Documented Type II homozygous 
hyperlipidemia with repeated plasma cholesterol levels of 500 mg/ml 
or greater, with one of the following:
    A. Myocardial ischemia, as described in 4.04B or 4.04C;
      OR
    B. Significant aortic stenosis documented by Doppler 
echocardiographic techniques or cardiac catheterization;
      OR
    C. Major disruption of normal life activities by repeated 
hospitalizations for plasmapheresis or other prescribed therapies, 
including liver transplant;
      OR
    D. Recurrent pancreatitis complicating hyperlipidemia.
    104.15  Kawasaki syndrome. With one of the following:
    A. Major coronary artery aneurysm;
      OR
    B. Chronic heart failure, as described in 104.02.

[FR Doc. 94-2844 Filed 2-9-94; 8:45 am]
BILLING CODE 4190-11-P