[Federal Register Volume 91, Number 126 (Thursday, July 2, 2026)]
[Rules and Regulations]
[Pages 40804-40851]
From the Federal Register Online via the Government Publishing Office [www.gpo.gov]
[FR Doc No: 2026-13420]
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Vol. 91
Thursday,
No. 126
July 2, 2026
Part III
Social Security Administration
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20 CFR Parts 404 and 416
Revised Medical Criteria for Evaluating Cardiovascular Disorders; Final
Rule
Federal Register / Vol. 91 , No. 126 / Thursday, July 2, 2026 / Rules
and Regulations
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SOCIAL SECURITY ADMINISTRATION
20 CFR Parts 404 and 416
[Docket No. SSA-2019-0013]
RIN 0960-AI43
Revised Medical Criteria for Evaluating Cardiovascular Disorders
AGENCY: Social Security Administration.
ACTION: Final rule.
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SUMMARY: We are revising the criteria in the Listing of Impairments
(listings) that we use to evaluate claims involving cardiovascular
disorders in adults and children under titles II and XVI of the Social
Security Act (Act). The revisions reflect our adjudicative experience,
advances in medical knowledge, and comments we received from the public
in response to a notice of proposed rulemaking (NPRM).
DATES: This rule is effective October 30, 2026.
FOR FURTHER INFORMATION CONTACT: Michael J. Goldstein, Office of
Disability Policy, Social Security Administration, 6401 Security
Boulevard, Baltimore, Maryland 21235-6401, (410) 965-1020.
For information on eligibility or filing for benefits, call our
national toll-free number, 1-800-772-1213, or TTY 1-800-325-0778, or
visit our internet site, Social Security Online, at http://www.socialsecurity.gov.
SUPPLEMENTARY INFORMATION:
Background
The listings describe medical conditions that are so severe that we
presume any adult who has a medical condition(s) that satisfies the
criteria of a listing is unable to perform any gainful activity
regardless of their age, education, or work experience and, therefore,
is disabled.\1\ For children, the listings describe impairments we
consider severe enough to cause marked and severe functional
limitations.\2\ We use the listings at step 3 of the sequential
evaluation process to identify claims that we should clearly allow.\3\
We do not deny any claim solely because a person's medical condition(s)
does not satisfy the criteria of a listing.
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\1\ 20 CFR 404.1525(a) and 416.925(a).
\2\ 20 CFR 416.925(a).
\3\ 20 CFR 404.1520, 404.1525(a), 416.920, 416.924, and
416.925(a).
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We last published final rules that comprehensively revised the
cardiovascular disorders listings on January 13, 2006.\4\ We published
an Advance Notice of Proposed Rulemaking (ANPRM) for cardiovascular
disorders in the Federal Register on April 16, 2008.\5\
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\4\ 71 FR 2312 (2006).
\5\ 73 FR 20564 (2008).
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We are making final the rule for evaluating cardiovascular
disorders that we proposed in the NPRM published in the Federal
Register on June 29, 2022.\6\ The preamble to the NPRM provides the
background and rationale for these revisions. As explained in the NPRM,
the revisions were informed by recommendations from the Institute of
Medicine (IOM) \7\ contained in their report titled ``Cardiovascular
Disability: Updating the Social Security Listings'' (IOM report).\8\
The IOM report provides an important foundation because it was prepared
within the context of the statutory definition of disability and the
cardiovascular listings. The considerations under our disability
program may be different than those found in a clinical or research
setting. For example, the medical listings account for the most severe
impairments that limit a person's function and ability to engage in any
gainful activity, while clinical or research settings may seek to
address all those affected by a condition or impairment and focus on
decision-making regarding diagnosis and treatment of the medical
problem; their focus is not necessarily on the ability to engage in any
gainful activity. The IOM report specifically discusses these
differences: for example, the IOM notes that generally, clinical
guidelines do not address patient disability or employability as a
major topic of discussion and rarely indicate the relationship of
impairment severity to functional limitations that might affect work
capacity.\9\
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\6\ 87 FR 38838 (2022).
\7\ Institute of Medicine (IOM). (2010). Cardiovascular
Disability: Updating the Social Security Listings. Washington, DC:
The National Academies Press. Note: We did not adopt all of the IOM
report's recommendations. In some instances, certain recommendations
were already addressed in another listing, or they conflicted with
existing SSA policy. However, we did adopt multiple IOM
recommendations. See IOM Adoption Chart in Supporting and Related
Materials to this Docket for more details (see also 87 FR 38838).
\8\ On April 28, 2015, the membership of the National Academy of
Science voted to change the name of the IOM to the National Academy
of Medicine. At that time, reports and studies of the IOM continued
as activities of the Health and Medicine Division, a program unit
operating under the direction of the National Academies of Sciences,
Engineering, and Medicine. We will continue to use ``IOM'' and
``Institute of Medicine'' throughout this rule, as this is the name
reflected in the cited report.
\9\ IOM. (2010), 274.
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However, the revisions to our listings are not based solely on the
IOM report. We have additionally reviewed a comprehensive body of
relevant and reliable medical research, consulted with agency medical
experts, and reviewed disability claims involving cardiovascular
disorders to ensure that the revised criteria still reflect listing-
level severity based on current medical practice. You can view the
preamble to the NPRM by visiting http://www.regulations.gov and
searching for document ``SSA-2019-0013.'' There are some differences in
the introductory text and listing text from the NPRM to this final
rule, which we explain below. Those differences reflect, in large part,
our response to public comments we received about our proposed rule.
Why are we revising the listings for evaluating cardiovascular
disorders?
We developed this final rule as part of our ongoing review of the
listings. We are revising the listings for evaluating cardiovascular
disorders to update their medical criteria, and to clarify how we
evaluate cardiovascular disorders.
When will we begin to use this final rule?
As we noted in the dates section of this preamble, this final rule
will be effective on October 30, 2026.
We delayed the effective date of the rule to give us time to update
our systems and to provide training and guidance to all of our
adjudicators before we implement the final rule. The current rules will
continue to apply until the effective date of the final rule. When the
final rule becomes effective, we will apply it to new applications
filed on or after the effective date of the rule, and to claims that
are pending on or after the effective date.\10\
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\10\ This means that we will use this final rule on and after
the effective date in any case in which we make a determination or
decision, including new applications, pending claims, and continuing
disability reviews (CDRs), as applicable. See 20 CFR 404.901,
404.1590, 416.990, and 416.1401. We expect that Federal courts will
review our final decisions using the rules that were in effect at
the time we issued the decisions. If a court reverses our final
decision and remands a case for further administrative proceedings
after the effective date of this final rule, we will apply this
final rule to the entire period at issue in the decision we make
after the court's remand.
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We present a series of tables below. These tables summarize the
revisions we are making to the cardiovascular disorders introductory
text and listings. Following the tables, we discuss the changes in
detail.
The following table summarizes the current and revised sections of
the adult cardiovascular disorders introductory text and listings:
BILLING CODE 4191-02-P
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The following table summarizes the current and revised sections of
the childhood cardiovascular disorders introductory text and listings:
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Listings 4.07 (Aortic valvular disease), 4.08 (Cardiomyopathy), and
4.16/104.16 (Cardiac allograft vasculopathy) are new listings. We added
these listings to more directly address very serious conditions that
can progress quickly and significantly limit an adult's ability to
perform gainful activity or cause marked and severe limitations in a
child's function. The impairments in these listings were previously
evaluated under listings 4.02, 4.04, 4.05, 4.06, 4.09, 11.00, and
104.09.
The following tables show the revisions to the cardiovascular
disorders listings criteria that involve changes to healthcare
utilization and condition/episode requirements, along with the
rationale for each change, and supporting resources.\11\ A version of
this table was in the Notice of Proposed Rulemaking; this updated
version includes current resources and changes in the criteria which
were made in the final rule. Following this table, we discuss all of
the changes to the cardiovascular disorders listings in more detail.
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\11\ Note: We made several additions and changes to the tables
based on feedback from public comments described later in this
document, including updating listing criteria and terminology,
adding more detailed rationales for our changes, and replacing or
supplementing some of the older resources with newer references and
guidelines.
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Please be advised that the tables below contain only the changes
that we are finalizing that relate to healthcare utilization, and not
all revised listing criteria contain changes that relate to healthcare
utilization.
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BILLING CODE 4191-02-C
We are making several changes from the NPRM to this final rule for
cardiovascular disorders:
The following is a high-level summary of the major changes from the
NPRM to this final rule. Below, in the section titled Public Comments
on the NPRM, we describe in greater detail our responses to public
comments, including the changes we made from the NPRM as a result of
the comments. We also made minor, editorial changes from the NPRM for
clarity and readability.
Chronic heart failure (chronic HF): We changed the acronym
we use for chronic heart failure from ``CHF'' to ``chronic HF.'' We
revised the terminology we use to describe the types of heart failure
in paragraph 4.00D1 (What is chronic HF?) and listing 4.02 (Chronic
heart failure) to align with current medical terminology. In the
introductory text, we expanded the list of appropriate medically
acceptable imaging (paragraph 4.00D2 (What evidence of chronic HF do we
need?)), expanded the discussion of symptoms of chronic HF (paragraph
104.00C2b (Your medical history and physical examination)), and
included increased ventricular volume in our discussion of cardiomegaly
(paragraph 104.00C2a (Cardiomegaly or ventricular dysfunction)). In
4.02A1a (Left ventricular end diastolic dimension), we changed the
threshold criterion for left ventricular end diastolic dimension
(LVEDD) and provided different cutoffs for males and females. We also
made a minor corresponding revision to the language describing a
``period of stability'' in paragraph 4.02A1b to replace the term
``acute heart failure'' with the term ``exacerbation of heart
failure,'' which was our original intent and aligns with the language
used in the description of a ``period of stability'' in paragraphs
4.02A1, 4.02A2, and 4.02C and ensures consistency of this description
throughout listing 4.02.
Ischemic heart disease: We revised the introductory text
in paragraphs 4.00E1 (What is ischemic heart disease (IHD)?) and 4.00E2
(What causes chest discomfort of myocardial origin?) to fully capture
the causes of IHD. We also added language about non-obstructive
coronary artery disease (paragraph 4.00E6 (What is variant angina?))
and instantaneous wave-free ratio (iFR) (paragraph 4.00E9f (In 4.04D2,
instantaneous wave-free ratio (iFR)). We added a new listing 4.04D2
(Instantaneous wave-free ratio) to provide another measure of listing-
level IHD.
Peripheral vascular disease: We replaced the term
``peripheral arterial disease'' with ``peripheral artery disease''
throughout section 4.00G (How do we evaluate peripheral vascular
disease?) and listing 4.12 (Peripheral artery disease) to reflect
current medical terminology. In the introductory text, we clarified the
symptoms associated with peripheral artery disease (PAD) (paragraph
4.00G1 (What is peripheral vascular disease (PVD)?)) and lymphedema
(paragraphs 4.00G4 and 104.00F9 (What is lymphedema and how do we
evaluate it?)). We also updated terminology describing ankle-brachial
measurements and toe-brachial measurements we use to evaluate PAD to be
consistent with modern medical practice (paragraphs 4.00G5 (When will
we purchase exercise Doppler studies for peripheral artery disease
(PAD)?), 4.00G6 (Are there any other studies that are helpful in
evaluating PAD?), 4.00G7 (How do we evaluate PAD under 4.12?), and 4.12
(Peripheral artery disease)).
Congenital heart disease: In the introductory text, we
added ``pulmonary atresia'' as an example of congenital valvular
defects in paragraph 104.00D1c (Valvular defects or obstructions to
ventricular outflow). We also added ``pulmonary atresia with intact
ventricular septum'' to the list of single ventricle anomalies in
paragraphs 4.00H3 and 104.00D4 (What is single ventricle?).
Cardiomyopathy: We added functional criteria to listing
4.08A.
Other Changes: We revised paragraph 4.00C15b (Cardiac
catheterization reports) to incorporate language that further describes
the type of information typically provided in cardiac catheterization
reports. We also added language that discusses the evaluation of
genetic connective tissue disorders to paragraphs 4.00I8 and 104.00F10
(What is Marfan syndrome and how do we evaluate it?).
References to the cardiovascular disorders listings in
other body systems: As we finalize revisions to the cardiovascular
disorders listings, we are revising references in the introductory text
for other body systems to mirror changes made in the cardiovascular
listings. Specifically, we made a revision to the term ``peripheral
arterial disease'' in paragraph 1.00B5 and to the term ``Cardiovascular
system'' in paragraph 114.00J2m.
Public Comments on the NPRM
In the NPRM, we provided the public with a 60-day comment period,
which was scheduled to end on August 29, 2022. During the comment
period we received multiple comments requesting that SSA extend the
comment period to give commenters more time to evaluate and respond to
the proposed rule. In response to those requests, we extended the
comment period until September 30, 2022.\12\ We received 14 public
comments.\13\ Those comments came from advocacy groups, legal services
organizations, medical organizations, and individual commenters.
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\12\ 87 FR 51933 (2022).
\13\ Three of the comment letters were requests from the public
for an extension of the comment period.
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We carefully considered all of the public comments related to this
rulemaking. Below, we respond to all of the significant issues raised
by the commenters that were within the scope of this rulemaking. We
have not summarized or responded to comments that were outside the
scope of the proposed rule. Some commenters noted provisions with which
they agreed. We did not summarize or respond to those comments.
Cardiovascular Disorders
Chronic Heart Failure
Comment: One commenter noted that throughout the child and adult
listings, ``chronic heart failure'' is abbreviated as ``CHF.'' They
noted that CHF is commonly used in the medical community to refer to
congestive heart failure. The commenter recommended using the
abbreviation ``chronic HF'' instead.
Response: We adopted this comment.
Comment: Two commenters recommended that we revise the terminology
in paragraph 4.00D1 (SSA Note: In the NPRM we identified this section
as What is chronic heart failure (CHF)?) and listing 4.02A (Medically
documented presence of 4.02A1 or A2) to align with current terminology
reflecting the two types of chronic HF: heart failure with reduced EF
(HFrEF) (EF<40%) and heart failure with preserved EF (HFpEF) (EF>50%).
One of these commenters suggested including heart failure with mid-
range EF (HFmrEF) (EF 40-49%). Another commenter provided proposed text
to describe chronic HF in 4.00D1. The same commenter recommended
changing the description of heart failure in paragraph 104.00C1a (Heart
failure) to match their suggested description in 4.00D1, and
recommended replacing the term diastolic heart failure and systolic
heart failure in 4.02A with the preferred terminology.
Response: We partially adopted these comments. We revised the
terminology in final paragraphs 4.00D1a(i) (Heart failure with reduced
EF (HFrEF)) and 4.00D1a(ii) (Heart failure with preserved EF (HFpEF))
to add HFrEF and HFpEF
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to describe the two types of HF.\14\ We also replaced systolic failure
with HFrEF in revised listing 4.02A1 (Heart failure with reduced
ejection fraction) and diastolic failure with HFpEF in revised listing
4.02A2 (Heart failure with preserved ejection fraction). We did not
include HFmrEF in our revisions; although the terminology and
identification as a distinct category of HF has been accepted by the
clinical community, the available research does not provide a clear
picture of the clinical significance of HFmrEF and its impact on a
person's functioning. Further, there is a lack of consensus as to
whether the addition of HFmrEF can be uniformly applied in practice and
clinical trials.\15\ Therefore, we did not include HFmrEF in our
revisions.
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\14\ Types of heart failure. (n.d.). American Heart Association.
(https://www.heart.org/en/health-topics/heart-failure/what-is-heart-
failure/types-of-heart-
failure#:~:text=This%20is%20also%20known%20as%20heart%20failure%20wit
h%20preserved%20ejection,41%25%20and%2049%25%20EF).
\15\ Heidenreich, P.A., Bozkurt, B., Aguilar, D., Allen, L.A.,
Byun, J.J., Colvin, M.M., Deswal, A., Drazner, M.H., Dunlay, S.M.,
Evers, L.R., Fang, J.C., Fedson, S.E., Fonarow, G.C., Hayek, S.S.,
Hernandez, A.F., Khazanie, P., Kittleson, M.M., Lee, C.S., Link,
M.S., Milano, C.A., . . . ACC/AHA Joint Committee Members (2022).
2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A
Report of the American College of Cardiology/American Heart
Association Joint Committee on Clinical Practice Guidelines.
Circulation, 145(18), e895-e1032. (https://doi.org/10.1161/CIR.0000000000001063).
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Although the commenter suggested matching the description of
chronic HF in paragraph 104.00C1 to their proposed description of
chronic HF in 4.00D1, they also provided proposed text that did not
align with their suggested revisions for 4.00D1. We adopted portions of
their proposed text rather than fully matching the proposed text with
the description of chronic HF in 4.00D1 to account for the discrepancy.
Comment: One commenter recommended that we add right ventricular
failure as an important refractory cause of heart failure to the
definition of chronic HF in paragraph 4.00D1 (SSA Note: In the NPRM we
identified this section as What is chronic heart failure (CHF)). The
same commenter recommended that we add infection and coronary artery
insufficiency as possible causes of heart failure to paragraph
104.00C1b (Chronic HF is considered in these listings).
Response: We did not adopt these comments. The introductory text is
intended to provide information generally about chronic HF for the
public. It is not intended to provide an exhaustive discussion of the
underlying causes of heart failure. Specifically, in paragraphs 4.00D1b
and 104.00C1b (Chronic HF is considered in these listings), we explain
that chronic HF is considered in these listings as a single category
regardless of the underlying cause(s).
Comment: A commenter recommended that we expand the list of
appropriate medically acceptable imaging in paragraph 4.00D2 (What
evidence of chronic HF do we need?) to include cardiac magnetic
resonance imaging (MRI).
Response: We adopted this comment. A cardiac MRI is a noninvasive
test that provides important information in evaluating chronic HF and
meets the definition of appropriate medical imaging in paragraph
4.00A3d.\16\
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\16\ Heidenreich, P.A., Bozkurt, B., Aguilar, D., Allen, L.A.,
Byun, J.J., Colvin, M.M., Deswal, A., Drazner, M.H., Dunlay, S.M.,
Evers, L.R., Fang, J.C., Fedson, S.E., Fonarow, G.C., Hayek, S.S.,
Hernandez, A.F., Khazanie, P., Kittleson, M.M., Lee, C.S., Link,
M.S., Milano, C.A., . . . ACC/AHA Joint Committee Members (2022).
2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A
Report of the American College of Cardiology/American Heart
Association Joint Committee on Clinical Practice Guidelines.
Circulation, 145(18), e895-e1032. (https://doi.org/10.1161/CIR.0000000000001063).
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Comment: We received comments recommending that we revise paragraph
4.00D2a(ii) (SSA Note: In the NPRM we identified an EF of 30 percent or
less during a period of stability) and listing 4.02A1 (Heart failure
with reduced ejection fraction) to require an EF of less than or equal
to 40 percent, rather than our current requirement of an EF of 30
percent or less.
Response: We did not adopt these comments. The American Society of
Echocardiography (ASE) and the European Association of Cardiovascular
Imaging (EACVI) considers an EF in the range of 30 percent to 40
percent as moderately abnormal and less than 30 percent as severely
abnormal.\17\ While ASE and EACVI do not describe how ``severe'' or
``moderate'' abnormalities relate to a patient's ability to engage in
substantial gainful activity, the 2022 ACC/AHA guidelines referenced by
commenters only distinguishes between ``mildly reduced'' EF (41-49
percent) and ``reduced'' EF as 40 percent or below. There is a
difference between being diagnosed with a heart impairment and having
an impairment severe enough to meet a listing. The 40 percent suggested
by the commenters is a threshold for a diagnosis of heart failure, but
is not indicative of the severity required to meet the listings. For
example, for some individuals with an EF of 40 percent, the ACC/AHA
guidelines recommend simply treating the impairment with beta blockers,
which is not indicative of a level of medical severity necessary to
meet a medical listing. We further note that although the ACC/AHA
guidelines do not include a formal conclusion or distinction
differentiating the severity of EF values below 40 percent, the
guidelines do include multiple references to an EF less than 30 percent
as a sign of severity, which is consistent with our criteria.\18\ An EF
between 30 percent and 40 percent does not indicate an impairment that
would prevent a person from performing any gainful activity.
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\17\ See Supplemental Table 3 in. Lang, R.M., Badano, L.P., Mor-
Avi, V., Afilalo, J., Armstrong, A., Ernande, L., Flachskampf, F.A.,
Foster, E., Goldstein, S.A., Kuznetsova, T., Lancellotti, P.,
Muraru, D., Picard, M.H., Rietzschel, E.R., Rudski, L., Spencer,
K.T., Tsang, W., & Voigt, J.-U. (2015). Recommendations for Cardiac
Chamber Quantification by Echocardiography in Adults: An Update from
the American Society of Echocardiography and the European
Association of Cardiovascular Imaging. Journal of the American
Society of Echocardiography, 28(1), 1-39.e14, p. 39.e8. (https://doi.org/10.1016/j.echo.2014.10.003).
\18\ Heidenreich, P.A., Bozkurt, B., Aguilar, D., Allen, L.A.,
Byun, J.J., Colvin, M.M., Deswal, A., Drazner, M.H., Dunlay, S.M.,
Evers, L.R., Fang, J.C., Fedson, S.E., Fonarow, G.C., Hayek, S.S.,
Hernandez, A.F., Khazanie, P., Kittleson, M.M., Lee, C.S., Link,
M.S., Milano, C.A., . . . ACC/AHA Joint Committee Members (2022).
2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A
Report of the American College of Cardiology/American Heart
Association Joint Committee on Clinical Practice Guidelines.
Circulation, 145(18), e895-e1032. (https://doi.org/10.1161/CIR.0000000000001063). See specifically Table 16 (e955) and
discussion of ``severely depressed'' EF (e978).
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Comment: One commenter suggested defining mechanical circulatory
support device (MCSD), the type of MCSD utilized, and length of time
required to establish severity for listing criterion. The commenter
questioned the use of ``Impella devices.''
Response: We partially adopted this comment. We added a discussion
of devices using the Impella technology to paragraphs 4.00D4e and
104.00C4 (How do we evaluate chronic HF treated with a mechanical
circulatory support device?) to clarify that such devices do not
satisfy the requirements of listing 4.02D1 (An implanted mechanical
circulatory support device except extracorporeal membrane oxygenation
(ECMO)) because they are intended for short-term usage only. We did not
revise the text to specify a time period for an implanted MCSD to meet
the criterion in this listing. The clinical conditions for which an
implanted MCSD would be used reflect the underlying severity of chronic
HF and the potential complications. Furthermore, the use of an
implanted device carries additional risks, such as infections, blood
clots, and renal failure. As a result of this comment, we realized that
we inadvertently did not include
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``implanted'' in proposed listings 4.02D1 or 104.02D (Mechanical
circulatory support device), as we intended, and we added this term to
revised 4.02D1 and 104.02D (An implanted mechanical circulatory support
device except extracorporeal membrane oxygenation (ECMO)). We note that
adding ``implanted'' to listings 4.02D1 and 104.02D provides additional
clarity but does not change the substance of those listings because the
acceptable MCSDs described by the listings are always implanted.
Comment: Two commenters suggested that we use a left ventricular
end-diastolic dimension (LVEDD) indexed to body size. One of the
commenters suggested that we provide separate LVEDD cutoffs for men and
women, rather than specifying an absolute cutoff for LVEDD in listing
4.02A1 (Heart failure with reduced ejection fraction).
Response: We partially adopted these comments. We did not include
an LVEDD dimension indexed to body size, but we did provide separate
cutoffs for men and women. While indexing LVEDD to body size can be
useful in clinical practice, uncorrected measurements are generally
available in the medical record and sufficient for determining the most
severe forms of heart failure.\19\ Requiring an indexed LVEDD is not
practical for disability evaluation purposes because indexed LVEDD
measurements are unavailable in many cases. LVEDD is not a stand-alone
criterion for establishing disability; it must be considered in
combination with the impact of chronic HF on the functional limitations
we describe in listing 4.02B (Resulting in 4.02B1, B2, or B3). After
consultation with agency medical experts and reviewing pertinent
research, we did not include indexed LVEDD measurements in the
listings. In cases where an indexed LVEDD measurement is available in
the medical record, adjudicators will evaluate the indexed LVEDD
measurement along with all other evidence in the record when evaluating
disability.
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\19\ See Hayward, C., Perez, C., Patel, H., Mouyis, K., Patel,
K., Akhtar, M., Harding, D., Adasuriya, G., Gillott, H., Harvey, G.,
Sotto, I., & Bhattacharyya, S. (2019). The impact of misclassifying
left ventricular size if indexing to body surface area is not
performed. Imaging, A11.1-A11. (https://doi.org/10.1136/heartjnl-2019-bcs.11). In this study, the uncorrected LVEDD and the indexed
LVEDD led to the same clinical classification 89.2 percent of the
time. (See also Daimon, M., Watanabe, H., Nakanishi, K., Abe, Y.,
Hirata, K., Ishii, K., Iwakura, K., Izumi, C., Abe, H., Negishi, K.,
Ito, H., Tanabe, K., Tanaka, N., & Nakatani, S. (2024). Is left
ventricular diameter indexed for body surface area appropriate for
assessing left ventricular dilation? Journal of cardiology, 84(1),
67-69. (https://doi.org/10.1016/j.jjcc.2024.03.004)). This study
showed that indexing left ventricle (LV) diameters for body surface
area (BSA) might overestimate LV dilation, particularly in subjects
with a small body size.
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We agree with the commenter that different LVEDD cutoffs for men
and women are appropriate and we have changed the LVEDD threshold to
greater than 6.8 cm for men and 6.1 cm for women during a period of
stability. Clinical guidelines from the ASE and the EACVI provide these
separate cutoffs for identifying severe dilation for males and females,
and these cutoffs are easy to apply and are used by practitioners and
researchers.\20\ Additionally, studies have shown that they correlate
with severity.\21\ We also made a minor corresponding revision to the
language describing a ``period of stability'' in paragraph 4.02A1b to
replace the term ``acute heart failure'' with the term ``exacerbation
of heart failure,'' which was our original intent, aligns with the
language used in the description of a ``period of stability'' in
paragraphs 4.02A1, 4.02A2, and 4.02C, and ensures consistency of this
description throughout listing 4.02.
---------------------------------------------------------------------------
\20\ Lang, R.M., Badano, L.P., Mor-Avi, V., Afilalo, J.,
Armstrong, A., Ernande, L., Flachskampf, F.A., Foster, E.,
Goldstein, S.A., Kuznetsova, T., Lancellotti, P., Muraru, D.,
Picard, M.H., Rietzschel, E.R., Rudski, L., Spencer, K.T., Tsang,
W., & Voigt, J.-U. (2015). Recommendations for Cardiac Chamber
Quantification by Echocardiography in Adults: An Update from the
American Society of Echocardiography and the European Association of
Cardiovascular Imaging. Journal of the American Society of
Echocardiography, 28(1), 1-39.e14. (https://doi.org/10.1016/j.echo.2014.10.003); See page 18 in The American Society of
Echocardiography Recommendations for Cardiac Chamber Quantification
In Adults: A Quick Reference Guide From The ASE Workflow And Lab
Management Task Force. (n.d.). (https://www.asecho.org/wp-content/uploads/2018/08/WFTF-Chamber-Quantification-Summary-Doc-Final-July-18.pdf).
\21\ Narayanan, K., Reinier, K., Teodorescu, C., Uy-Evanado, A.,
Aleong, R., Chugh, H., Nichols, G.A., Gunson, K., London, B., Jui,
J., & Chugh, S.S. (2014). Left Ventricular Diameter and Risk
Stratification for Sudden Cardiac Death. Journal of the American
Heart Association, 3(5), e001193. (https://doi.org/10.1161/JAHA.114.001193).
---------------------------------------------------------------------------
Comment: One commenter suggested that we retain the requirement for
an LVEDD of greater than 6 cm in listing 4.02A1 (Heart failure with
reduced ejection fraction) rather than changing it to a value of equal
to or greater than 7 cm. The commenter noted that only patients with
the most severe forms of heart failure would be captured under the 7 cm
criterion and that 6 cm is likely an appropriate threshold to delineate
those patients who will benefit from some advanced cardiovascular
therapies. The same commenter recommended that we include an LV volume
index in addition to or instead of LVEDD.
Response: We did not adopt these comments, but we did revise the
LVEDD criterion to better reflect the cutoffs for males and females
used in clinical practice, as discussed in our response to the previous
comment. An LVEDD of 6 cm reflects a mildly to moderately abnormal
enlargement of the heart.\22\ While 6 cm may be an appropriate
threshold to delineate patients who will benefit from some advanced
cardiovascular therapies, this is not the purpose of our Listing of
Impairments. Rather, our listing for chronic HF is meant to capture
more severe forms of the condition that represent an inability to
perform any gainful activity. An LVEDD threshold of greater than 6.8 cm
for males and greater than 6.1 cm for females more clearly establishes
a severely enlarged heart with signs and symptoms associated with the
functional limitations we require in listing 4.02B (Resulting in
4.02B1, B2, or B3).\23\
---------------------------------------------------------------------------
\22\ Lang, R.M., Badano, L.P., Mor-Avi, V., Afilalo, J.,
Armstrong, A., Ernande, L., Flachskampf, F.A., Foster, E.,
Goldstein, S.A., Kuznetsova, T., Lancellotti, P., Muraru, D.,
Picard, M.H., Rietzschel, E.R., Rudski, L., Spencer, K.T., Tsang,
W., & Voigt, J.-U. (2015). Recommendations for Cardiac Chamber
Quantification by Echocardiography in Adults: An Update from the
American Society of Echocardiography and the European Association of
Cardiovascular Imaging. Journal of the American Society of
Echocardiography, 28(1), 1-39.e14. (https://doi.org/10.1016/j.echo.2014.10.003).
\23\ Institute of Medicine (IOM). (2010). Cardiovascular
Disability: Updating the Social Security Listings (pg. 89).
Washington, DC: The National Academies Press; Narayanan, K.,
Reinier, K., Teodorescu, C., Uy-Evanado, A., Aleong, R., Chugh, H.,
Nichols, G.A., Gunson, K., London, B., Jui, J., & Chugh, S.S.
(2014). Left Ventricular Diameter and Risk Stratification for Sudden
Cardiac Death. Journal of the American Heart Association, 3(5),
e001193. (https://doi.org/10.1161/JAHA.114.001193).
---------------------------------------------------------------------------
In clinical practice, left ventricular volume index scores are most
helpful in evaluating ventricular function in the early stages of heart
failure with normal or mildly reduced EF. At present, reliable left
ventricle volume index scores are time-consuming and not always
feasible. Consequently, left ventricle volume index scores are seldomly
included in echocardiogram reports, whereas LVEDD are routinely
included in echocardiogram reports.\24\
[[Page 40825]]
After consulting with agency medical experts and reviewing pertinent
research, we did not include left ventricular volume index scores in
the listings.
---------------------------------------------------------------------------
\24\ Ito, K., Li, S., Homma, S., Thompson, J.L.P., Buchsbaum,
R., Matsumoto, K., Anker, S.D., Qian, M., Di Tullio, M.R., & WARCEF
Investigators (2021). Left ventricular dimensions and cardiovascular
outcomes in systolic heart failure: the WARCEF trial. ESC heart
failure, 8(6), 4997-5009. https://doi.org/10.1002/ehf2.13560. (See
also Lang, R.M., Badano, L.P., Mor-Avi, V., Afilalo, J., Armstrong,
A., Ernande, L., Flachskampf, F.A., Foster, E., Goldstein, S.A.,
Kuznetsova, T., Lancellotti, P., Muraru, D., Picard, M.H.,
Rietzschel, E.R., Rudski, L., Spencer, K.T., Tsang, W., & Voigt, J.-
U. (2015). Recommendations for Cardiac Chamber Quantification by
Echocardiography in Adults: An Update from the American Society of
Echocardiography and the European Association of Cardiovascular
Imaging. Journal of the American Society of Echocardiography, 28(1),
1-39.e14. (https://doi.org/10.1016/j.echo.2014.10.003)). These
guidelines indicate that there are limitations with each method of
measuring and calculating LV end diastolic volume, and that indexing
to BSA, and 3D measurement and reporting of LV volumes are
recommended when feasible depending on image quality.
---------------------------------------------------------------------------
Comment: Several commenters suggested that we revise the proposed
requirements in listing 4.02A2 (Heart failure with preserved ejection
fraction), including revising the required left atrial volume index
(LAVI) score and adding alternative criteria. One commenter suggested
that an LAVI of greater than 34 ml/m\2\ should be the required cutoff,
while another commenter suggested the cutoff should be an LAVI of 34
ml/m\2\. Another commenter suggested that the cutoff should be greater
than or equal to 30 ml/m\2\. One of the commenters also suggested that
we add diastolic function requirements as an alternative, while another
specifically suggested that we include a tricuspid regurgitant jet
velocity greater than 2.8 m/s and abnormal tissue dopplers over the
mitral valve as a requirement. One of the commenters also suggested
that we include a left ventricular EF (LVEF) of greater than or equal
to 50 percent, a tissue velocity septal E/e ratio on echocardiography
greater than 9, or a lateral E/e ratio greater than 13.
Response: We did not adopt these comments. An LAVI of 34 ml/m\2\,
as suggested by some commenters, falls within the normal range and
serves as a cutoff measurement to identify a heart abnormality.
Generally, higher LAVI measurements indicate greater severity of the
condition. While an LAVI value between 34 and 40 ml/m\2\ may support a
diagnosis of heart failure, as indicated in one public commenter's
reference to a 2016 article from the Journal of American Society of
Echocardiography, such scores are consistent with only mild
enlargement.\25\ By contrast, an LAVI value of 40 ml/m\2\ or greater
identifies people with more severe forms of heart failure and more
clearly establishes an inability to perform any gainful activity than
the other suggested thresholds.\26\
---------------------------------------------------------------------------
\25\ See Table 3 in Nagueh SF, Smiseth OA, Appleton CP, et al.
(2016). Recommendations for the Evaluation of Left Ventricular
Diastolic Function by Echocardiography: An Update from the American
Society of Echocardiography and the European Association of
Cardiovascular Imaging Journal of the American Society of
Echocardiography, 29(4), 277-314, p. 288. (https://doi.org/10.1016/j.echo.2016.01.011).
\26\ See page 18 in The American Society of Echocardiography
Recommendations for Cardiac Chamber Quantification In Adults: A
Quick Reference Guide From The ASE Workflow And Lab Management Task
Force. (n.d.). (https://www.asecho.org/wp-content/uploads/2018/08/WFTF-Chamber-Quantification-Summary-Doc-Final-July-18.pdf).
---------------------------------------------------------------------------
We did not include additional criteria to evaluate diastolic
function because we already provide criteria, including the LAVI, to
evaluate diastolic function. We did not add the suggested LVEF
criterion because the suggested EF is implicit in the HFpEF category
(i.e., a normal EF). Furthermore, we did not add criteria to include
tricuspid regurgitant jet velocity, abnormal tissue dopplers, or tissue
velocity septal E/e ratios. These measurements may provide value in the
overall evaluation of one's cardiovascular condition; however, they are
not consistently contained in echocardiogram reports. The measurements
we include in listing 4.02A (Medically documented presence of 4.02A1 or
A2) are meant to capture the most severe conditions that prevent a
person from being able to engage in any gainful activity. The criteria
we include in 4.02A2 are not a comprehensive list of every measurement
used to diagnose and assess heart failure. Alternative measures that
are not included in the listing criteria may be evaluated under our
rules for medical equivalence.\27\
---------------------------------------------------------------------------
\27\ 20 CFR 404.1526 and 416.926.
---------------------------------------------------------------------------
Comment: One commenter noted that the proposed criterion in listing
4.02A2 (Heart failure with preserved ejection fraction) may not be
standard criterion, and questioned whether the proposed criterion
captures diastolic failure of a non-hypertrophic etiology. They noted
it was unclear which criteria were needed to establish a diagnosis.
Response: We disagree with the commenter's concern that the
criteria are not standard. The measurements in 4.02A2 are commonly
included in echocardiogram reports and used to identify left atrial
enlargement, which leads to chronic HF. We use the criteria in 4.02A2
to identify people with diastolic failure that may prevent a person
from engaging in any gainful activity. Satisfying the criteria in
4.02A2 is insufficient to find a person disabled at the listing level;
the person's limitations caused by the heart failure must also satisfy
the criteria in listing 4.02B (Resulting in 4.02B1, B2, or B3). There
are many non-hypertrophic causes of diastolic dysfunction, including,
but not limited to, coronary artery disease, arrythmias (such as atrial
fibrillation), and non-hypertrophic cardiomyopathy. These and other
non-hypertrophic causes of diastolic dysfunction may be evaluated under
other listings, such as 4.04 (Ischemic heart disease), 4.05 (Recurrent
arrythmias), and 4.08 (Cardiomyopathy).
Comment: Several commenters suggested that we add elevated b-type
natriuretic peptide (BNP) levels as another way to assess chronic HF at
listing 4.02A2 (Heart failure with preserved ejection fraction).
Response: We did not adopt this comment. BNP (and Immunoreactive
amino terminal pro-brain natriuretic peptide (NT-proBNP)) measurements
alone are insufficient for determining listing-level severity. These
levels vary in relation to heart failure severity and may be influenced
by other factors such as age, sex, body mass index, and other medical
conditions. Further, these measurements are most often obtained during
periods of instability, while the listings contemplate functional
ability during periods of stability. We discuss how we use BNP and NT-
proBNP in paragraph 4.00D1b (Chronic HF is considered in these
listings).
Comment: One commenter suggested that we remove the requirement of
an EF of 20 percent or less from listing 4.02C (Heart failure with left
ventricular ejection fraction of 20 percent or less), noting the cutoff
is too low and arbitrary.
Response: We did not adopt this comment. The IOM recommended a
criterion for chronic HF with an EF on a sustained basis of 20 percent
or less.\28\ An EF of only 20 percent means the heart's pumping action
is less than a third of normal, and therefore critically affects a
person's ability to perform gainful activity.\29\ Most people with
heart disease this advanced have a greater risk of mortality and major
functional limitations, such as shortness of breath or fatigue, even
during mild exertion. In addition to consulting with the IOM and
reviewing the medical research supporting this criterion, we reviewed
disability claims involving chronic HF to ensure that the revised
[[Page 40826]]
criteria reflect listing-level severity based on medical practice. This
criterion is an administrative expedient to quickly identify people
whose chronic HF is at a listing-level of severity.
---------------------------------------------------------------------------
\28\ IOM. (2010), 84, 89.
\29\ Content--Health Encyclopedia--University of Rochester
Medical Center. (n.d.). (https://www.urmc.rochester.edu/encyclopedia/content?contenttypeid=56&contentid=DM14); IOM. (2010),
84, 89; Runge, M.S., Patterson, C., Stouffer, G.A., & Netter, F.H.
(2010). Netter's Cardiology (2nd ed.). Philadelphia, PA: Saunders
Elsevier; Fukunaga, N., Ribeiro, R. V.P., Lafreniere-Roula, M.,
Manlhiot, C., Badiwala, M.V., & Rao, V. (2020). Left Ventricular
Size and Outcomes in Patients With Left Ventricular Ejection
Fraction Less Than 20%. The Annals of Thoracic Surgery, 110(3), 863-
869. (https://doi.org/10.1016/j.athoracsur.2020.01.005).
---------------------------------------------------------------------------
Ischemic Heart Disease
Comment: One commenter suggested several revisions to more fully
capture the causes of ischemic heart disease in paragraphs 4.00E1 (What
is ischemic heart disease (IHD)?) and 4.00E2 (What causes chest
discomfort of myocardial origin?). The same commenter also suggested
revisions to paragraph 4.00E6 (What is variant angina?) to include non-
obstructive coronary artery disease in our discussion of variant
angina.
Response: We partially adopted the commenter's suggestion to revise
4.00E1, 4.00E2, and 4.00E6. Although we did not propose changes to
these sections in the NPRM, we agree that it is appropriate to include
language addressing non-obstructive coronary artery disease to more
fully explain potential sources of chest discomfort. These changes do
not affect the substantive criteria of the listings, as they describe
other possible origins of chest discomfort as background information
but do not contain additional requirements to meet any listing.
Comment: One commenter stated that the term ``fractional flow
reserve'' (FFR) at listing 4.04D1 (Fractional flow reserve) was too
restrictive and would exclude other similarly effective measures of
coronary physiology that are commonly used (including other non-
hyperemic pressure ratios such as diastolic hyperemia-free ratio (DFR),
resting full-cycle ratio (RFR), diastolic pressure ratio (DPR), and/or
instantaneous wave-free ratio (iFR)). The commenter suggested we use
the term ``coronary artery physiology'' instead. Another commenter
suggested adding iFR as another method to measure stenosis severity in
section 4.00E (How do we evaluate ischemic heart disease?) and 4.04D1.
Response: We partially adopted these comments. We added iFR as a
second method to measure the severity of stenosis in 4.00E and 4.04D2
because iFR and FFR are two of the most commonly used physiological
methods of assessment and have been thoroughly validated for clinical
use.\30\ However, we did not add the term ``coronary artery
physiology'' to 4.04D1 because it does not align with specific measures
of stenosis severity that are required in the listing.
---------------------------------------------------------------------------
\30\ Lawton, J.S., Tamis-Holland, J., Bangalore, S., Bates, E.,
Beckie, T., Bischoff, J., Bittl, J., Cohen, M., DiMaio, J.M., Don,
C., Fremes, S., Gaudine, M., Goldberger, Z., Grant, M., Jaswal, J.,
Kurlansky, P., Mehran, R., Metkus, Jr., T., Nnacheta, L., Rao, S.,
C.A, . . . 2021 ACC/AHA/SCAI Guideline for Coronary Artery
Revascularization: A Report of the American College of Cariology/
American Heart Association Joint Committee on Clinical Practice
Guidelines. Circulation, 145 (3), e18-e114. (https://doi.org/10.1161/CIR.0000000000001038).
---------------------------------------------------------------------------
Comment: One commenter suggested we replace the term ``irregular
heartbeat'' in listing 4.04C (Documentation of three separate ischemic
episodes) with ``arrythmia thought to be due to ischemic cause''
because the term is nonspecific.
Response: We did not adopt this suggestion. We do not use the term
``irregular heartbeat'' in 4.04C. We used that term in the
Supplementary Information section of the NPRM and in paragraph 4.00I2
(What is cardiomyopathy and how do we evaluate it?). We did not use the
language suggested by the commenter for any listing criteria because
the listing criteria must identify specific evidence required to meet
the listing, and the phrase ``thought to be due to ischemic cause'' is
not specific.
Comment: Listing 4.04E (Exacerbations or complications of ischemic
heart disease) includes both planned and unplanned hospital admissions
for ischemic heart disease. One commenter questioned whether this
included planned staged interventions of coronary artery disease.
Response: Planned staged interventions are not exacerbations nor
would they be separate events showing symptoms requiring
hospitalization with improvement in the meantime to show it is an
exacerbation. The staged surgical treatment plan, which may be in
response to a complication or exacerbation, would be a single event and
would not, by itself, satisfy the criterion in 4.04E.
Congenital Heart Disease
Comment: One commenter expressed concerns that section 4.00H (How
do we evaluate congenital heart disease?) does not adequately cover all
forms of congenital heart disease. Specifically, their concern was that
we do not include congenital heart diseases that can progress to heart
failure, including right-sided heart failure. The commenter recommended
that we allow exceptions for congenital heart disease that are not
characterized in the listings.
Response: We did not adopt these comments. Section 4.00H1 (What is
congenital heart disease?) states that congenital heart disease is any
abnormality of the heart or the major blood vessels that is present at
birth. When congenital heart disease results in chronic HF, we evaluate
it under listing 4.02 (Chronic heart failure), regardless of the
underlying cause. As we have explained elsewhere, we do not intend to
provide exhaustive discussions of congenital heart conditions that may
be disabling or provide listing criteria for each condition. Notably,
our rules for medical equivalence provide flexibility in determining
whether an impairment is disabling by allowing us to consider whether
the person's impairment is at least equal in severity and duration to
the criteria of any listed impairment.\31\ Moreover, if we are unable
to find a person's cardiovascular disorder disabling based on meeting
or equaling a listed impairment, we will continue the sequential
evaluation and may find the person disabled at the final step of the
process.\32\
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\31\ 20 CFR 404.1520 and 416.920.
\32\ 20 CFR 404.1520(a)(4) and (g) and 416.920(a)(4) and (g).
---------------------------------------------------------------------------
Comment: One commenter proposed that we change the heading at
paragraph 4.00H3 (What is single ventricle?) to read ``What is
unrepaired congenital heart disease'' to ``include definitions of
single ventricle.'' Another commenter urged us to include ``unrepaired
cyanotic congenital heart disease'' in listing 4.06D (Single ventricle
(with or without Fontan procedures)), as they broadly interpret Fontan
circulation as ``unrepaired.''
Response: We did not adopt these comments. Paragraph 4.00H3 states
that the term ``single ventricle'' (also known as single ventricle
physiology or functional single ventricle) describes a diverse group of
congenital cardiac anomalies sharing the common feature that only one
of the two heart ventricles is adequately developed.\33\ This applies
whether the single ventricle has been repaired or not. Furthermore, we
note in 4.06D that the criterion applies whether or not Fontan
procedures have been performed.
---------------------------------------------------------------------------
\33\ Of note, the AHA guidelines use the term ``single
ventricle.'' Stout, K.K., Daniels, C.J., Aboulhosn, J.A., Bozkurt,
B., Broberg, C.S., Colman, J.M., . . . Van Hare, G.F. (2019). 2018
AHA/ACC Guideline for the Management of Adults With Congenital Heart
Disease: A Report of the American College of Cardiology/American
Heart Association Task Force on Clinical Practice Guidelines.
Journal of the American College of Cardiology, 73 (12), e81-e192.
(https://doi.org/10.1016/j.jacc.2018.08.1029).
---------------------------------------------------------------------------
Comment: A commenter suggested that while there is a theoretical
value in linking hypoxia to hematocrit in listing 4.06A1(Hematocrit of
55 percent or greater), there may be more variables that affect
hematocrit. The commenter provided specific variables they indicated
affect hematocrit in addition
[[Page 40827]]
to hypoxia. They noted that unless the criterion accounts for all such
variables, there may be no value in linking hypoxia to hematocrit. They
included specific variables that are not discussed in proposed 4.06A1.
Response: We did not make changes based on this comment. Listing
4.06A1 discusses chronic hypoxemia, not hypoxia. We agree there may be
variables other than hypoxemia that affect hematocrit, including the
examples the commenter provided. Although the comment specifically
referenced 4.06A1, which applies to adults, some of the commenter's
examples relate to conditions in children. The criterion in 4.06A1 is
only one of several alternatives for evaluating hypoxemia in congenital
heart disease. We also use arterial blood gas measurements and pulse
oximetry for evaluating hypoxemia when considering congenital heart
disease. Other causes of elevated hematocrit levels would not be
evaluated under this listing.
Comment: We received several comments related to use of pulse
oximetry in the adult and childhood listings related to hypoxemia. One
commenter noted that the 87 percent oxygen saturation rate under
listings 4.06A and 104.06A (Chronic hypoxemia) seemed low and added
that people can be limited when resting saturation is 90 percent.
Another commenter suggested the saturation cut-off should be set at
less than or equal to 89 percent.
Response: We did not adopt these comments. We use a threshold of 89
percent or below when oxygen saturation is obtained through arterial
blood gas (ABG) measurements. Pulse oximeter measurements may be
between 2 and 4 percent higher or lower than ABG measurements.\34\ The
87 percent measurement in listings 4.06A3 (SpO2)
and 104.06A3 (SpO2) accounts for this known
discrepancy between pulse oximeter and ABG measurements. In addition to
consulting with the IOM and reviewing the medical research supporting
this criterion, we reviewed disability claims involving congenital
heart disease to ensure that the revised criteria reflect listing-level
severity based on medical practice.
---------------------------------------------------------------------------
\34\ Torp KD, Modi P, Pollard EJ, et al. Pulse Oximetry. 2023
Jul 30. In: StatPearls [internet]. Treasure Island (FL): StatPearls
Publishing; 2024 Jan-. PMID: 29262014; Yale Medicine. (2023, January
3). Pulse Oximetry. (https://www.yalemedicine.org/conditions/pulse-oximetry). See also MedlinePlus. (2025, November 28). Pulse
Oximetry. (https://medlineplus.gov/lab-tests/pulse-oximetry/).
---------------------------------------------------------------------------
Comment: One commenter noted that the proposal in listing 4.06A
(Chronic hypoxemia) to require three separate
SpO2 measurements 30 days apart within a 12-month
period to show chronic hypoxemia may be onerous, unnecessary, and would
delay appropriate diagnosis.
Response: We do not agree that the requirements in 4.06A would
delay appropriate diagnosis. The requirements in our listings are
intended to evaluate the severity of a person's congenital heart
disease, not to establish a diagnosis. The criteria for three separate
measurements 30 days apart within a 12-month period demonstrates
chronicity of a person's hypoxemia. Although we indicate that the
evaluations must occur within a 12-month period, people may have the
requisite findings in a shorter time period. Further, gathering three
separate SpO2 measurements is one alternative for
documenting chronic hypoxemia in our listings.
Comment: One commenter noted that proposed listing 4.06 (Congenital
heart disease) includes no specific mention of target threshold stats
after the 6-minute walk test (6MWT) and encouraged more specificity
towards a cardiac versus chronic pulmonary issue. The same commenter
suggested we revise listing 4.06A3 (SpO2) to
include people who are unable to perform the 6MWT.
Response: We did not adopt these comments. The criterion in listing
4.06A3 requires a target threshold amount of less than or equal to 87
percent of oxygen saturation of blood hemoglobin on three evaluations
at least 30 days apart within a consecutive 12-month period, during a
6MWT or after a 6MWT. We do not need to differentiate between cardiac
and pulmonary conditions in listing 4.06 because the listing requires a
diagnosis of congenital heart disease documented by appropriate
medically acceptable imaging or cardiac catheterization. The listing
criteria cannot be met without first fulfilling one of these
requirements, which relate directly to cardiac conditions, not
pulmonary conditions.
If a person is unable to perform the 6MWT, we provide other
criteria for evaluating congenital heart disease under listing 4.06A
(Chronic hypoxemia), including hematocrit, arterial blood gas levels,
and oxygen saturation levels measured by pulse oximetry at rest.
Comment: Under their comments identified as pertaining to listing
criteria 4.06A (Chronic hypoxemia), one commenter indicated that
criteria should also exist for those patients who are not cyanotic or
hypoxic.
Response: We did not adopt this comment. In listing 4.06
(Congenital heart disease), we provide several alternatives for
evaluating congenital heart disease that do not require hypoxemia or
cyanosis, including hypertension (4.06C) and exacerbations or
complications requiring three hospitalizations in a 12-month period
(4.06E). While listing 4.06A requires a diagnosis of chronic hypoxemia,
a person with congenital heart disease who is not cyanotic or hypoxic
may still be found disabled under one of the other criteria in listing
4.06, other cardiovascular listings, our rules for functional
equivalence in children,\35\ or at step 5 of the adult sequential
evaluation process.\36\
---------------------------------------------------------------------------
\35\ 20 CFR 416.926a.
\36\ 20 CFR 404 1520(g) and 416.920(g).
---------------------------------------------------------------------------
Comment: One commenter suggested that we include criterion
pertaining to cardiac surgery and complications from cardiac
intervention in listing 4.06A1 (Hematocrit of 55 percent or greater).
Response: We did not adopt this comment. The occurrence of a
surgery does not necessarily mean a person will have limitations that
prevent them from performing substantial gainful activity and meet the
durational requirement. If the person has complications resulting from
a surgery, we will evaluate them under listing 4.06E (Exacerbations or
complications of congenital heart disease).
Comment: One commenter encouraged SSA to consider the duration of a
hospitalization as a marker for complexity, not just the need for
readmission under listing 4.06E (Exacerbations or complications of
congenital heart disease). The same commenter similarly encouraged SSA
to consider prolonged hospitalizations under listing 104.06E
(Exacerbations or complications of congenital heart disease). They
noted concerns that a person who is chronically hospitalized, which
they defined as more than 90 days, would not qualify for disability
under 104.06E but likely should.
Response: We did not make any changes based on these comments. We
consider the duration of a person's hospitalization as a marker of
complexity; both 4.06E and 104.06E require that each hospitalization
last at least 48 hours, including hours in a hospital emergency
department immediately before the hospitalization. A person with
prolonged hospitalization as described by the commenter is likely to
have medical findings that satisfy another criterion. The condition(s)
leading to the prolonged hospitalization may also medically equal
another cardiac listing under our equivalence
[[Page 40828]]
policy.\37\ Furthermore, satisfying the hospitalization criteria is
only one way that we may find a person disabled under the adult and
childhood listings for congenital heart disease.
---------------------------------------------------------------------------
\37\ 20 CFR 404.1526 and 416.926.
---------------------------------------------------------------------------
Vascular Disease
Comment: We received comments suggesting that we use the term
``peripheral artery disease'' in place of ``peripheral arterial
disease'' throughout section 4.00G (How do we evaluate peripheral
vascular disease?) and listing 4.12 (SSA Note: In the NPRM we titled
this section ``Peripheral arterial disease''), and replace the term
``ankle-brachial systolic blood pressure ratio'' with ``ankle-brachial
index,'' which is the common term used for the test.
Response: We adopted these comments. We must use appropriate modern
medical terminology to specify the medical criteria we use to evaluate
cardiovascular disorders. Our research indicates that ``ankle-brachial
index'' is the more commonly used term among professionals in the field
of cardiology.\38\ For consistency, we also replaced the term
``arterial'' with ``artery'' (in this final rule listing 4.12 is now
titled ``Peripheral artery disease''). Additionally, we replaced ``toe/
brachial systolic blood pressure ratio'' with ``toe-brachial index.''
---------------------------------------------------------------------------
\38\ A review of the website for the Journal of the American
Medical Association (JAMA), a peer-reviewed medical journal
published 48 times a year by the American Medical Association, found
that the term ``ankle-brachial index'' was used more than ``ankle-
brachial systolic blood pressure ratio.''
---------------------------------------------------------------------------
Comment: A commenter recommended adding ``lymphatics'' to the list
describing disorders of the veins or arteries at paragraph 4.00A1c
(Disorders of the veins or arteries) and adding text that describes the
symptoms of lymphedema to paragraph 4.00G1 (What is peripheral vascular
disease (PVD)?). The same commenter recommended broadening paragraph
104.00F9 (What is lymphedema and how do we evaluate it?) to address
``lymphatic disorder'' or ``lymphatic perfusion disorder.''
Response: We partially adopted these comments. We added language to
paragraph 4.00G4a and 104.00F9a (Lymphedema) that describes lymphedema.
We did not revise paragraphs 4.00A1c or 4.00G1. The lymphatic system is
not part of the cardiovascular system. Disorders of lymphatic
circulation, such as lymphedema, are related to the immune system of
the body. Although the signs and symptoms of lymphatic disease are
similar to those associated with peripheral vascular disease, lymphatic
disease is not part of the vascular system and is treated in a
completely different manner. We believe it is more appropriate to
provide general guidance about lymphedema in this section rather than
focus on specific lymphatic disorders.
Comment: One commenter suggested editorial changes to paragraph
4.00G6 (Are there any other studies that are helpful in evaluating
PAD?) to include brief discussion of Doppler waveforms and
plethysmographic tracings. The commenter further suggested that we
include ``stenting'' along with our discussion of peripheral grafting
in paragraph 4.00G9 (How do we use listing 4.12 if you have had a
peripheral graft?) to better reflect current medical technologies and
treatment.
Response: We adopted the suggested editorial changes.
Comment: One commenter questioned whether venous dopplers are the
only method to establish the diagnosis of chronic venous insufficiency.
Response: Venous dopplers are not the only method to establish a
diagnosis of chronic venous insufficiency. In listing 4.11 (Chronic
venous insufficiency), we allow for a person's chronic venous
insufficiency to be documented by duplex ultrasound ``or other
appropriate diagnostic technique.''
Comment: One commenter recommended clarifying section 4.00G (How do
we evaluate peripheral vascular disease?) to include that peripheral
artery disease can cause leg or foot pain.
Response: We adopted this comment. This change better encapsulates
symptoms of claudication, which include pain not only in the calf, but
also other parts of the lower extremities, such as the thighs or
buttocks. This wording is consistent with the language in listing 4.12
(Peripheral artery disease), which discusses intermittent claudication
or leg pain, as opposed to simply calf pain.
Comment: One commenter recommended that we add a third option to
the criteria in listing 4.11 (Chronic venous insufficiency) to account
for leg pain that interferes with mobility and provided a specific
criterion for consideration.
Response: We did not adopt this comment. The commenter suggested a
standalone criterion that does not include objective medical findings
and does not quantify the limitation in mobility. Without specific
quantified criteria for limitations in mobility, we would not be able
to ensure the consistent application of the proposed criterion. To
consider pain as a listing criterion, we would also require medical
signs documenting the chronic venous insufficiency. Symptoms such as
pain are subjective and difficult to quantify. We will not substitute
symptoms such as pain for a medical sign (or diagnostic finding) in the
listing criteria. However, if we are unable to find a person's
cardiovascular disorder meets or medically equals a listed impairment,
we will continue the sequential evaluation process and evaluate the
person's symptoms, including pain, as set forth in our regulations.\39\
---------------------------------------------------------------------------
\39\ 20 CFR 404.1529 and 416.929.
---------------------------------------------------------------------------
Comment: A commenter had questions pertaining to the duration
requirement of listing 4.11B (Two or more episodes of ulceration),
specifically whether this listing requires the presence of an
ulceration that has not healed following at least 6 months of
treatment, or whether it would also need to last for 12 continuous
months.
Response: A temporal requirement such as the requirement described
in 4.11B serves as a specific indicator of listing-level severity and
does not establish that the medically determinable impairment (MDI)
meets the duration requirement. To meet the duration requirement, the
MDI(s) must have lasted, or be expected to last, for a continuous
period of at least 12 months and the person's resulting inability to
perform substantial gainful activity by reason of the MDI(s) must also
have lasted, or be expected to last, for not less than 12 months
without interruption or stopping.\40\
---------------------------------------------------------------------------
\40\ 20 CFR 404.1509 and 416.909. See also SSR 23-1p (2023).
Available at: https://www.ssa.gov/OP_Home/rulings/di/01/SSR2023-01-di-01.html.
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Comment: One commenter inquired as to why we did not include a
listing addressing deep venous thromboses (DVT) or pulmonary emboli
(PE).
Response: We understand the commenter's concerns related to DVTs
and PEs. When DVTs or PEs are chronic, there are a number of factors we
consider in determining the appropriate body system(s) for evaluation.
When the chronic DVT or PE has a specific cause, we will evaluate the
impairment under the listing related to that cause. For example, an
underlying disorder of thrombosis would be evaluated under the
hematological body system. In other cases, the chronic DVT or PE may
result in another MDI. For example, the chronic DVTs may cause heart
failure which would be evaluated under listing 4.02 (Chronic heart
failure). In many cases, the chronic DVT may result in signs and
symptoms similar to those for chronic venous insufficiency. In such
cases, we would evaluate the impairment under the medical
[[Page 40829]]
equivalence policy for listing 4.11 (Chronic venous insufficiency).
Other Cardiovascular Disorders
Comment: One commenter suggested broadening listing 4.07 (Aortic
valvular disease) to include all valvular heart diseases, as all valve
diseases can cause heart failure symptoms and disability.
Response: We did not adopt this comment. The introductory text at
paragraph 4.00I3 (How do we evaluate valvular heart disease?)
specifically notes we may evaluate other forms of valvular disease
under listings 4.02 (Chronic heart failure), 4.04 (Ischemic heart
disease), 4.05 (Recurrent arrhythmias), 4.06 (Congenital heart
disease), or a listing in 11.00 (Neurological Disorders), depending on
its effects on the person. The listings are not intended to be an
exhaustive compilation of disabling conditions. Rather, they are used
to identify cases at an early stage of the sequential evaluation
process that meet a strict threshold for the statutory definition of
disability. They describe impairments that we consider severe enough to
prevent an adult from doing any gainful activity. If an impairment does
not meet a listing, this does not mean that we will deny a claim.
Rather, we will continue the sequential evaluation.
Comment: One commenter suggested that listing 4.08 (Cardiomyopathy)
should include a functional component and should not be dependent on an
exercise tolerance test (ETT). The commenter expressed concerns about
logistical barriers related to practices of locally available
physician's offices that may inhibit access to vendors who can properly
perform ETTs for some offices (e.g., Disability Determination
Services), and suggested that the listing needs to take this into
consideration. The same commenter expressed concerns about the ability
to order echocardiograms and the resulting effect on the ability of
those offices to evaluate claimants under listings 4.02C and 4.07.
Another commenter suggested that functional details would help clarify
guidelines regarding the cardiomyopathy listing.
Response: We adopted the suggestion to add functional criteria to
listing 4.08. Although proposed listing 4.08 provided criteria for
evaluating cardiomyopathy when ETTs present significant risks to the
person, we agree that consideration of a person's functioning is
appropriate. This criterion is consistent with the criterion we provide
in listing 4.02B1b (Very serious limitation) for evaluating chronic HF
when ETTs cannot be performed.
Regarding the commenter's concern about some offices having
barriers to ordering ETTs or echocardiograms, the listing criteria are
based on a person's medical condition and the types of findings
typically present in disability claims. An echocardiogram is a common
diagnostic test that is typically included in the medical evidence of
record for claimants with these impairments. In addition, we allow
other acceptable medical testing. Furthermore, listing 4.08 provides
several alternative criteria that do not consider ETTs or
echocardiograms for evaluating cardiomyopathy. SSA would follow the
existing processes (which are outside of the scope of the listing
criteria and this rulemaking) to address any logistical concerns and
barriers to acquiring the necessary medical evidence, which may
include, when appropriate, ordering an echocardiogram.
Comment: One commenter suggested we establish sex-specific criteria
in evaluating hypertrophic cardiomyopathy in listing 4.08
(Cardiomyopathy) to account for differences in body size and heart
dimensions.
Response: We did not adopt this comment. In the clinical guidelines
for diagnosing and evaluating hypertrophic cardiomyopathy, the American
College of Cardiology, the American Society of Echocardiology, and the
American Heart Association do not provide standards for cardiac wall
measurements based on sex or body size.\41\ Therefore, we think it is
inappropriate to incorporate such requirements in our listings.
---------------------------------------------------------------------------
\41\ Heidenreich, P.A., Bozkurt, B., Aguilar, D., Allen, L.A.,
Byun, J.J., Colvin, M.M., Deswal, A., Drazner, M.H., Dunlay, S.M.,
Evers, L.R., Fang, J.C., Fedson, S.E., Fonarow, G.C., Hayek, S.S.,
Hernandez, A.F., Khazanie, P., Kittleson, M.M., Lee, C.S., Link,
M.S., Milano, C.A., . . . ACC/AHA Joint Committee Members (2022).
2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A
Report of the American College of Cardiology/American Heart
Association Joint Committee on Clinical Practice Guidelines.
Circulation, 145(18), e895-e1032. (https://doi.org/10.1161/CIR.0000000000001063).
---------------------------------------------------------------------------
Comment: One commenter suggested adding all etiologic factors to
listing 4.10 (Dissecting aneurysm of the aorta or major branches), as
it currently does not specifically mention other connective tissue
disorders or penetrating aortic ulcers as other etiological factors.
Response: We did not adopt this comment. Under listing 4.10, we
evaluate dissecting aneurysm of the aorta or major branches due to any
cause. The listing text is not meant to be an exhaustive compilation of
the causes of dissecting aneurysms; therefore, it is not necessary to
list other etiological factors.
Comment: One commenter asked if listings 4.16 and 104.16 (Cardiac
allograft vasculopathy) address issues such as vasculitis, including
Kawasaki disease.
Response: Cardiac allograft vasculopathy is a condition that
affects the blood vessels of the heart in people who have had a heart
transplant. The condition does not include those who develop narrowing
of the arteries due to other conditions. People who develop narrowing
or blockage of arteries of the heart in the absence of a heart
transplant are evaluated under listing 4.04 (Ischemic heart disease).
Systemic vasculitis is an immune system disorder which we evaluate
under listings 14.03 and 114.03 (Systematic vasculitis). We provide
information about how we evaluate Kawasaki disease in paragraph
104.00F8 (How do we evaluate Kawasaki disease?).
Comment: One commenter suggested we include other genetic
connective tissue disorders with serious cardiovascular effects, such
as Loeys-Dietz Syndrome, in paragraphs 4.00I8 and 104.00F10 (What is
Marfan syndrome and how do we evaluate it?).
Response: We adopted this comment to explain how genetic connective
tissue disorders other than Marfan syndrome are evaluated under the
listings. We added a new paragraph to sections 4.00I and 104.00F (How
do we evaluate other cardiovascular disorders?) that discusses the
evaluation of connective tissue disorders with cardiovascular effects,
such as Loeys-Dietz syndrome and Ehlers-Danlos syndrome.
Miscellaneous
Comment: One commenter questioned why ``reduced oxygen
concentration'' is listed as a mechanism for hypoxemia in paragraphs
4.00A1b(iv) and 104.00A1b(iv) (Hypoxemia), when hypoxemia is synonymous
with reduced oxygen concentration.
Response: We agree that including the words ``reduced oxygen
concentration'' is redundant. Therefore, we removed ``reduced oxygen
concentration in the arterial blood'' as a cause of hypoxemia.
Comment: One commenter noted that in paragraph 4.00C8d (We will
wait to purchase an exercise test), ``percutaneous transluminal
coronary angioplasty'' and ``percutaneous coronary intervention'' are
the same procedure, and we should consider using only one term.
Response: We did not adopt this comment. While these terms are both
names for the same procedure, they are each used in medical records. We
kept
[[Page 40830]]
both terms in 4.00C8d to account for the use of both terms in medical
records.
Comment: One commenter recommended that we revise our language in
paragraph 4.00C15 (How do we evaluate cardiac catheterization
evidence?) to better describe the type of information commonly provided
by a cardiac catheterization. The commenter also provided specific
language for consideration.
Response: We partially adopted this comment. As the commenter
suggested, we removed the last sentence of paragraph 4.00C15a (We will
not purchase) and we revised paragraph 4.00C15b (Cardiac
catheterization reports). Specifically, we incorporated additional
information into the description of typical cardiac catheterization
report content, while retaining other findings commonly seen by
adjudicators in medical evidence. We did not include some of the
suggested language that only described background information and was
less helpful in understanding the requirements to meet any listing.
Comment: One commenter recommended clarifying that paragraph
4.00C16 (What details should exercise Doppler test reports contain?)
applies to Doppler studies of the lower extremities and recommended
specific language.
Response: We partially adopted this comment. While relatively rare,
exercise Doppler tests can be performed for conditions other than
peripheral vascular disease. We recognize that this distinction was not
clear in our proposed language; therefore, while we did not include all
of the exact language suggested by the commenter, we revised the
language to distinguish between elements of a report common to all
exercise Doppler tests and those specific to exercise Doppler tests for
peripheral vascular disease.
Comment: A commenter noted that the 3-month waiting period after
treatment begins under paragraph 4.00J2 (How do we relate treatment to
functional status?) may be unnecessary due to the rapid progression of
some peoples' disease and the improbable improvement with treatment.
Response: We did not adopt this comment. In paragraph 4.00B4 (When
will we wait before we ask for more evidence?), we explain that we may
need to defer evaluation of an impairment for a period of up to 3
months from the date treatment began. We further state in paragraph
4.00B4b (In these situations) that ``we will not wait if we have enough
information to make a determination or decision based on all of the
relevant evidence in your case.''
Comment: One commenter questioned whether determinations regarding
ability to perform an ETT made by ``medical sources'' in listing
4.02B1a (A medical source has concluded) and listing 4.08
(Cardiomyopathy) includes medical consultants.
Response: We did not make any changes in response to this comment.
Paragraph 4.00D4c(i) (Your impairment satisfies the first part) states
that if the case record does not include a conclusion from a medical
source that an ETT would present a significant risk to you, a medical
consultant as defined in paragraph 4.00A3a (Medical consultant) may
make such a conclusion.
Comment: A commenter indicated that the term ``prescribed
treatment'' lacked clarity and suggested replacing it with ``guideline-
recommended treatment'' in listing 4.12 (SSA Note: In the NPRM we
titled this section ``Peripheral arterial disease'').
Response: We did not adopt this comment. The term ``failure to
follow prescribed treatment'' is a term of art that is described in our
regulations at 20 CFR 404.1530 and 416.930. We hold a person
responsible to follow treatment prescribed by their medical source if
that treatment is expected to restore the ability to work. The term
``guideline-recommended treatment'' does not adequately convey that
requirement.
Comments Specific To Evaluating Cardiovascular Disorders in Children
Comment: A commenter suggested replacing the word ``corrective''
with ``palliative'' in paragraph 104.00B4 (When will we wait before we
ask for more evidence?).
Response: We did not adopt this comment. Paragraph 104.00B4
discusses when we will wait before we ask for additional evidence. The
term ``corrective'' covers conditions where improvement may occur post-
surgery and is appropriate for a situation when we will wait before
asking for more evidence. The term ``palliative'' implies a likeliness
that the person will have significant lifelong impairment in function.
This term would not be an appropriate replacement, because the term
would apply to situations when we do not wait for additional evidence.
Comment: One commenter recommended removing the word ``congestive''
from paragraph 104.00B4a(iii) (If you have started new drug therapy).
Response: We adopted this comment. Although we did not propose
changing the wording in 104.00B4a(iii), we agree removing
``congestive'' reflects the terminology more commonly used in the
medical field.
Comment: A commenter recommended we add ``volume'' as a measure of
ventricle size as evidence necessary to show cardiomegaly in paragraph
104.00C2b(i) (Symptoms of congestion). The same commenter recommended
we remove the requirement for a 6-foot PA film to show cardiomegaly.
Response: Although the commenter identified the incorrect section
for the discussion of cardiomegaly, we adopted the recommendation and
added increased ventricular volume in the discussion of cardiomegaly in
paragraph 104.00C2a (Cardiomegaly or ventricular dysfunction) because
increased ventricular volume is a finding that is helpful in evaluating
cardiomegaly and is often documented in medical records. However, we
did not make additional changes based on the recommendation to remove
the requirement for 6-foot PA film, as we had already proposed removing
this requirement in the NPRM and ultimately did so in this final rule.
Comment: A commenter noted that the absence of tachycardia may no
longer be a relevant assessment of the severity of heart failure in
discussing listing 104.02A (Persistent tachycardia at rest).
Response: We did not make changes based on this comment. Assessment
of tachycardia is an important and common factor in evaluating heart
failure. Even in its absence, listing 104.02 (Chronic heart failure)
provides several criteria by which a person may be found disabled,
including several criteria that do not consider tachycardia.
Comment: A commenter recommended that we consider revising the
criterion under listing 104.02A (Persistent tachycardia at rest) and
listing 104.02B (Persistent tachypnea at rest) to define tachycardia
and tachypnea as a resting heart rate or respiratory rate
(respectively) in excess of the upper limit of normal for that age. The
commenter also suggested that we use the criteria on more than one
evaluation more than 3 days apart, rather than at least 90 days apart.
The same commenter also noted that the 12-month assessment period is
too long and would result in delays.
Response: We did not adopt these comments. The commenter appears to
suggest that we use a broad definition of tachycardia and tachypnea
rather than providing specific cutoffs for each age range. The
definition suggested is overly broad and there does not appear to be
consensus among the medical community as to what the ``upper limit of
normal'' is for various age ranges.
[[Page 40831]]
Furthermore, the use of a definition without specific cutoffs could
lead to inconsistent determinations and decisions because of the
underlying variation in the cutoffs used by the medical community.
Regarding the commenter's suggestion to require more than 3 days
between evaluations, as we stated in the NPRM, we believe that a longer
time period between evaluations is necessary to ensure that the
underlying condition is chronic and not acute. We believe that
requiring evaluations to occur at least 90 days apart is an appropriate
time period for establishing the chronicity of the underlying
condition. Although we indicate that the evaluations must occur within
a 12-month period, we expect that many people may have the requisite
findings in a shorter time period.
Comment: With respect to listing 104.02A (Persistent tachycardia at
rest), a commenter noted that tachycardia and tachypnea are not
exclusive to chronic HF and may also be seen in acute or chronic HF or
heart failure exacerbation.
Response: Although we understand the commenter's concern, listing
104.02 (Chronic heart failure) is specifically used to evaluate chronic
HF, and not other disorders which may present with tachycardia and
tachypnea.
Comment: One commenter noted that persistent tachycardia can also
include measurement by palpation of pulse, in addition to the proposed
apical heart rate.
Response: We did not make changes based on this comment. A pulse
rate taken by palpation is a measurement of the pressure waves created
by contraction of the left ventricle, an indirect measurement, whereas
measurement of the apical pulse rate is a direct measurement of the
left ventricle's contraction; therefore, this direct measurement is a
more accurate measurement of the child's heart rate than the indirect
measurement. The apical pulse gives the most accurate reading.\42\
---------------------------------------------------------------------------
\42\ Zimmerman B., Williams D. Peripheral Pulse. 2025 July 6.
In: StatPearls [internet]. Treasure Island (FL): StatPearls
publishing; 2025 Jan. PMID: 31194332. (https://www.ncbi.nlm.nih.gov/books/NBK542175/).; Cleveland Clinic. Apical Pulse. (https://my.clevelandclinic.org/health/articles/23346-apical-pulse).
---------------------------------------------------------------------------
Comment: One commenter suggested we consider growth failure instead
of persistent tachycardia and tachypnea for the pediatric population
under listing 104.02 (Chronic heart failure). Another commenter
expressed concerns that growth failure over the 12-month evaluation
period under listing 104.02C (Growth failure) may not be reflected on
growth charts as it is generally treated aggressively, with providers
not waiting 12 months to initiate treatment. The same commenter also
suggested expanding the symptoms of chronic HF in paragraph 104.00C2b
(Your medical history and physical examination) to include signs and
symptoms related to feeding intolerance and associated complications.
Response: We did not adopt the first comment because we already
provide criteria for evaluating growth failure in chronic HF in
104.02C. It is one of several ways to evaluate chronic HF in children,
along with persistent tachycardia and tachypnea. Additionally, although
we understand the second commenter's concerns, the criteria for growth
failure in 104.02C are intended to establish that the growth failure
persists while on a regimen of prescribed treatment, including
nutritional support. Furthermore, while we indicate that the
evaluations must occur within a 12-month period, many people will have
the requisite findings in a shorter time period. We adopted the
suggestion to expand the symptoms of chronic HF in 104.00C2b to include
signs and symptoms related to feeding intolerance and associated
complications.
Comment: One commenter questioned the requirement in listing
104.02E (Exacerbations or complications of chronic heart failure) that
children with chronic heart failure experience three hospitalizations
within a consecutive 12-month period. They noted three hospitalizations
``seems high'' and suggested requiring only two hospitalizations within
a consecutive 12-month period. The same commenter similarly suggested
that we reconsider the hospitalization criterion in listing 104.06E
(Exacerbations or complications of congenital heart disease), which
also requires three hospitalizations within a 12-month period.
Response: We did not make changes based on these comments. For
children, the requirement for three hospitalizations within a
consecutive 12-month period is grounded in our rules for functional
equivalence, specifically how we define ``marked'' and ``extreme''
limitations in the ``Health and physical well-being'' domain.\43\ If a
child has frequent exacerbations of their impairment (i.e., episodes of
illness or exacerbations that occur on average of three times per year,
or once every 4 months, each lasting 2 weeks or more) that result in
significant, documented signs or symptoms, we may consider that to be a
``marked'' limitation in this domain.\44\ If the frequent exacerbations
result in significant, documented symptoms or signs that are
substantially in excess of the requirements for showing a ``marked''
limitation, we may find the child to have an ``extreme'' limitation in
this domain.\45\ Although the hospitalization requirement is less than
the 2 weeks contemplated in the definition of a ``marked'' limitation,
the 2-week period will also consider the period immediately before the
hospitalization and the post-hospitalization recovery. Exacerbations
and complications requiring frequent hospitalization demonstrate a
level of care beyond the usual course of treatment for cardiovascular
disorders and are consistent with listing-level severity.
---------------------------------------------------------------------------
\43\ 20 CFR 416.926a(e)(2)(iv) and 416.926a(e)(3)(iv).
\44\ 20 CFR 416.926a(e)(2)(iv).
\45\ 20 CFR 416.926a(e)(3)(iv).
---------------------------------------------------------------------------
Further, the hospitalization criterion in 104.02E and 104.06E is
just one of several ways to document listing-level severity under each
of these listings. We are also able to evaluate exacerbations or
complications of chronic HF and congenital heart disease resulting in
fewer than three hospitalizations in a consecutive 12-month period
using our rules for medical equivalence,\46\ under our rules for
functional equivalence,\47\ or under other listing criteria.
---------------------------------------------------------------------------
\46\ 20 CFR 404.1526 and 416.926.
\47\ 20 CFR 416.926a.
---------------------------------------------------------------------------
Comment: In response to a question we posed on the consideration of
the atrial measurements in listing 4.02A2 (Heart failure with preserved
ejection fraction), one commenter noted that the criteria found in
listing 4.02A1 (Heart failure with reduced ejection fraction) are not
applicable to children. They suggested that eligibility for children
should be determined based on z-scores, biomarkers, or clinical
findings as opposed to diastolic dimensions and wall thickness. They
noted that left atrial enlargement is difficult to use as a criterion
in children since there is no normative data for all age groups and the
presence of some types of heart disease precludes this measurement.
Furthermore, they noted that other end organ failure (renal disease,
growth failure, neurodevelopmental disorders) needs to be considered
for their impact.
Response: We did not make any changes in the final rule based on
the commenter's concerns. Our final criteria for evaluating chronic HF
in children do not include the types of specific measurements we use
for adults. As we noted in the NPRM, we proposed to revise paragraph
104.00C2 (What evidence of chronic HF do we need?) to
[[Page 40832]]
remove ``specific findings for documenting cardiomegaly,'' because such
findings are infrequently included in a child's case record and this
absence presents difficulty in case adjudication. We believe that
requiring z-scores or biomarkers would also be infrequently present and
thus poses similar difficulty in case adjudication. Our rules already
provide for evaluating other end organ failure. If the chronic HF
results in end organ failure, such as renal disease and
neurodevelopmental disorders, we will evaluate the effects under the
appropriate body system or under our rules for functional
equivalence.\48\ We already provide specific guidance for evaluating
growth failure in paragraph 104.00C3 (How do we evaluate growth failure
due to chronic HF?).
---------------------------------------------------------------------------
\48\ 20 CFR 416.926a.
---------------------------------------------------------------------------
Comment: One commenter recommended revisions to paragraph 104.00D1
(What is congenital heart disease?) to include additional treatments,
such as an interventional catheterization procedure, and examples of
congenital heart abnormalities, such as truncus arteriosus, total
anomalous pulmonary venous return, and Epstein malformation.
Response: We adopted the recommended revisions to provide
additional examples of congenital heart disease and its treatment.
Comment: One commenter recommended that we expand the list of
impairments that may require life-saving surgery before age 1 in
paragraph 104.00D2c (For 104.06D, life-threatening congenital heart
disease) to include ``pulmonary atresia'' and ``critical pulmonary
stenosis.''
Response: We did not adopt this comment. The examples of life-
saving surgeries in 104.00D2c are not meant to be an exhaustive list of
every life-saving surgery before age 1. However, we did make a related
change to introductory text paragraph 104.00D1 (What is congenital
heart disease?) as a result of the comment. We added pulmonary atresia
as an example of congenital valvular defects in paragraph 104.00D1c
(Valvular defects or obstructions to ventricular outflow).
Comment: A commenter suggested revising the text in paragraph
104.00D4 (What is single ventricle?) and adding ``pulmonary atresia
with intact ventricular septum'' to the list of single ventricle
anomalies.
Response: We partially adopted this comment. We added ``pulmonary
atresia with intact ventricular septum'' to the list of single
ventricle anomalies. We did not make the suggested minor edits to the
text, because this paragraph describes background information, and the
minor edits had unnecessary detail that is not informative for
adjudication. However, we did include the additional discussion of
Fontan circulation in 104.00D4. We made parallel changes to paragraph
4.00H3 (What is single ventricle?).
Comment: We received a comment related to the use of arterial
saturation in listing 104.06 (Congenital heart disease). A commenter
recommended we consider replacing the proposed emphasis on arterial
saturation with oximetry and imaging diagnosis at listing 104.06A
(Chronic hypoxemia), but if arterial saturation were to be used, the
commenter recommended that the threshold should be set to less than 95
percent in room air. The commenter noted that the current threshold
would not be appropriate for children considering that the adult
threshold is set to 89 percent. The commenter also suggested a better
alternative would be an echocardiographic diagnosis of anatomic
abnormality.
Response: We did not adopt this comment. As we previously stated,
the criterion for ABG measurements is only one alternative for
establishing disability under the listing for congenital heart disease.
Although ABGs may be infrequently done on children, these tests may be
found in the medical evidence for some children. A threshold of less
than 95 percent on room air is not appropriate because it does not
reflect a level of hypoxemia that would result in marked and severe
limitations (i.e., that threshold does not reflect a disabling
impairment).
Echocardiography is appropriate medically acceptable imaging that
we use to establish congenital heart disease as an MDI. However, an
anatomical abnormality established by echocardiography does not
necessarily establish a degree of limitation that a child would
experience. To establish the degree of limitations, we consider chronic
hypoxemia or other medical findings.
Comment: One commenter suggested revising the description of
congenital heart disease in listing 104.06C (Single ventricle) to
account for those with single ventricle congenital heart disease. The
commenter provided specific language they suggested we include in the
listing, including specific functional limitations.
Response: We did not adopt this comment. The criterion in 104.06C
is sufficient to establish a disabling impairment and it is not
necessary to include the functional component suggested by the
commenter. In addition to consulting with the IOM and reviewing the
medical research supporting this criterion, we reviewed disability
claims involving single ventricle congenital heart disease to ensure
that the revised criteria reflect listing-level severity based on
medical practice.
Comment: One commenter suggested we consider additional assessments
to evaluate chronic HF and congenital heart disease in children. They
specifically recommended including poor feeding, poor weight gain,
frequent respiratory infections, irritability, and hepatomegaly.
Response: We did not adopt this comment. We provide a criterion for
evaluating poor weight gain in listing 104.02C (Growth failure). We
also provide a criterion for considering frequent respiratory
infections in listings 104.02E (Exacerbations or complications of
chronic heart failure) and 104.06E (Exacerbations or complications of
congenital heart disease). Furthermore, the listings are not meant to
be an exhaustive list of all signs, symptoms, and complications of
cardiac impairments. They are meant to identify the common medical
findings that cause marked and severe limitation in most children.
Signs and symptoms not found in the listings may still be evaluated
under the functional equivalence rules.\49\ Some conditions may also be
evaluated under their related body system, such as evaluating
hepatomegaly under the Digestive Disorders listings.
---------------------------------------------------------------------------
\49\ 20 CFR 416.926a.
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Comment: One commenter recommended that we revise the text in
paragraph 104.00E4a (Implanted cardiac defibrillators), describing
children at risk for sudden cardiac arrest due to arrhythmias,
including adding ``hypertrophic cardiomyopathy'' and ``rare forms of
ischemic cardiomyopathy'' to the description.
Response: We did not adopt this comment. This section addresses the
evaluation of children with implanted cardiac defibrillators who do not
meet the listing requirements. The information about cardiomyopathy
merely identifies the largest group of children at risk for sudden
cardiac death and is not intended to provide information about the
evaluation of cardiomyopathy. We provide more specific information
about cardiomyopathy in paragraph 104.00F3 (What is cardiomyopathy and
how do we evaluate it?).
Comment: One commenter suggested adding specific language to
paragraph 104.00F1 (What is ischemic heart disease (IHD) and how do we
evaluate
[[Page 40833]]
it in children?) to address the causes of IHD in children.
Response: We did not adopt the commenter's suggested language. Our
evaluation of IHD in children focuses on the functional limitations
resulting from IHD and thus addressing specific causes of IHD is not
required for adjudication of these cases.
Comment: One commenter suggested revising the description of
cardiomyopathy we proposed in paragraph 104.00F3a (There are various
types of cardiomyopathy) to include subtypes of dilated, hypertrophic,
restrictive, noncompaction, and arrhythmogenic.
Response: We partially adopted this comment. We included the
suggested ``arrhythmogenic'' as it is a more common type of
cardiomyopathy and removed ``hypertensive,'' because it is less
commonly used to categorize cardiomyopathies. However, we did not
include all the suggested types because there is lack of consensus as
to what constitutes a separate type of cardiomyopathy. We also made
these changes in the introductory text for consistency. Although the
final text does not enumerate all five subtypes listed by the
commenter, our text already refers to several of them. These are
included for illustrative purposes and are not meant to be an
exhaustive list of every type or subtype of cardiomyopathy.\50\ The
specific subtype of cardiomyopathy is not a consideration under our
childhood listings. We will evaluate cardiomyopathy in children under
listing 4.04 (Ischemic heart disease) in part A, listing 104.02
(Chronic heart failure), or listing 104.05 (Recurrent arrhythmias),
depending on its effects on the child.
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\50\ National Heart, Lung, and Blood Institute (2024, December
6). Cardiomyopathy Types. (https://www.nhlbi.nih.gov/health/cardiomyopathy/types); Brieler, J., Breeden, M., Tucker, J. (2017).
Cardiomyopathy: An Overview. American Family Physician, 96 (10),
640-646, (https://www.aafp.org/pubs/afp/issues/2017/1115/p640.html).
These articles illustrate the diversity of classifications of
cardiomyopathy.
---------------------------------------------------------------------------
Comment: One commenter noted that we should consider the person's
status classification when evaluating children listed for a transplant
under paragraph 104.00F5c (We will not assume). They noted that
children placed on the transplant list as category ``1A'' are most
certainly disabled as they require extensive medical intervention
including intensive care hospitalization, life support measures, and
certain cardiac supporting intravenous medications with a Swan-Ganz
catheter, or mechanical-assist devices.
Response: We did not adopt this comment. Although we do not
consider a child's status on the transplant waiting list, we will
consider the evidence that resulted in that classification. For
children who are in classification 1A, we expect that the evidence
supporting 1A will, in most cases, result in a finding of disability
based on meeting or medically equaling a cardiovascular disorder
listing or functionally equaling the listings.\51\
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\51\ 20 CFR 416.924.
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Comment: One commenter suggested we retain the listing for
rheumatic heart disease (current listing 104.13).
Response: We did not adopt this suggestion. As we state in final
paragraph 104.00F6 (How do we evaluate chronic rheumatic fever or
rheumatic heart disease?), we evaluate the manifestations of rheumatic
heart disease under other criteria, such as listing 104.02 (Chronic
heart failure) or listing 104.05 (Recurrent arrhythmias). If a child's
rheumatic heart disease does not meet or medically equal a listing, we
will evaluate it under our functional equivalence rules.\52\
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\52\ 20 CFR 416.924.
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Other Concerns Raised by Commenters
Comment: We received several comments expressing concerns with the
age of the IOM report and that our updates to the listings are based on
outdated science. One commenter noted our changes to the listings may
not make the listings more scientifically accurate and may result in
some people no longer qualifying for benefits through the listings.
Response: The IOM report is one of several sources that informed
these revisions to the cardiovascular disorders listings. Despite the
age of the IOM report, it remains relevant because it was drafted
within the context of the statutory definition of disability and the
cardiovascular listings. These considerations may be different than
those found in a strictly clinical setting. However, in drafting the
NPRM and this final rule, we additionally reviewed current research,
consulted with agency cardiologists and other medical experts, and
reviewed disability claims involving cardiovascular disorders to ensure
the IOM recommendations are still relevant. We are confident the policy
changes we are finalizing reflect relevant and current medical
practice, and the impact of cardiovascular impairments on the person's
ability to sustain gainful activity, or ability to perform age-
appropriate activities (for children).
We recognize the importance of clinical practice in formulating
policy. In drafting this final rule, we reviewed and considered
information more recently released, including the joint guidelines
promulgated by the American Heart Association, the American College of
Cardiologists, and the Heart Failure Society of America.\53\ However,
the clinical guidelines focus on the diagnosis and treatment of people
with cardiac conditions and do not necessarily consider a person's
level of impairment or functioning as it relates to the definition of
disability in the Act.\54\ In addition, we considered recent
information from other sources, including the comments we received from
the public in response to the NPRM and the published sources of medical
literature and research we list in the references section of the NPRM
and those cited in this final rule.
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\53\ Heidenreich, P.A., Bozkurt, B., Aguilar, D., Allen, L.A.,
Byun, J.J., Colvin, M.M., Deswal, A., Drazner, M.H., Dunlay, S.M.,
Evers, L.R., Fang, J.C., Fedson, S.E., Fonarow, G.C., Hayek, S.S.,
Hernandez, A.F., Khazanie, P., Kittleson, M.M., Lee, C.S., Link,
M.S., Milano, C.A., . . . ACC/AHA Joint Committee Members (2022).
2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A
Report of the American College of Cardiology/American Heart
Association Joint Committee on Clinical Practice Guidelines.
Circulation, 145(18), e895-e1032. (https://doi.org/10.1161/CIR.0000000000001063).
\54\ See sections 216(i)(1), 223(d), and 1614(a)(3) of the Act
(42 U.S.C. 416(i)(1), 423(d), 1382c(a)(3)).
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Comment: One commenter suggested that we consider whether reliance
on healthcare use as a proxy for severity raises concerns regarding
racial equity.
Response: We appreciate the commenter's suggestion. However, to be
disabled under the Act, a person must have one or more medically
determinable impairments--impairments that result from anatomical,
physiological, or psychological abnormalities shown by medically
acceptable clinical and laboratory diagnostic techniques.\55\ If there
is insufficient medical evidence for us to determine whether a person
is disabled, we may ask the person to attend one or more examinations
or tests at our expense.\56\ Once we have evidence that shows a
medically determinable impairment, we consider all the available
evidence from all sources, medical and non-medical, when we make a
disability determination.
---------------------------------------------------------------------------
\55\ See sections 216(i)(1), 223(d)(1) and 1614(a)(3) of the Act
(42 U.S.C. 416(i)(1), 423(d)(1), and 1382c(a)(3)). 20 CFR 404.1521
and 416.921.
\56\ 20 CFR 404.1517 and 416.917.
---------------------------------------------------------------------------
The listings describe impairments that are so severe they prevent
people from doing any substantial gainful activity regardless of their
age, education, or
[[Page 40834]]
work experience.\57\ People with very serious cardiovascular disorders,
such as those described in these listings, often receive the kinds of
diagnostic treatments and tests discussed in the listings because of
urgent medical need.
---------------------------------------------------------------------------
\57\ 20 CFR 404.1525(a) and 416.925(a).
---------------------------------------------------------------------------
However, we do not penalize those who do not have access to the
kinds of medical evidence that we describe in these listings.
Furthermore, we provide several alternative criteria for people with
cardiovascular impairments to establish that their impairment is of
listing-level severity. If a person's cardiovascular disorder does not
meet the requirements of any listing, we can still find that person
disabled based on a finding of medical equivalence or functional
equivalence in child claims or at a later step in our adjudication
process.\58\
---------------------------------------------------------------------------
\58\ 20 CFR 404.1520, 416.920, and 416.924.
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Comment: We received several comments expressing concerns with the
hospitalization criteria in our listings for cardiovascular disorders.
Several commenters noted our listings do not account for current
medical practice or updated research on the role of healthcare
utilization as a proxy for severity. They noted that SSA does not
provide compelling evidence to explain why hospitalizations are an
appropriate metric by which to measure severity. Another commenter
noted that many procedures that used to require inpatient
hospitalization are now routinely performed on an outpatient basis, and
that using hospitalizations as a predictor of disability would exclude
people who are getting the exact same treatment as those in 2010.
Another commenter suggested that severity and prognosis should be
classified based on trajectory of symptoms rather than frequencies of
exacerbations or number of hospitalizations. Several commenters
expressed concerns that using hospitalization as a proxy for disability
would disproportionately disadvantage low-income people in rural areas.
Response: We decided to retain the hospitalization criteria as one
of several alternative criteria in the affected listings because our
intent is to reflect impairments that are so severe they result in an
inability to perform any gainful activity. The hospitalization criteria
reflect a need for a level of care beyond conventional outpatient
treatments or isolated or brief hospitalizations for cardiovascular
disorders. We understand the concerns regarding the decrease in
hospitalizations due to the use of outpatient procedures and other
advances in treatment. For people who are able to access these
outpatient procedures and other advances in treatment, our other
listing criteria specify the documentation required to evaluate
cardiovascular disorders. However, in many locations, especially rural
settings, people do not have access to preventative care, regular
treatment, or more advanced treatment. As such, their only recourse for
treating and managing severe cardiovascular disease is
hospitalization.\59\ None of the cardiovascular listings require a
specific number of hospitalizations as the only way to meet the
listing. The hospitalization criterion in the listings for chronic
heart failure (4.02 and 104.02), ischemic heart disease (4.04),
congenital heart disease (4.06 and 104.06), and cardiomyopathy (4.08)
gives people another avenue of establishing a listing-level impairment.
---------------------------------------------------------------------------
\59\ National Academies of Sciences, Engineering, and Medicine.
2018. Health-Care Utilization as a Proxy in Disability
Determination. Washington, DC: The National Academies Press.
(https://doi.org/10.17226/24969).
---------------------------------------------------------------------------
Although some of our listings include criteria for repeated
hospitalizations, our rules for medical equivalence \60\ and functional
equivalence in children \61\ provide a means for adjudicators to
consider the clinical care that emphasizes quality of, rather than
quantity of, medical treatment. These rules consider all findings and
other evidence in the record, including those impacted by a person's
level of access to medical care (as well as the preference of some
medical providers to reduce the use of emergency department and
hospital-level medical interventions). The medical equivalence rules
provide some flexibility in determining whether a person is disabled at
step 3 of the sequential evaluation process by considering whether the
person's impairment is at least equal in severity and duration to the
criteria of any listed impairment. If we are unable to find a person's
cardiovascular disorder meets or medically equals a listing, we may
still find the person disabled at the final step of the sequential
evaluation process.\62\ In children, we may still find the person
disabled under our rules for functional equivalence.\63\
---------------------------------------------------------------------------
\60\ 20 CFR 404.1526 and 416.926.
\61\ 20 CFR 416.926a.
\62\ 20 CFR 404.1520(g) and 416.920(g).
\63\ 20 CFR 416.926a.
---------------------------------------------------------------------------
Comment: We received several comments expressing concerns with our
proposed listings that require at least 30-days elapse between
hospitalizations to constitute a separate event. One commenter called
the requirement ``arbitrary.''
Response: We decided to retain the requirement that at least 30
days elapse between hospitalizations to ensure that we are evaluating
separate listing-level episodes of exacerbations or complications. We
disagree that the 30-day requirement is arbitrary in defining separate
events. The Centers for Medicare and Medicaid Services (CMS) uses 30
days between hospitalizations as a benchmark in their regulations. For
example, the CMS defines ``readmission'' as the admission of a person
to the same or another applicable hospital within a time period of 30
days from the date of a previous discharge.\64\ CMS determined that 30
days is a ``clinically meaningful period'' for hospitals to work with
their communities to reduce readmissions by ensuring patients are
clinically ready at discharge and they receive appropriate planning for
follow-up care after discharge.\65\ Further, clinical research often
uses 30 days as a benchmark in studying readmission rates.\66\ While
admission rates may trend lower in the future, the current 30-day
benchmark is an appropriate period to delineate separate events in
evaluating the severity of cardiovascular disorders. In some instances,
hospitalizations that are less than 30 days apart may be evaluated
using our rules for medical equivalence \67\ or our rules for
functional equivalence in children.\68\
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\64\ 42 CFR 412.152.
\65\ 77 FR 53258, 53377 (2012).
\66\ Jiang, H.J., & Hensche, M. (2023). Characteristics of 30-
Day All-Cause Hospital Readmissions, 2016-2020 (Healthcare Cost and
Utilization Project Statistical Brief #304). Agency for Healthcare
Research and Quality. (www.hcup-us.ahrq.gov/reports/statbriefs/sb304-readmissions-2016-2020.pdf); James, J., Tan, S., Stretton, B.,
Kovoor, J.G., Gupta, A.K., Gluck, S., Gilbert, T., Sharma, Y. and
Bacchi, S. (2023). Why do we evaluate 30-day readmissions in general
medicine? A historical perspective and contemporary data. Internal
Medicine Journal, 53(6), 1070-1075. (https://doi.org/10.1111/imj.16115).
\67\ 20 CFR 404.1526 and 416.926.
\68\ 20 CFR 416.926a.
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What is our authority to make rules and set procedures for determining
whether a person is disabled under our statutory definition?
Under the Act, we have authority to make rules and regulations and
to establish necessary and appropriate procedures to carry out such
provisions.\69\ Furthermore, the Act directs us to adopt rules and
regulations that ``provide for the nature and extent of the proofs and
evidence'' needed to establish the right to benefits.\70\
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\69\ See sections 205(a), 702(a)(5), and 1631(d)(1) of the Act
(42 U.S.C. 405(a), 902(a)(5), 1383(d)(1)).
\70\ See sections 205(a) and 1631(d)(1) of the Act (42 U.S.C.
205(a), 1383(d)(1)).
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[[Page 40835]]
How long will this final rule be in effect?
This final rule will remain in effect for 5 years after the date it
becomes effective, unless we extend, revise, or issue it again. We will
continue to monitor this rule to ensure that it continues to meet
program purposes, and we may revise it before the end of the 5-year
period if warranted.
How will we implement this final rule?
We will begin to apply this final rule to new applications, pending
claims, and continuing disability reviews (CDR), as applicable, as of
the effective date of this final rule.\71\
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\71\ We will use the final rule beginning on its effective date.
We will apply the final rule to new applications filed on or after
the effective date, and to claims that are pending on and after the
effective date. This means that we will use the final rule on and
after its effective date in any case in which we make a
determination or decision, including CDRs, as applicable. See 20 CFR
404.901, 404.1590, 416.990, and 416.1401.
---------------------------------------------------------------------------
Regulatory Procedures
Executive Order 12866
We consulted with the Office of Management and Budget (OMB) and
determined that this final rule meets the criteria for a significant
regulatory action under section 3(f) of Executive Order (E.O.) 12866,
and is subject to OMB review. Therefore, OMB reviewed the rule. Details
about the economic impacts of this rule follow.
Anticipated Accounting Costs of This Final Rule
Anticipated Costs to Our Programs
Our Actuarial Services currently estimate that implementation of
the final rule will result in net increases of $446 million in
scheduled Old-Age, Survivors, and Disability Insurance (OASDI) benefit
payments and $94 million in Federal Supplemental Security Income (SSI)
payments for the 10-year period covering fiscal years (FYs) 2026-2035.
This estimate assumes the final rule will be implemented and effective
for all disability determinations made on or after February 1, 2026. At
the time of NPRM publication, Actuarial Services estimated net
increases of $308 million in scheduled OASDI benefits and $71 million
in Federal SSI payments for the 10-year period covering FYs 2022-2031,
and assumed the rule would be effective for all disability
determinations made on or after April 1, 2023.
We note that the 10-year projection period used for all of
Actuarial Services' regulatory estimates is not arbitrarily selected.
The period over which estimates are provided corresponds precisely to
the 10-year projection period in the President's Budget used as the
baseline for the estimates. Therefore, if the assumed effective date of
a regulatory action provided to Actuarial Services is not the beginning
of that 10-year period, then the estimates will cover less than 10
years.
The current estimates are higher than those included in the NPRM
for three primary reasons: (1) the 10-year estimate in the NPRM covered
8 years and 6 months in which the rule was effective, while the 10-year
estimate for this final rule covers 9 years and 8 months in which the
rule is effective, so the estimate for this final rule effectively
includes more than one year of additional OASDI and SSI payments; (2)
the Cost-of-Living Adjustment (COLA) effective for benefits paid in
2023 was 8.7 percent, which was significantly larger than the 2023 COLA
of 2.5 percent that was assumed in the NPRM estimate; and (3) the
estimation window has shifted from 2022-2031 to 2026-2035, so the
average monthly benefits assumed for this final rule are higher than in
the NPRM because of assumed benefit increases over time. The changes we
made from the proposed to the final rule did not cause any estimated
change in allowances.
Anticipated Net Administrative Costs to the Social Security
Administration
SSA's Finance, and Management division estimates a net
administrative savings of less than 15 work years and $2 million
annually.
Anticipated Costs to the Public
We do not believe there are any more than de minimis costs to the
public associated with this rulemaking. As discussed earlier in our
responses to comments on the Notice of Proposed Rulemaking as well as
in the Paperwork Reduction Action section below, the requirements
contained in this rulemaking will not impose new additional costs
outside of the normal course of business for applicants or change how
the public interacts with our disability programs. We do not anticipate
that the requirements contained in the new cardiovascular listings will
impose additional costs or documentation requirements on applicants or
cause the affected applicants to pursue a different course of treatment
than they otherwise would have done under our existing rules.
Anticipated Benefits to the Public
The revisions to the cardiovascular listings update the medical
criteria and clarify how we evaluate cardiovascular disorders. The
revisions also improve the clarity, readability, and application of the
listings as well as consistency across the listings as a whole. The
revisions also help promote uniform national disability policy and can
simplify claims adjudication.
Congressional Review Act
Pursuant to Subtitle E of the Small Business Regulatory Enforcement
Fairness Act of 1996 (also known as the Congressional Review Act, 5
U.S.C. 801 et seq.), the Office of Information and Regulatory Affairs
designated this final rule as not meeting the criteria in 5 U.S.C.
804(2), and it is therefore not a major rule as defined by the
Congressional Review Act.
Executive Order 13132 (Federalism)
We analyzed this final rule in accordance with the principles and
criteria established by E.O. 13132 and determined that it will not have
sufficient Federalism implications to warrant the preparation of a
Federalism assessment. We also determined that the final rule will not
preempt any State law or State regulations or affect the States'
abilities to discharge traditional State governmental functions.
Executive Order 14192
As previously discussed in the NPRM, we consider this rule a
transfer rule with no more than de minimis costs.\72\ As such, it is
not a regulatory action under E.O. 14192.
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\72\ Based upon the criteria established in E.O. 13771, now
superseded by E.O. 14192. For further information see OMB M- Memo
25-20 (``Guidance Implementing Section 3 of Executive Order 14192,
Titled `Unleashing Prosperity Through Deregulation' '').
---------------------------------------------------------------------------
Regulatory Flexibility Act
We certify that this final rule will not have a significant
economic impact on a substantial number of small entities because it
affects individuals only. Therefore, the Regulatory Flexibility Act, as
amended, does not require us to prepare a regulatory flexibility
analysis.
Paperwork Reduction Act
This final rule only updates the criteria in the Listing of
Impairments that we use to evaluate disability claims involving
cardiovascular disorders under titles II and XVI of the Social Security
Act. It does not create any new or affect any existing collections.
Accordingly, this final rule does not impose any burdens under the
Paperwork Reduction Act and does not require further OMB approval.
(Catalog of Federal Domestic Assistance Program Nos. 96.001, Social
Security--Disability Insurance; 96.002, Social
[[Page 40836]]
Security--Retirement Insurance; 96.004, Social Security--Survivors
Insurance; and 96.006, Supplemental Security Income)
List of Subjects
20 CFR Part 404
Administrative practice and procedure; Blind, Disability benefits;
Old-age, survivors, and disability insurance; Reporting and
recordkeeping requirements; Social Security.
20 CFR Part 416
Administrative practice and procedure; Aged, Blind, Disability cash
payments; Public assistance programs; Reporting and recordkeeping
requirements; Supplemental Security Income (SSI).
Mark Steffensen,
General Counsel, Social Security Administration.
For the reasons set out in the preamble, we are amending subpart P
of part 404 of chapter III of title 20 of the Code of Federal
Regulations as set forth below:
PART 404--FEDERAL OLD-AGE, SURVIVORS AND DISABILITY INSURANCE
(1950-)
Subpart P--Determining Disability and Blindness
0
1. The authority citation for subpart P of part 404 continues to read
as follows:
Authority: 42 U.S.C. 402, 405(a)-(b) and (d)-(h), 416(i),
421(a) and (h)-(j), 422(c), 423, 425, 902(a)(5), and 1320e-3; sec.
211(b), Pub. L. 104-193, 110 Stat. 2105, 2189; sec. 202, Pub. L.
108-203, 118 Stat. 509 (42 U.S.C. 902 note).
0
2. Amend appendix 1 to subpart P of part 404 as follows:
0
a. Revise item 5 of the introductory text before part A;
0
b. Revise the body system name for section 4.00 in the table of
contents in part A;
0
c. Amend section 1.00 by revising paragraph 1.00B5;
0
d. Revise and republish section 4.00;
0
e. Revise the body system name for section 104.00 in the table of
contents in part B;
0
f. Revise and republish section 104.00;
0
g. Amend section 114.00 by revising paragraph 114.00J2m.
The revisions read as follows:
Appendix 1 to Subpart P of Part 404--Listing of Impairments
* * * * *
5. Cardiovascular Disorders (4.00 and 104.00): October 30, 2031.
* * * * *
Part A
Sec.
* * * * *
4.00 Cardiovascular Disorders
* * * * *
1.00 Musculoskeletal Disorders
* * * * *
B. * * *
5. We evaluate leg pain associated with peripheral vascular
claudication and foot ulceration associated with peripheral artery
disease under the listings in 4.00.
* * * * *
4.00 Cardiovascular Disorders
A. How do we define cardiovascular disorders and cardiovascular
terms?
1. What do we mean by a cardiovascular disorder?
a. We mean any disorder that affects the proper functioning of
the heart or the circulatory system (that is, arteries, veins,
capillaries, and the lymphatic drainage). The disorder can be
congenital or acquired.
b. Cardiovascular disorders result from one or more of four
consequences of heart disease:
(i) Chronic heart failure (chronic HF) or ventricular
dysfunction.
(ii) Discomfort or pain due to myocardial ischemia, with or
without necrosis of the heart muscle.
(iii) 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.
(iv) Hypoxemia (reduced oxygen concentration in the blood) due
to right-to-left shunt or pulmonary vascular disease.
c. Disorders of the veins or arteries (for example, obstruction,
rupture, or aneurysm) may cause impairments of the lower extremities
(peripheral vascular disease), the central nervous system, the eyes,
the kidneys, and other organs. We will evaluate peripheral vascular
disease under 4.11 or 4.12 and impairments of another body system(s)
under the listings for that body system(s).
2. What do we consider in evaluating cardiovascular disorders?
The listings in this section describe cardiovascular disorders based
on the medical and other evidence, including response to a regimen
of prescribed treatment and functional limitations.
3. What do the following terms or phrases mean in these
listings?
a. Medical consultant is a person defined in Sec. Sec.
404.1616(a) and 416.1016(a) of this chapter. This term does not
include medical sources who provide consultative examinations for
us. We use the abbreviation ``MC'' throughout this section to
designate a medical consultant.
b. Persistent means that the longitudinal clinical record shows
that, with few exceptions, the required finding(s) has been present,
or is expected to be present, for a continuous period of at least 12
months, such that a pattern of continuing severity is established.
By ``exceptions,'' we mean brief periods when the required
finding(s) is greatly reduced or gone. These periods are so brief or
inconsequential, the required finding(s) remains a factor in the
person's condition.
c. Recurrent means that the longitudinal clinical record shows
that, within a consecutive 12-month period, the finding(s) occurs at
least three times, with intervening periods of improvement of
sufficient duration that it is clear that separate events are
involved. By ``improvement of sufficient duration,'' we mean the
finding is greatly reduced or not present for long enough that the
required finding(s) is no longer a factor in the person's condition.
d. Appropriate medically acceptable imaging means that the
technique used is the proper one to evaluate and diagnose the
impairment and is commonly recognized as accurate for assessing the
cited finding.
e. A consecutive 12-month period means a period of 12
consecutive months, all or part of which must occur within the
period we are considering in connection with an application or
continuing disability review.
B. What documentation do we need to evaluate cardiovascular
disorders?
1. What basic documentation do we need? We need sufficiently
detailed reports of history, physical examinations, laboratory
studies, and any prescribed treatment and response to allow us to
assess the severity and duration of your cardiovascular disorder. A
longitudinal clinical record covering a period of not less than 3
months of observations and treatment is usually necessary, unless we
can make a determination or decision based on the current evidence
we already have.
2. Why is a longitudinal clinical record important? We will
usually need a longitudinal clinical record to assess the severity
and expected duration of your impairment(s). If you have a listing-
level impairment, you probably will have received medically
prescribed treatment. Whenever there is evidence of such treatment,
your longitudinal clinical record should include a description of
the ongoing management and evaluation provided by your medical
source(s). It should also include your response to this medical
management, as well as information about the nature and severity of
your impairment. The record will provide us with information on your
functional status over an extended period of time and show whether
your ability to function is improving, worsening, or unchanging.
3. What if you have not received ongoing medical treatment?
a. You may not have received ongoing treatment or have an
ongoing relationship with the medical community despite the
existence of a severe impairment(s). In this situation, we will base
our evaluation on the current evidence we have. If you do not
receive treatment, you cannot show an impairment that meets the
criteria of most of these listings. However, we may find you
disabled because you have another impairment(s) that, in combination
with your cardiovascular disorder, medically equals a listing, or we
may find you disabled at a later step in the evaluation process
based on a consideration of your residual functional
[[Page 40837]]
capacity and age, education, and work experience.
b. Unless we can decide your claim favorably on the basis of the
current evidence we already have, a longitudinal record is still
important. In instances when there is no or insufficient
longitudinal evidence, we may purchase a consultative examination(s)
to help us establish the existence, severity, and duration of your
impairment.
4. When will we wait before we ask for more evidence?
a. We will wait when we have information showing that your
impairment is not yet stable and the expected change in your
impairment might affect our determination or decision. In these
situations, we need to wait to properly evaluate the severity and
duration of your impairment during a stable period. Examples of when
we might wait are:
(i) If you have had a recent acute event; for example, a
myocardial infarction (heart attack);
(ii) If you have recently had a corrective cardiac procedure;
for example, coronary artery bypass grafting;
(iii) If you have started new drug therapy and your response to
this treatment has not yet been established; for example, beta-
blocker therapy for dilated cardiomyopathy.
b. In these situations, we will obtain more evidence 3 months
following the event before we evaluate your impairment. However, we
will not wait if we have enough information to make a determination
or decision based on all of the relevant evidence in your case.
5. Will we purchase any studies? In appropriate situations, we
may purchase studies necessary to substantiate the existence of a
medically determinable impairment or to document the severity of
your impairment, generally after we have evaluated the evidence we
already have. We will not purchase studies involving exercise
testing if there is significant risk involved or if there is another
medical reason not to perform the test. We will follow 4.00C6,
4.00C7, and 4.00C8 when we decide whether to purchase exercise
testing.
6. What studies will we not purchase? We will not purchase any
studies involving cardiac catheterization, such as coronary
angiography, arteriograms, or electrophysiological studies. However,
if the results of a catheterization are part of the existing
evidence we have, we will consider them together with the other
relevant evidence. See 4.00C15a.
C. How do we use cardiovascular test results?
1. What is an ECG?
a. ECG stands for electrocardiograph or electrocardiogram. An
electrocardiograph is a machine that records electrical impulses of
your heart on a strip of paper called an electrocardiogram or a
tracing. To record the ECG, a technician positions a number of small
contacts (or leads) on your arms, legs, and across your chest to
connect them to the ECG machine. An ECG may be done while you are
resting or exercising.
b. The ECG tracing may indicate that you have a heart
abnormality. It may indicate that your heart muscle is not getting
as much oxygen as it needs (ischemia), that your heart rhythm is
abnormal (arrhythmia), or that there are other abnormalities of your
heart, such as left ventricular enlargement.
How do we evaluate ECG evidence? We consider a number of factors
when we evaluate ECG evidence:
a. An original or legible copy of the 12-lead ECG obtained at
rest must be 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.
(i) Detailed descriptions or computer-averaged signals without
original or legible copies of the ECG as described in 4.00C2a are
not acceptable.
(ii) The effects of drugs or electrolyte abnormalities must be
considered as possible noncardiac causes of ECG abnormalities of
ventricular repolarization; that is, those involving the ST segment
and T wave. If available, the predrug (especially digitalis
glycosides) ECG should be submitted.
b. ECGs obtained in conjunction with treadmill, bicycle, or arm
exercise tests should meet the following specifications:
(i) ECG reports must include the original calibrated ECG
tracings or a legible copy;
(ii) A 12-lead baseline ECG must be recorded in the upright
position before exercise;
(iii) A 12-lead ECG should be recorded at the end of each minute
of exercise;
(iv) 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-recorded response to exercise;
(v) Post-exercise ECGs should be recorded using a generally
accepted protocol consistent with the prevailing state of medical
knowledge and clinical practice; and
(vi) All resting, exercise, and recovery ECG strips must have
the standardization inscribed on the tracing. The ECG strips should
be labeled to indicate the date, 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, heart
rate and blood pressure levels during testing, and the reason(s) for
terminating the test (including limiting signs or symptoms) must be
recorded.
3. What are exercise tests and what are they used for?
a. Exercise tests have you perform physical activity and record
how your cardiovascular system responds. Exercise tests usually
involve walking on a treadmill, but other forms of exercise, such as
an exercise bicycle or an arm exercise machine, may be used.
Exercise testing may be done for various reasons, such as to
evaluate the severity of your coronary artery disease or peripheral
vascular disease or to evaluate your progress after a cardiac
procedure or an acute event, like a myocardial infarction (heart
attack). Exercise testing is the most widely used testing for
identifying the presence of myocardial ischemia and for estimating
maximal aerobic capacity (usually expressed in METs--metabolic
equivalents) if you have heart disease.
b. We include exercise tolerance test (ETT) criteria in 4.02B2,
4.04A, 4.06B, and 4.08A2. To meet the ETT criteria in these
listings, the ETT must be a sign- or symptom-limited test in which
you exercise while connected to an ECG until you develop a sign or
symptom that indicates that you have exercised as much as is
considered safe for you.
c. In 4.12B, we also refer to exercise testing for peripheral
vascular disease. In this test, you walk on a treadmill, usually for
a specified period of time, and the person who administers the test
measures the effect of exercise on the flow of blood in your legs,
usually by using ultrasound. The test is also called an exercise
Doppler test. Even though this test is intended to evaluate
peripheral vascular disease, it will be stopped for your safety if
you develop abnormal signs or symptoms because of heart disease.
d. Each type of test is done in a certain way following specific
criteria, called a protocol. For our program, we also specify
certain aspects of how any exercise test we purchase is to be done.
See 4.00C10 and 4.00C17.
4. Do ETTs have limitations? An ETT provides an estimate of
aerobic capacity for walking on a grade, bicycling, or moving one's
arms in an environmentally controlled setting. Therefore, ETT
results do not correlate with the ability to perform other types of
exertional activities, such as lifting and carrying heavy loads, and
do not provide an estimate of the ability to perform activities
required for work in all possible work environments or throughout a
workday. Also, certain medications (such as beta blockers) and
conduction disorders (such as left or right bundle branch blocks)
can cause false-negative or false-positive results. Therefore, we
must consider the results of an ETT together with all the other
relevant evidence in your case record.
5. How does an ETT with measurement of maximal or peak oxygen
uptake (VO2) differ from other ETTs? Occasionally,
medical evidence will include the results of an ETT with
VO2, which is also called a cardiopulmonary exercise
test. While ETTs without measurement of VO2 provide only
an estimate of aerobic capacity, measured maximal or peak oxygen
uptake provides an accurate measurement of aerobic capacity, which
is often expressed in METs (metabolic equivalents). The MET level
may not be indicated in the report of attained maximal or peak
VO2 testing, but can be calculated as follows: 1 MET =
3.5 milliliters (ml) of oxygen uptake per kilogram (kg) of body
weight per minute. For example, a 70 kg (154 lb.) person who
achieves a maximal or peak VO2 of 1225 ml in 1 minute has
attained 5 METs (1225 ml/70 kg/1 min = 17.5 ml/kg/min. 17.5/3.5 = 5
METs). In listings 4.02B2, 4.06B, and 4.08A2, we use the peak
VO2 (oxygen uptake) measurement when evaluating the
results of a cardiopulmonary exercise test.
6. When will we consider whether to purchase an exercise test?
a. We will consider whether to purchase an exercise test when:
(i) There is a question whether your cardiovascular disorder
meets or medically equals the severity of one of the listings, or
there is no timely test in the evidence we
[[Page 40838]]
have (see 4.00C9), and we cannot find you disabled on some other
basis; or
(ii) We need to assess your residual functional capacity and
there is insufficient evidence in the record to make a determination
or decision.
b. We will not purchase an exercise test when we can make our
determination or decision based on the evidence we already have.
7. What must we do before purchasing an exercise test?
a. Before we purchase an exercise test, an MC, preferably one
with experience in the care of patients with cardiovascular disease,
must review the pertinent history, physical examinations, and
laboratory tests that we have to determine whether the test would
present a significant risk to you or if there is some other medical
reason not to purchase the test (see 4.00C8).
b. If you are under the care of a medical source (see Sec. Sec.
404.1502 and 416.902 of this chapter) for a cardiovascular disorder,
this source has not performed an exercise test, and there are no
reported significant risks to testing, we will request a statement
from that source explaining why it was not done or should not be
done before we decide whether we will purchase the test.
c. The MC, in accordance with the regulations and other
instructions on consultative examinations, will generally not
override the medical source's conclusion about the risk of exercise
testing to you. In the rare situation in which the MC does override
the medical source's conclusion, the MC must prepare a written
rationale documenting the reasons for overriding the conclusion.
d. If you do not have a medical source or we cannot obtain a
statement from your medical source, the MC is responsible for
assessing the risk of exercise testing based on a review of the
records we have before purchasing an exercise test for you.
e. We must also provide your records to the medical source who
performs the exercise test for review prior to conducting the test
if the source does not already have them. The medical source who
performs the exercise test has the ultimate responsibility for
deciding whether you would be at risk.
8. When will we not purchase an exercise test or wait before we
purchase an exercise test?
a. We will not purchase an exercise test when an MC finds that
you have one of the following significant risk factors:
(i) Unstable angina not previously stabilized by medical
treatment;
(ii) Uncontrolled cardiac arrhythmias causing symptoms or
hemodynamic compromise;
(iii) An implanted cardiac defibrillator;
(iv) Symptomatic severe aortic stenosis;
(v) Uncontrolled symptomatic heart failure;
(vi) Aortic dissection;
(vii) Severe pulmonary hypertension (pulmonary artery systolic
pressure greater than 60 mm Hg);
(viii) Left main coronary stenosis of 50 percent or greater that
has not been bypassed;
(ix) Moderate stenotic valvular disease with a systolic gradient
across the aortic valve of 50 mm Hg or greater;
(x) Severe arterial hypertension (systolic greater than 200 mm
Hg or diastolic greater than 110 mm Hg); or
(xi) Hypertrophic cardiomyopathy with a systolic gradient of 50
mm Hg or greater.
b. We also will not purchase an exercise test when you are
prevented from performing exercise testing due to another impairment
affecting your ability to use your arms and legs.
c. We will not purchase an ETT to document the presence of a
cardiac arrhythmia.
d. We will wait to purchase an exercise test until 3 months
after you have had one of the following events. This will allow for
maximal, attainable restoration of functional capacity.
(i) Acute myocardial infarction;
(ii) Surgical myocardial revascularization (bypass surgery);
(iii) Other open-heart surgical procedures; or
(iv) Percutaneous transluminal coronary angioplasty (PTCA) or
percutaneous coronary intervention (PCI) with or without stenting.
e. If you are deconditioned after an extended period of bedrest
or inactivity and could improve with activity, or if you are in
acute heart failure and are expected to improve with treatment, we
will wait an appropriate period of time until you are ready and
there are no medical reasons that prevent us from purchasing an
exercise test.
9. What do we mean by a ``timely'' test?
a. We consider exercise test results to be timely for 12 months
after the date they are performed, provided there has been no change
in your clinical status that may alter the severity of your
cardiovascular disorder.
b. However, an exercise test that is older than 12 months,
especially an abnormal one, can still provide information important
to our adjudication. For example, a test that is more than 12 months
old can provide evidence of ischemic heart disease or peripheral
vascular disease, information on decreased aerobic capacity, or
information about the duration or onset of your impairment. Such
tests can be an important component of the longitudinal record.
c. When we evaluate a test that is more than 12 months old, we
must consider the results in the context of all the relevant
evidence, including why the test was performed and whether there has
been an intervening event or improvement or worsening of your
impairment.
d. We will purchase a new exercise test only if we cannot make a
determination or decision based on the evidence we have.
10. How must ETTs we purchase be performed?
a. The ETT must be a sign- or symptom-limited test characterized
by a progressive multistage regimen. It 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 4.00C2b and this section. A radionuclide
perfusion scan may be useful for detecting or confirming ischemia
when resting ECG abnormalities, medications, or other factors may
decrease the accuracy of ECG interpretation of ischemia. (The
perfusion imaging is done at the termination of exercise, which may
be at a higher MET level than that at which ischemia first occurs.
If the imaging confirms the presence of reversible ischemia, the
exercise ECG may be useful for detecting the MET level at which
ischemia initially appeared.) Exercise tests may also be performed
using echocardiography to detect stress-induced ischemia and left
ventricular dysfunction (see 4.00C12 and 4.00C13).
b. The exercise test must be paced to your capabilities and be
performed following the generally accepted standards for adult
exercise test laboratories. 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 should use similar workloads. The exercise protocol may
need to be modified in individual cases to allow for a lower initial
workload with more slowly graded increments than the standard Bruce
protocol.
c. Levels of exercise must be described in terms of workload and
duration of each stage; for example, treadmill speed and grade, or
bicycle ergometer work rate in kpm/min or watts.
d. The exercise laboratory's physical environment, staffing, and
equipment must meet the generally accepted standards for adult
exercise test laboratories.
11. How do we evaluate ETT results? We evaluate ETT results on
the basis of the work level at which the test becomes abnormal, as
documented by onset of signs or symptoms and any ECG or imaging
abnormalities. The absence of an ischemic response on an ETT alone
does not exclude the diagnosis of ischemic heart disease. We must
consider the results of an ETT in the context of all of the other
evidence in your case record.
12. When are ETTs done with imaging? When resting ECG
abnormalities preclude interpretation of ETT tracings relative to
ischemia, a radionuclide (for example, thallium-201 or technetium-
99m) perfusion scan or echocardiography in conjunction with an ETT
provides better results. You may have resting ECG abnormalities when
you have a conduction defect--for example, Wolff-Parkinson-White
syndrome, left bundle branch block, left ventricular hypertrophy--or
when you are taking digitalis or other antiarrhythmic drugs, or when
resting ST changes are present. Also, these techniques can provide a
reliable estimate of ejection fraction.
13. Will we purchase ETTs with imaging? We may purchase an ETT
with imaging in your case after an MC, preferably one with
experience in the care of patients with cardiovascular disease, has
reviewed your medical history and physical examination, any
report(s) of appropriate medically acceptable imaging, ECGs, and
other appropriate tests. We will consider purchasing an ETT with
imaging when other information we have is not adequate for us to
assess whether you have severe ventricular dysfunction or myocardial
ischemia, there is no significant risk involved (see 4.00C8a), and
we cannot make our determination or decision based on the evidence
we already have.
[[Page 40839]]
14. What are drug-induced stress tests? These tests are designed
primarily to provide evidence about myocardial ischemia or prior
myocardial infarction, but do not require you to exercise. These
tests are used when you cannot exercise or cannot exercise enough to
achieve the desired cardiac stress. Drug-induced stress tests can
also provide evidence about heart chamber dimensions and function;
however, these tests do not provide information about your aerobic
capacity and cannot be used to help us assess your ability to
function. Some of these tests use agents, such as Persantine or
adenosine, that dilate the coronary arteries and are used in
combination with nuclear agents, such as thallium or technetium (for
example, Cardiolyte or Myoview), and a myocardial scan. Other tests
use agents, such as dobutamine, that stimulate the heart to contract
more forcefully and faster to simulate exercise and are used in
combination with a 2-dimensional echocardiogram. We may, when
appropriate, purchase a drug-induced stress test to confirm the
presence of myocardial ischemia after a review of the evidence in
your file by an MC, preferably one with experience in the care of
patients with cardiovascular disease.
15. How do we evaluate cardiac catheterization evidence?
a. We will not purchase cardiac catheterization; however, if you
have had catheterization, we will make every reasonable effort to
obtain the report and any ancillary studies. We will consider the
quality and type of data provided and its relevance to the
evaluation of your impairment.
b. Cardiac catheterization reports commonly include an
evaluation of each main coronary artery and whether there is
evidence of obstruction, as well as the grade of obstruction
(percent stenosis). Cardiac catheterization reports may include
information about the pressures of left and right side of the heart,
evidence of coronary artery size and flow patterns, and chamber
size. These reports may also include evaluation of left ventricular
wall motion, left ventricular ejection fraction (left
ventriculography), fractional flow reserve (FFR) (a measure of flow
access across an obstruction), and the instantaneous wave-free ratio
(iFR) (a measure of stenosis severity).
16. What details should exercise Doppler test reports contain?
The reports of exercise Doppler tests must describe the level of
exercise; for example, the speed and grade of the treadmill
settings, the duration of exercise, changes in the person's
condition during exercise (including the presence and location of
leg symptoms), and the reasons for stopping exercise if the expected
level of exercise was not attained. If the exercise Doppler test was
done to evaluate peripheral vascular disease, the report must also
provide the blood pressures at the ankle and other pertinent sites
measured after exercise, and also provide the time required for the
systolic blood pressure to return toward or to the pre-exercise
level. The graphic tracings, if available, should also be included
with the report. All tracings must be annotated with the
standardization used by the testing facility.
17. How must exercise Doppler tests we purchase be performed?
When we purchase an exercise Doppler test, you must exercise on a
treadmill at 2 mph on a 12 percent grade for up to 5 minutes. The
reports must include the information specified in 4.00C16. Because
this is an exercise test, we must evaluate whether such testing
would put you at significant risk, in accordance with the guidance
found in 4.00C6, 4.00C7, and 4.00C8.
D. How do we evaluate chronic heart failure?
1. What is chronic heart failure (chronic HF)?
a. Heart failure is the inability of the heart to pump enough
oxygenated blood to body tissues. This syndrome is characterized by
symptoms and signs of pulmonary or systemic congestion (fluid
retention) or limited cardiac output. Certain laboratory findings of
cardiac functional and structural abnormality support the diagnosis
of chronic HF. Ejection fraction (EF) is the percentage of the blood
in the ventricle actually pumped out with each contraction. EF in
heart failure is a continuum ranging from low EF due to muscle
dysfunction to preserved EF resulting from high intracardiac
pressures. We consider heart failure to be chronic when the
condition persists or recurs over time despite treatment. There are
two main types of chronic HF:
(i) Heart failure with reduced EF (HFrEF) or predominant
systolic dysfunction is characterized by the inability of the heart
to contract normally and expel sufficient blood due to a dilated,
poorly contracting left ventricle, and
(ii) Heart failure with preserved EF (HFpEF) or predominant
diastolic dysfunction is characterized by the inability of the heart
to relax and fill normally due to a thickened ventricular muscle,
poor ability of the left ventricle to distend, and increased
ventricular filling pressure.
b. Chronic HF is considered in these listings as a single
category whether due to atherosclerosis (narrowing of the arteries),
cardiomyopathy, hypertension, or rheumatic, congenital, or other
heart disease. If the chronic HF is the result of primary pulmonary
hypertension secondary to disease of the lung, we evaluate your
impairment under the listings in 3.00 (for example, 3.09) or 4.00,
as appropriate. For the purposes of 4.02B3, a finding of elevated B-
type natriuretic peptide (BNP) or N-terminal pro-B-type natriuretic
peptide (NT-proBNP) in the blood assists in differentiating chronic
HF from non-heart failure symptoms.
2. What evidence of chronic HF do we need?
a. Cardiomegaly or ventricular dysfunction must be present and
demonstrated by appropriate medically acceptable imaging, such as
cardiac magnetic resonance imaging (MRI), chest x-ray,
echocardiography (M-Mode, 2-dimensional, and Doppler), radionuclide
studies, or cardiac catheterization.
(i) Abnormal cardiac imaging provides objective measures of both
left ventricular function and structural abnormality in heart
failure. Examples of abnormal findings include increased left
ventricular end-diastolic dimension (LVEDD), decreased EF, increased
left atrial chamber size, increased left atrial volume index (LAVI),
increased ventricular filling pressures measured at cardiac
catheterization, or increased left ventricular wall or septum
thickness.
(ii) An LVEDD greater than 6.8 cm for males or 6.1 cm for
females, or an EF of 30 percent or less during a period of stability
(that is, not during an episode of exacerbation of heart failure)
may be associated clinically with systolic dysfunction.
(iii) LAVI is measured in milliliters (ml) indexed to body
surface area (BSA) measured in squared meters (m\2\). Indexing is a
method of standardizing measurements to different body sizes.
Diastolic dysfunction may be clinically associated with LAVI of 40
ml, BSA/m\2\ or greater. The imaging report will contain a
measurement for the left atrium volume. The index is calculated by
dividing the left atrium volume by BSA.
(iv) However, these measurements alone do not reflect your
functional capacity, which we evaluate by considering all of the
relevant evidence. In some situations, we may need to purchase an
ETT to help us assess your functional capacity.
(v) Other findings on appropriate medically acceptable imaging
may include increased pulmonary vascular markings, pleural effusion,
and pulmonary edema. These findings need not be present on each
report, since chronic HF may be controlled by prescribed treatment.
(vi) Other findings on an echocardiogram report (such as an
LVEDD indexed to BSA) may be at least of medically equal
significance to required findings in the listing criteria. These
findings may be evaluated under our medical equivalence policy, as
appropriate. See Sec. Sec. 404.1526 and 416.926 of this chapter.
b. Your medical history and physical examination should describe
characteristic symptoms and signs of pulmonary or systemic
congestion (fluid retention) or of limited cardiac output associated
with the abnormal findings on appropriate medically acceptable
imaging. When an acute episode of heart failure is triggered by a
remediable factor, such as an arrhythmia, dietary sodium overload,
or high altitude, cardiac function may be restored and a chronic
impairment may not be present.
(i) Symptoms of congestion or of limited cardiac output include
easy fatigue, weakness, shortness of breath (dyspnea), cough, or
chest discomfort at rest or with activity. People with chronic HF
may also experience shortness of breath upon lying flat (orthopnea)
or episodes of shortness of breath that wake them from sleep
(paroxysmal nocturnal dyspnea). They may also experience cardiac
arrhythmias resulting in palpitations, lightheadedness, or fainting.
(ii) Signs of congestion may include hepatomegaly, ascites,
increased jugular venous distention or pressure, rales, peripheral
edema, or rapid weight gain. However, these signs need not be found
on all examinations because congestion may be controlled by
prescribed treatment or may not be present at the time of
evaluation.
[[Page 40840]]
3. Is it safe for you to have an ETT if you have chronic HF? The
presence of chronic HF is not necessarily a contraindication to an
ETT, unless you are having an acute episode of heart failure.
Measures of cardiac performance are valuable in helping us evaluate
your ability to do work-related activities. ETT has been used safely
in people with chronic HF. Therefore, we may purchase an ETT for
evaluation under 4.02B2 if an MC, preferably one experienced in the
care of patients with cardiovascular disease, determines that the
test poses no significant risk to you. ST segment changes from
digitalis use in the treatment of chronic HF do not preclude the
purchase of an ETT. (See 4.00C6 for when we will consider the
purchase of an ETT. See 4.00C7-4.00C8 for what we must do before we
purchase an ETT and when we will not purchase one.)
4. How do we evaluate chronic HF using 4.02?
a. We must have objective evidence, as described in 4.00D2, that
you have chronic HF.
b. To meet the required level of severity for this listing, your
impairment must satisfy the requirements of the criteria in A and B
or satisfy either C or D.
c. In 4.02B1, we follow a two-part process to evaluate your
impairment. Your impairment must satisfy the requirements in the
first part of this process before we will move to the second part.
(i) Your impairment satisfies the first part if a medical source
has concluded that the performance of an ETT would present a
significant risk to you. This medical source, such as a
cardiologist, may be providing your care. If your case record does
not include a conclusion from a medical source that an ETT would
present a significant risk to you, an MC as defined in 4.00A3a may
make such a conclusion if evidence in your case record supports it.
(ii) In the second part of the process, we will evaluate
activities of daily living (ADL). ADLs include, but are not limited
to, such activities as doing household chores, grooming and hygiene,
shopping at a grocery store, taking public transportation, or paying
bills. We will assess whether you have persistent symptoms of
chronic heart failure (for example, easy fatigue, weakness,
shortness of breath, or chest discomfort) at rest or with activity
that very seriously limit your ability to perform ADLs
independently, appropriately, effectively, and on a sustained basis.
Even if you are able to perform some ADLs, we may find your ability
is very seriously limited and that your impairment satisfies the
second part of the evaluation.
d. Listing 4.02B2b requires a decrease in systolic blood
pressure below the baseline level or below any systolic pressure
reading recorded during exercise. We have this requirement because,
normally, systolic blood pressure and heart rate increase gradually
with exercise. Decreases in systolic blood pressure below the
baseline level that occur during exercise are often associated with
ischemia-induced left ventricular dysfunction resulting in decreased
cardiac output. However, a blunted response (that is, failure of the
systolic blood pressure to rise 10 mm Hg or more), particularly in
the first 3 minutes of exercise, may be drug-related and is not
necessarily associated with left ventricular dysfunction. Also, some
people with increased sympathetic responses because of
deconditioning or apprehension may increase their systolic blood
pressure and heart rate above their baseline level just before and
early into exercise. This can be associated with a drop in systolic
pressure in early exercise that is not due to left ventricular
dysfunction. Therefore, an early decrease in systolic blood pressure
must be interpreted within the total context of the test; that is,
the presence or absence of symptoms such as lightheadedness,
ischemic changes, or arrhythmias on the ECG.
e. How do we evaluate chronic HF treated with a mechanical
circulatory support device? We use 4.02D1 to evaluate chronic HF
treated with an implanted mechanical circulatory support device
(MCSD), such as a left ventricle assistive device (LVAD) or a right
ventricle assistive device (RVAD). Implanted MCSDs are intended for
long-term circulatory support in helping the heart pump blood. For
the purposes of 4.02D1, an MCSD does not include extracorporeal
membrane oxygenation (ECMO) or devices using Impella technology.
Although these are forms of mechanical circulatory support, we do
not include them in 4.02D1 because they are intended only for short-
term circulatory support (maximum 30 days), used in a setting of
imminent or actual cardiac arrest.
E. How do we evaluate ischemic heart disease?
1. What is ischemic heart disease (IHD)? IHD is a condition in
which the heart muscle does not receive enough blood to function
normally (ischemia). Ischemia can result when one or more of your
coronary arteries is narrowed or obstructed or, in rare situations,
constricted due to vasospasm. The obstruction may be the result of
an embolus, a thrombus, or plaque. When heart muscle tissue dies as
a result of the reduced blood supply, it is called a myocardial
infarction (heart attack). In the absence of coronary artery
obstruction, ischemia can also occur due to coronary microvascular
dysfunction, which results in the heart muscle getting insufficient
oxygen.
2. What causes chest discomfort of myocardial origin?
a. Chest discomfort of myocardial ischemic origin, commonly
known as angina pectoris, is usually caused by obstructive coronary
artery disease (CAD). However, ischemic discomfort may be caused by
a noncoronary artery impairment, such as aortic stenosis,
hypertrophic cardiomyopathy, pulmonary hypertension, or anemia.
Ischemic chest discomfort may also be caused by microvascular
coronary artery dysfunction with non-obstructive CAD or in the
absence of CAD.
b. Instead of typical angina pectoris, some people with IHD
experience atypical angina, anginal equivalent, variant angina, or
silent ischemia, all of which we may evaluate using 4.04. We discuss
the various manifestations of ischemia in 4.00E3-4.00E7.
3. What are the characteristics of typical angina pectoris?
Discomfort of myocardial ischemic origin (angina pectoris) is
discomfort that is precipitated by effort or emotion and promptly
relieved by rest, sublingual nitroglycerin (that is, nitroglycerin
tablets that are placed under the tongue), or other rapidly acting
nitrates. Typically, the discomfort is located in the chest (usually
substernal) and described as pressing, crushing, squeezing, burning,
aching, or oppressive. Sharp, sticking, or cramping discomfort is
less common. 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 treatment or rest.
4. What is atypical angina? Atypical angina describes discomfort
or pain from myocardial ischemia that is felt in places other than
the chest. The common sites of cardiac pain are the inner aspect of
the left arm, neck, jaw(s), upper abdomen, and back, but the
discomfort or pain can be elsewhere. When pain of cardiac ischemic
origin presents in an atypical site in the absence of chest
discomfort, the source of the pain may be difficult to diagnose. To
represent atypical angina, your discomfort or pain should have
precipitating and relieving factors similar to those of typical
chest discomfort, and we must have objective medical evidence of
myocardial ischemia; for example, ECG or ETT evidence or appropriate
medically acceptable imaging.
5. What is anginal equivalent? Often, people with IHD will
complain of shortness of breath (dyspnea) on exertion without chest
pain or discomfort. In a minority of such situations, the shortness
of breath is due to myocardial ischemia; this is called anginal
equivalent. To represent anginal equivalent, your shortness of
breath should have precipitating and relieving factors similar to
those of typical chest discomfort, and we must have objective
medical evidence of myocardial ischemia; for example, ECG or ETT
evidence or appropriate medically acceptable imaging. In these
situations, it is essential to establish objective evidence of
myocardial ischemia to ensure that you do not have effort dyspnea
due to non-ischemic or non-cardiac causes.
6. What is variant angina?
a. Variant angina (Prinzmetal's angina, vasospastic angina)
refers to the occurrence of anginal episodes at rest, especially at
night, accompanied by transitory ST segment elevation (or, at times,
ST depression) on an ECG. It is due to severe spasm of a coronary
artery, causing ischemia of the heart wall, and is often accompanied
by major ventricular arrhythmias, such as ventricular tachycardia.
We will consider variant angina under 4.04 only if you have spasm of
a coronary artery in relation to an obstructive lesion of the
vessel. If you have an arrhythmia as a result of variant angina, we
may consider your impairment under 4.05.
b. Variant angina may also occur in the absence of obstructive
CAD. In this situation, an ETT will not demonstrate ischemia. The
diagnosis will be established by showing the typical transitory ST
segment changes during attacks of pain, and the absence of
obstructive lesions shown by catheterization. In the absence of
obstructive CAD, the treatment of ischemia due to coronary
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vasospasm or microvascular dysfunction is limited to medications
such as calcium channel blockers, statins, and nitrates. In such
situations, we will consider the frequency of anginal episodes
despite prescribed treatment when evaluating your residual
functional capacity.
c. Vasospasm that is catheter-induced during coronary
angiography is not variant angina.
7. What is silent ischemia?
a. Myocardial ischemia, and even myocardial infarction, can
occur without perception of pain or any other symptoms; when this
happens, we call it silent ischemia. Pain sensitivity may be altered
by a variety of diseases, most notably diabetes mellitus and other
neuropathic disorders. People also vary in their threshold for pain.
b. Silent ischemia occurs most often in:
(i) People with documented past myocardial infarction or
established angina without prior infarction who do not have chest
pain on ETT, but have a positive test with ischemic abnormality on
ECG, perfusion scan, or other appropriate medically acceptable
imaging.
(ii) People with documented past myocardial infarction or angina
who have ST segment changes on ambulatory monitoring (Holter
monitoring) that are similar to those that occur during episodes of
angina. ST depression shown on the ambulatory recording should not
be interpreted as positive for ischemia unless similar depression is
also seen during chest pain episodes annotated in the diary that the
person keeps while wearing the Holter monitor.
(iii) People who have diabetes mellitus with neuropathy. People
with diabetes mellitus can have a higher threshold for pain because
of the neuropathy and may not feel chest pain or discomfort from
cardiac ischemia.
c. ST depression can result from a variety of factors, such as
postural changes and variations in cardiac sympathetic tone. In
addition, there are differences in how different Holter monitors
record the electrical responses. Therefore, we do not consider the
Holter monitor reliable for the diagnosis of silent ischemia except
in the situation described in 4.00E7b(ii).
8. What other sources of chest discomfort are there? Chest
discomfort of nonischemic origin may result from other
cardiovascular disorders, such as pericarditis. Noncardiac disorders
may also produce symptoms mimicking that of myocardial ischemia.
These disorders include acute anxiety or panic attacks,
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
spine arthritis). Hyperventilation may also mimic ischemic
discomfort. Thus, in the absence of documented myocardial ischemia,
such disorders should be considered as possible causes of chest
discomfort.
9. How do we evaluate IHD using 4.04?
a. We must have objective evidence, as described under 4.00C,
that your symptoms are due to myocardial ischemia.
b. In 4.04A, we need evidence, such as an ECG interpretation,
from an acceptable medical source who reviewed your ETT findings and
found them positive for ischemia. These ETT findings may include ECG
tracings or systolic blood pressure measurements. If your case
record does not have such an interpretation from an acceptable
medical source, an MC, as defined in 4.00A3a, may review your ETT
findings and interpret them as being positive for ischemia if
evidence in your case record supports it.
(i) ETT findings may show the classically accepted changes in
ECG tracings of horizontal or down sloping ST depression or of ST
elevation. For example, ECG tracings may show horizontal or down
sloping depression, in the absence of digitalis glycoside treatment
or hypokalemia, of the ST segment of at least -0.10 millivolts (-1.0
mm) in at least three consecutive complexes that are on a level
baseline in any lead other than a VR, and depression of at least -
0.10 millivolts lasting for at least 1 minute of recovery.
Alternatively, the ECG tracings may show at least 0.10 millivolt (1
mm) ST elevation above resting baseline in non-infarct leads during
both exercise and 1 or more minutes of recovery.
(ii) ETT findings may also show a decrease of 10 mmHg or more in
systolic pressure below the baseline systolic blood pressure or the
preceding systolic pressure measured during exercise due to left
ventricular dysfunction, despite an increase in workload. This
finding is the same finding required in 4.02B2b. See 4.00D4d for
full details.
c. In 4.04C, each ischemic episode must result in an unplanned
hospitalization. Examples of ischemic episodes that may result in
unplanned hospitalizations include unplanned revascularizations,
myocardial infarctions, unstable angina, or dysrhythmias.
Revascularization means angioplasty (with or without stent
placement) or bypass surgery.
(i) How do we calculate separate ischemic episodes? Reocclusion
that occurs after a revascularization procedure but during the same
hospitalization and that requires a second procedure during the same
hospitalization will not be counted as another ischemic episode. If
you are hospitalized for documented myocardial infarction and have a
revascularization procedure during the same hospitalization, this
event will be counted as one ischemic episode.
(ii) How do we evaluate ischemic episodes not amenable to
revascularizations? If your ischemic episodes are not amenable to
revascularization, we will evaluate them using the appropriate
listing (for example, 4.04D). Not amenable means that the
revascularization procedure could not be done because of another
medical impairment or because the vessel was not suitable for
revascularization.
d. We will use 4.04D only when you have symptoms due to
myocardial ischemia as described in 4.00E3-4.00E7 while on a regimen
of prescribed treatment, you are at risk for ETT (see 4.00C8), and
we do not have a timely ETT or a timely normal drug-induced stress
test for you. See 4.00C9 for what we mean by a timely test.
e. In 4.04D1, fractional flow reserve (FFR) is a measurement of
the pressure differences across an obstructive lesion, giving an
estimate of the severity of stenosis. An FFR measurement of 1.0
indicates normal blood flow. An FFR measurement equal to or less
than 0.80 indicates stenosis capable of producing serious myocardial
ischemia in an artery appropriate for revascularization. An FFR
measurement that is greater than 0.80 indicates stenosis not likely
to produce significant ischemia. FFR also helps define the adequacy
of collateral flow that directly affects function in ischemic heart
disease.
f. In 4.04D2, instantaneous wave-free ratio (iFR) measures the
pressure ratio during a specified period of diastole when the
coronary resistance is minimized and stable. An iFR value equal to
or less than 0.89 indicates a hemodynamically significant stenosis
that is appropriately treated with percutaneous coronary
intervention.
g. In 4.04D3 and 4.04D4, the term ``nonbypassed'' means that the
blockage is in a vessel that is potentially bypassable; that is,
large enough to be bypassed and considered to be a cause of your
ischemia. These vessels are usually major arteries or one of a major
artery's major branches. A vessel that has become obstructed again
after angioplasty or stent placement and has remained obstructed or
is not amenable to another revascularization is considered a
nonbypassed vessel for purposes of the listings. When you have had
revascularization, we will not use the pre-operative findings to
assess the current severity of your coronary artery disease under
4.04D, although we will consider the severity and duration of your
impairment before your surgery in making our determination or
decision.
F. How do we evaluate arrhythmias?
1. What is an arrhythmia? An arrhythmia is a change in the
regular beat of the heart. Your heart may seem to skip a beat or
beat irregularly, very quickly (tachycardia), or very slowly
(bradycardia). Although we use the term arrhythmia in the listings,
the term ``dysrhythmia'' may also be used in the medical evidence to
describe this condition.
2. What are the different types of arrhythmias?
a. There are many types of arrhythmias. Arrhythmias are
identified by where they occur in the heart (atria or ventricles)
and by what happens to the heart's rhythm when they occur.
b. Arrhythmias arising in the cardiac atria (upper chambers of
the heart) are called atrial or supraventricular arrhythmias.
Ventricular arrhythmias begin in the ventricles (lower chambers). In
general, ventricular arrhythmias caused by heart disease are the
most serious.
3. How do we evaluate arrhythmias using 4.05?
a. We will use 4.05 when you have arrhythmias that are not fully
controlled by medication, an implanted pacemaker, or an
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implanted cardiac defibrillator, and you have recurrent episodes of
syncope or near syncope. If your arrhythmias are controlled, we will
evaluate your underlying heart disease using the appropriate
listing. For other considerations when we evaluate arrhythmias in
the presence of an implanted cardiac defibrillator, see 4.00F4.
b. We consider near syncope to be a period of altered
consciousness, since syncope is a loss of consciousness or a faint.
It is not merely a feeling of light-headedness, momentary weakness,
or dizziness.
c. For purposes of 4.05, there must be a documented association
between the syncope or near syncope and the recurrent arrhythmia.
The recurrent arrhythmia, not some other cardiac or non-cardiac
disorder, must be established as the cause of the associated
symptom. This documentation of the association between the symptoms
and the arrhythmia may come from the usual diagnostic methods,
including Holter monitoring (also called ambulatory
electrocardiography) and tilt-table testing with a concurrent ECG.
Although an arrhythmia may be a coincidental finding on an ETT, we
will not purchase an ETT to document the presence of a cardiac
arrhythmia.
4. What do we consider when you have an implanted cardiac
defibrillator and you do not have arrhythmias that meet the
requirements of 4.05?
a. Implanted cardiac defibrillators are used to prevent sudden
cardiac death in people who have had, or are at high risk for,
cardiac arrest from life-threatening ventricular arrhythmias. The
largest group at risk for sudden cardiac death consists of people
with cardiomyopathy (ischemic or non-ischemic) and reduced
ventricular function. However, life-threatening ventricular
arrhythmias can also occur in people with little or no ventricular
dysfunction. The shock from the implanted cardiac defibrillator
rescues a person from what may have been cardiac arrest. However, as
a consequence of the shock(s), similar to the effects of treatments
for other cardiovascular disease, a person may experience
psychological distress, which we may evaluate under the listings in
12.00.
b. Most implantable cardiac defibrillators have rhythm-
correcting and pacemaker capabilities. In some people, these
functions may result in the termination of ventricular arrhythmias
without an otherwise painful shock. (The shock is like being kicked
in the chest.) Implanted cardiac defibrillators may deliver
inappropriate shocks, often repeatedly, in response to benign
arrhythmias or electrical malfunction. Also, exposure to strong
electrical or magnetic fields, such as from magnetic resonance
imaging, can trigger or reprogram an implanted cardiac
defibrillator, resulting in inappropriate shocks. We must consider
the frequency of, and the reason(s) for, the shocks when evaluating
the severity and duration of your impairment.
c. In general, the exercise limitations imposed on people with
an implanted cardiac defibrillator are those dictated by the
underlying heart impairment. However, the exercise limitations may
be greater when the implanted cardiac defibrillator delivers an
inappropriate shock in response to the increase in heart rate with
exercise, or when there is exercise-induced ventricular arrhythmia.
G. How do we evaluate peripheral vascular disease?
1. What is peripheral vascular disease (PVD)? Generally, PVD is
any impairment that affects either the arteries (peripheral artery
disease) or the veins (venous insufficiency) in the extremities,
particularly the lower extremities. The usual effect is blockage of
the flow of blood either from the heart (arterial) or back to the
heart (venous). If you have peripheral artery disease, you may have
pain in your leg after walking a distance that goes away when you
rest (intermittent claudication); at more advanced stages, you may
have pain in your leg or foot at rest or you may develop ulceration
or gangrene. Neuropathy may mask these typical symptoms. If you have
venous insufficiency, you may have swelling, varicose veins, skin
pigmentation changes, or skin ulceration.
2. How do we assess limitations resulting from PVD? We will
assess your limitations based on your symptoms together with
physical findings and Doppler studies or other appropriate
diagnostic techniques. However, if the PVD has resulted in
amputation, we will evaluate any limitations related to the
amputation under the listings in 1.00.
3. What is brawny edema? Brawny edema (4.11A) is swelling that
is usually dense and feels firm due to the presence of increased
connective tissue; it is also associated with characteristic skin
pigmentation changes. It is not the same thing as pitting edema.
Brawny edema generally does not pit (indent on pressure), and the
terms are not interchangeable. Pitting edema does not satisfy the
requirements of 4.11A.
4. What is lymphedema and how do we evaluate it?
a. Lymphedema is edema of the extremities due to a disorder of
the lymphatic circulation; at its worst, it is called elephantiasis.
Primary lymphedema is caused by abnormal development of lymph
vessels and may be present at birth (congenital lymphedema), but
more often develops during the teens (lymphedema praecox). It may
also appear later, usually after age 35 (lymphedema tarda).
Secondary lymphedema is due to obstruction or destruction of normal
lymphatic channels due to tumor, surgery, repeated infections, or
parasitic infection such as filariasis. Lymphedema most commonly
affects one extremity. Symptoms of lymphedema include but are not
limited to swelling in an extremity, changes in skin, and pain or
sensory changes in the affected area. The symptoms may limit
movement in affected joints.
b. Lymphedema does not meet the requirements of 4.11, although
it may medically equal the listing. We evaluate lymphedema by
considering whether the underlying cause meets or medically equals
any listing, or whether the lymphedema medically equals a
cardiovascular disorders listing such as 4.11 or a listing in 1.00
or 14.00. If no listing is met or medically equaled, we evaluate any
functional limitations imposed by your lymphedema when we assess
your residual functional capacity.
5. When will we purchase exercise Doppler studies for evaluating
peripheral artery disease (PAD)? If we need additional evidence of
your PAD, we will generally purchase exercise Doppler studies (see
4.00C16 and 4.00C17) when your resting ankle-brachial index is at
least 0.50 but less than 0.80, and only rarely when it is 0.80 or
above. We will not purchase exercise Doppler testing if you have a
disease that results in abnormal arterial calcification or small
vessel disease, but will use your resting toe systolic blood
pressure or resting toe-brachial index. (See 4.00G7c and 4.00G8.)
There are no current medical standards for evaluating exercise toe
pressures. Because any exercise test stresses your entire
cardiovascular system, we will purchase exercise Doppler studies
only after an MC, preferably one with experience in the care of
patients with cardiovascular disease, has determined that the test
would not present a significant risk to you and that there is no
other medical reason not to purchase the test (see 4.00C6, 4.00C7,
and 4.00C8).
6. Are there any other studies that are helpful in evaluating
PAD? Doppler studies done using a recording ultrasonic Doppler unit
and strain-gauge or air plethysmography are other useful tools for
evaluating PAD. A recording Doppler, which prints a tracing of the
arterial pulse wave in the femoral, popliteal, dorsalis pedis, and
posterior tibial arteries, is an evaluation tool that compares
waveforms in normal and compromised peripheral blood flow.
Qualitative analysis of the Doppler waveforms and plethysmographic
tracings is helpful in the overall assessment of the severity of the
occlusive disease. Tracings help in assessing severity if you have
small vessel disease related to diabetes mellitus or other diseases
with similar vascular changes, or diseases causing medial
calcifications when ankle pressure is either normal or falsely high.
When there is evidence of medial calcification of the ankle arteries
or the ankle-brachial index is 0.50 or greater, other appropriate
tests for PAD include magnetic resonance angiography, computed
tomography angiography, contrast angiography, and graded treadmill
tests.
7. How do we evaluate PAD under 4.12?
a. The ankle blood pressure referred to in 4.12A and B is the
higher of the pressures recorded from the posterior tibial and
dorsalis pedis arteries in the affected leg. The higher pressure
recorded from the two sites is the more significant measurement in
assessing the extent of arterial insufficiency. Techniques for
obtaining ankle systolic blood pressures include Doppler (See
4.00C16 and 4.00C17), plethysmographic studies, or other techniques.
We will request any available tracings generated by these studies so
that we can review them.
b. In 4.12A, the ankle-brachial index is the ratio of the
systolic blood pressure at the ankle to the systolic blood pressure
at the brachial artery; both taken at the same time
[[Page 40843]]
while you are lying on your back. We do not require that the ankle
and brachial pressures be taken on the same side of your body. This
is because, as with the ankle pressure, we will use the higher
brachial systolic pressure measured. The criterion in 4.12A is met
when your resting ankle-brachial index is less than 0.50. If your
resting ankle-brachial index is 0.50 or above, we will use 4.12B to
evaluate the severity of your PAD, unless you also have a disease
causing abnormal arterial calcification or small vessel disease,
such as diabetes mellitus. See 4.00G7c and 4.00G8.
c. We will use resting toe systolic blood pressures or resting
toe-brachial indices (determined the same way as the ankle-brachial
index, see 4.00G7b) when you have intermittent claudication and a
disease that results in abnormal arterial calcification (for
example, Monckeberg's sclerosis or diabetes mellitus) or small
vessel disease (for example, diabetes mellitus). These diseases may
result in misleadingly high blood pressure readings at the ankle.
However, high blood pressures due to vascular changes related to
these diseases seldom occur at the toe level. While the criteria in
4.12C and 4.12D are intended primarily for people who have a disease
causing abnormal arterial calcification or small vessel disease, we
may also use them for evaluating anyone with PAD.
8. How are toe pressures measured? Toe pressures are measured
routinely in most vascular laboratories through one of three
methods: most frequently, photoplethysmography; less frequently,
plethysmography using strain gauge cuffs; and Doppler ultrasound.
Toe pressure can also be measured by using any blood pressure cuff
that fits snugly around the big toe and is neither too tight nor too
loose. A neonatal cuff or a cuff designed for use on fingers or toes
can be used in the measurement of toe pressure.
9. How do we use listing 4.12 if you have had a peripheral graft
or stenting? Peripheral grafting serves the same purpose as coronary
grafting; that is, to bypass a narrow or obstructed arterial
segment. If intermittent claudication recurs or persists after
peripheral grafting, we may purchase Doppler studies to assess the
flow of blood through the bypassed vessel and to establish the
current severity of the peripheral artery impairment. However, if
you have had peripheral grafting or stenting done for your PAD, we
will not use the findings from before the surgery to assess the
current severity of your impairment, although we will consider the
severity and duration of your impairment prior to your surgery in
making our determination or decision.
H. How do we evaluate congenital heart disease?
1. What is congenital heart disease? Congenital heart disease is
any abnormality of the heart or the major blood vessels that is
present at birth. Congenital heart disease includes abnormal
structure of the individual heart chambers, valves, and blood
vessels, and abnormal relative relationship of the chambers to each
other that alters the normal pattern of blood flow. Surgery in
childhood is the usual treatment, and with improving surgical
techniques and medical management, more children with congenital
heart disease are surviving into adulthood. Rarely, a person with
congenital heart disease may not have received the usual surgery in
childhood, and later, as an adult, they are no longer a surgical
candidate, as for example, in Eisenmenger syndrome.
2. What is Eisenmenger syndrome? Eisenmenger syndrome refers to
any surgically untreated congenital heart defect with intracardiac
communication that over time leads to pulmonary hypertension,
reversal of blood flow, and hypoxemia.
a. Lesions in Eisenmenger syndrome, such as large septal
defects, are characterized by elevated pulmonary pressures or a high
pulmonary flow rate. In response, the pulmonary blood vessels
pathologically change, leading eventually to pulmonary hypertension.
Development of Eisenmenger syndrome represents a point at which
pulmonary hypertension is irreversible and the cardiac lesion is
likely inoperable.
b. Examples of congenital heart disease that if untreated may
cause pulmonary vascular disease leading to Eisenmenger syndrome
include atrial septal defect (ASD), ventricular septal defect (VSD),
and large patent ductus arteriosus (PDA).
3. What is single ventricle? The term ``single ventricle'' (also
known as ``single ventricle physiology'' or ``functional single
ventricle'') describes a diverse group of congenital cardiac
anomalies sharing the common feature that only one of the two heart
ventricles is adequately developed. At birth, one ventricle must
functionally do the work of two, pumping blood for both the body
(systemic) and the lungs (pulmonary). Because of this feature, the
ultimate plan for cardiac reconstruction is similar for most of
these anomalies. People with single ventricle will generally undergo
staged reconstructive ``Fontan procedures,'' ultimately resulting in
a ``Fontan circulation.'' Fontan circulation describes the
hemodynamic state in which virtually all systemic venous return-
blood passively flows directly into the pulmonary arteries via
surgical or catheter-placed shunts, without the blood passing
through a ventricle. The Fontan circulation results in difficulties
augmenting, and sometimes maintaining, cardiac output. Some of the
anomalies described as single ventricle include the following:
(a) Hypoplastic left heart syndrome;
(b) Hypoplastic right ventricle;
(c) Tricuspid valve atresia;
(d) Pulmonary atresia with intact ventricular septum;
(e) Double inlet left ventricle; and
(f) Some variations of double outlet right ventricle.
4. How do we evaluate conditions associated with congenital
heart disease?
a. We evaluate congenital heart disease that results in chronic
heart failure with evidence of ventricular dysfunction or in
recurrent arrhythmias under 4.02 or 4.05, respectively. Otherwise,
we evaluate your impairment under 4.06.
b. We evaluate pulmonary hypertension due to congenital heart
disease under 4.06B or 4.06C. We evaluate pulmonary hypertension not
due to congenital heart disease under the listings in 3.00 (for
example, 3.09).
c. We need pulse oximetry measurements documented by medical
sources using methods consistent with the prevailing state of
medical knowledge and clinical practice to evaluate chronic
hypoxemia in congenital heart disease under 4.06A3. These pulse
oximetry measurements also must be consistent with the other
evidence in the case record.
d. We evaluate single ventricle physiology under 4.06D and will
consider you disabled if your medical evidence documents that you
have any congenital heart disorder that results in single ventricle
physiology (functional single ventricle). In addition to the above
congenital heart disorders, examples of palliative surgical
procedures that indicate single ventricle physiology include the
Glenn, Fontan, and Norwood procedures.
I. How do we evaluate other cardiovascular disorders?
1. How do we evaluate hypertension? Hypertension (high blood
pressure) over time may significantly raise the pressures in the
heart to the point of ineffective heart muscle function known
generally as hypertensive heart disease that we can evaluate under
4.02. Other body systems, such as the brain, kidneys, or eyes may
also be affected. We evaluate these impairments by reference to the
specific body system(s) that is affected. We will also consider any
limitations imposed by your hypertension when we assess your
residual functional capacity.
2. What is cardiomyopathy and how do we evaluate it?
Cardiomyopathy is a disease of the heart muscle. The heart loses its
ability to pump blood (heart failure), and in some instances, heart
rhythm is disturbed, leading to irregular heartbeats (arrhythmias).
Usually, the exact cause of the muscle damage is never found
(idiopathic cardiomyopathy).
a. There are various types of cardiomyopathy, which fall into
two major categories: ischemic and nonischemic cardiomyopathy.
Ischemic cardiomyopathy typically refers to heart muscle damage that
results from coronary artery disease, including heart attacks.
Nonischemic cardiomyopathy includes, but is not limited to several
types: dilated, hypertrophic, restrictive, and arrhythmogenic.
Cardiomyopathy includes hypertrophic cardiomyopathy, endomyocardial
fibrosis, or cardiac amyloidosis AL (light-chain) type.
b. We evaluate cardiomyopathy under 4.08. Depending on the
underlying cause of the cardiomyopathy or its effects on you, we may
also evaluate your cardiomyopathy under 4.02, 4.04, or 4.05. If your
cardiomyopathy results in vascular insult to the brain, we may also
evaluate it under 11.04.
c. Under 4.08A1a, we need a conclusion from a medical source
that the performance of an exercise test would present a significant
risk to you. If your case record does not have a conclusion from a
medical source that an exercise test would present a significant
risk to you, an MC as defined in 4.00A3a may make such a conclusion
if evidence in your case record supports it.
3. How do we evaluate valvular heart disease? We evaluate aortic
valvular disease
[[Page 40844]]
under 4.07. We may also evaluate aortic valvular disease, as well as
other forms of valvular disease, under 4.02, 4.04, 4.05, 4.06, or a
listing in 11.00, depending on its effects on you.
4. What do we consider when we evaluate heart transplant
recipients?
a. After your heart transplant, we will consider you disabled
under 4.09 for 1 year following the surgery because there is a
greater likelihood of rejection of the organ and infection during
the first year. If you develop cardiac allograft vasculopathy after
your transplant, we will evaluate this impairment under 4.16.
b. However, heart transplant patients generally meet our
definition of disability before they undergo transplantation. We
will determine the onset of your disability based on the facts in
your case.
c. We will not assume that you became disabled when your name
was placed on a transplant waiting list. This is because you may be
placed on a waiting list soon after diagnosis of the cardiac
disorder that may eventually require a transplant. Physicians
recognize that candidates for transplantation often have to wait
months or even years before a suitable donor heart is found, so they
place their patients on the list as soon as permitted.
d. When we do a continuing disability review to determine
whether you are still disabled, we will evaluate your residual
impairment(s), as shown by the evidence in your case record,
including any side effects of medication. We will consider all
evidence indicative of cardiac dysfunction in deciding whether
medical improvement (as defined in Sec. Sec. 404.1594 and 416.994
of this chapter) has occurred.
5. What is cardiac allograft vasculopathy and how do we evaluate
it? Cardiac allograft vasculopathy (CAV) may affect a person who has
received a heart transplant and involves thickening in the walls of
the coronary arteries that may progress quickly into serious
vascular stenosis and heart dysfunction. Stenosis in CAV is caused
by a pathological process different from classic atherosclerosis and
treatment often is only palliative. We evaluate CAV under 4.16.
6. When does an aneurysm have ``dissection not controlled by
prescribed treatment,'' as required under 4.10? An aneurysm (or
bulge in the aorta or one of its major branches) is dissecting when
the inner lining of the artery begins to separate from the arterial
wall. We consider the dissection not controlled when you have
persistence of chest pain due to progression of the dissection, an
increase in the size of the aneurysm, or compression of one or more
branches of the aorta supplying the heart, kidneys, brain, or other
organs. An aneurysm with dissection can cause heart failure, renal
(kidney) failure, or neurological complications. If you have an
aneurysm that does not meet the requirements of 4.10 and you have
one or more of these associated conditions, we will evaluate the
condition(s) using the appropriate listing.
7. What is hyperlipidemia and how do we evaluate it?
Hyperlipidemia is the general term for an elevation of any or all of
the lipids (fats or cholesterol) in the blood; for example,
hypertriglyceridemia, hypercholesterolemia, and
hyperlipoproteinemia. These disorders of lipoprotein metabolism and
transport can cause defects throughout the body. The effects most
likely to interfere with function are those produced by
atherosclerosis (narrowing of the arteries) and coronary artery
disease. We will evaluate your lipoprotein disorder by considering
its effects on you.
8. What is Marfan syndrome and how do we evaluate it?
a. Marfan syndrome is a genetic connective tissue disorder that
affects multiple body systems, including the skeleton, eyes, heart,
blood vessels, nervous system, skin, and lungs. There is no specific
laboratory test to diagnose Marfan syndrome. The diagnosis is
generally made by medical history (including family history),
physical examination (including an evaluation of the ratio of arm/
leg size to trunk size), a slit lamp eye examination, and a heart
test(s) (such as an echocardiogram). In some cases, a genetic
analysis may be useful, but such analyses may not provide any
additional helpful information.
b. The effects of Marfan syndrome can range from mild to severe.
In most cases, the disorder progresses as you age. Most people with
Marfan syndrome have abnormalities associated with the heart and
blood vessels. Your heart's mitral valve may leak, causing a heart
murmur. Small leaks may not cause symptoms, but larger ones may
cause shortness of breath, fatigue, and palpitations. Another effect
is that the wall of the aorta may be weakened and abnormally stretch
(aortic dilation). This aortic dilation may tear, dissect, or
rupture, causing serious heart problems or sometimes sudden death.
We will evaluate the cardiovascular manifestations of your Marfan
syndrome under the appropriate criteria, such as 4.10, or, if
necessary, consider the functional limitations imposed by your
impairment.
c. Other genetic connective tissue disorders, such as Loeys-
Dietz syndrome or Ehlers-Danlos syndrome, may have abnormalities
associated with the heart and blood vessels. We will evaluate the
cardiovascular manifestations of your genetic connective tissue
disorder under the appropriate criteria, such as 4.10, or, if
necessary, consider the functional limitations imposed by your
impairment.
J. How do we evaluate other issues that affect the cardiovascular
system?
1. How do we consider the effects of obesity when we evaluate
your cardiovascular disorder? Obesity is a medically determinable
impairment that may be associated with cardiovascular disorders. The
additional body mass may make it harder for the chest and lungs to
expand or may cause the heart to work harder to pump blood to carry
oxygen to the body. The combined effects of obesity with a
cardiovascular disorder can be greater than the effects of each of
the impairments considered separately. We consider the additional
and cumulative effects of obesity when we determine whether you have
a severe cardiovascular disorder, a listing-level cardiovascular
disorder, a combination of impairments that medically equals the
severity of a listed impairment, and when we assess your residual
functional capacity.
2. How do we relate treatment to functional status? In general,
conclusions about the severity of a cardiovascular disorder cannot
be made on the basis of the type of treatment rendered or
anticipated. 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, we may need to defer evaluation of
the impairment for a period of up to 3 months from the date
treatment began to permit consideration of treatment effects, unless
we can make a determination or decision using the evidence we have.
See 4.00B4.
3. How do we consider hospitalizations? When we evaluate
hospitalizations for chronic heart failure (4.02B3), ischemic heart
disease (4.04E), congenital heart disease (4.06E), and
cardiomyopathy (4.08D), the hospitalizations do not all have to be
for the same cardiovascular disorder(s). They may be for three
different exacerbations or complications resulting from your
cardiovascular disorder. The hospitalizations must be at least 30
days apart, and each one must last at least 48 hours, including
hours in a hospital emergency department immediately before the
hospitalization.
K. How do we evaluate cardiovascular disorders that do not meet one
of these listings?
1. These listings are only examples of common cardiovascular
disorders that we consider severe enough to prevent you from doing
any gainful activity. If your impairment(s) does not meet the
criteria of any of these listings, we must also consider whether you
have an impairment(s) that satisfies the criteria of a listing in
another body system.
2. If you have a severe medically determinable impairment(s)
that does not meet a listing, we will determine whether your
impairment(s) medically equals a listing. See Sec. Sec. 404.1526
and 416.926 of this chapter. If your impairment(s) does not meet or
medically equal a listing, you may or may not have the residual
functional capacity to engage in substantial gainful activity. We
will proceed to the fourth step and, if necessary, the fifth step of
the sequential evaluation process in Sec. Sec. 404.1520 and 416.920
of this chapter. We will use the rules in Sec. Sec. 404.1594 or
416.994 of this chapter, as appropriate, when we decide whether you
continue to be disabled.
4.01 Category of Impairments, Cardiovascular Disorders
4.02 Chronic heart failure (see 4.00D) while on a regimen of
prescribed treatment, with symptoms and signs described in 4.00D2.
The required level of severity for this impairment is met when the
requirements are satisfied by A and B; or C alone; or D alone.
A. Medically documented presence of one of the following:
[[Page 40845]]
1. Heart failure with reduced ejection fraction documented by
appropriate medically acceptable imaging during a period of
stability (not during an episode of exacerbation of heart failure),
with either a. or b.
a. Left ventricular end diastolic dimension greater than 6.8 cm
for males or 6.1 cm for females during a period of stability (not
during an episode of exacerbation of heart failure); or
b. Ejection fraction of 30 percent or less during a period of
stability (not during an episode of exacerbation of heart failure);
OR
2. Heart failure with preserved ejection fraction documented by
appropriate medically acceptable imaging during a period of
stability (not during an episode of exacerbation of heart failure),
with either a. or b.
a. Left ventricular posterior wall plus septal thickness
totaling 2.5 cm or greater, with an enlarged left atrium greater
than or equal to 4.5 cm, or
b. Left atrial volume index (LAVI) greater than or equal to 40
ml, BSA/m\2\ (milliliters to body surface area in squared meters).
AND
B. Resulting in one of the following:
1. Recurrent (see 4.00A3c) symptoms of heart failure, resulting
in both a and b:
a. A medical source (see 4.00D4c(i)) has concluded that the
performance of an exercise test would present a significant risk to
the person; and
b. Very serious limitation in the ability to perform activities
of daily living independently, appropriately, effectively, and on a
sustained basis; or
2. Inability to perform on an exercise tolerance test at a
workload equivalent to 5 METs or less if using a standard treadmill
(or bicycle) test without gas exchange, or at 15 ml/kg/min peak
VO2 (oxygen consumption) on a cardiopulmonary exercise
test, due to either a or b.
a. Dyspnea, fatigue, palpitations, or chest discomfort; or
b. Decrease of 10 mmHg or more in systolic pressure below the
baseline systolic blood pressure or the preceding systolic pressure
measured during exercise (see 4.00D4d) due to left ventricular
dysfunction, despite an increase in workload; or
3. Exacerbations or complications of chronic heart failure (see
4.00D1b) requiring three hospitalizations within a consecutive 12-
month period (see 4.00A3e) and at least 30 days apart. Each
hospitalization must last at least 48 hours, including hours in a
hospital emergency department immediately before the hospitalization
(see 4.00J3);
OR
C. Heart failure with left ventricular ejection fraction of 20
percent or less while on a regimen of prescribed therapy, on two
evaluations at least 90 days apart within a consecutive 12-month
period (see 4.00A3e) during a period of stability (not during an
episode of exacerbation of heart failure);
OR
D. One of the following while hospitalized, at home, or both:
1. An implanted mechanical circulatory support device except
extracorporeal membrane oxygenation (ECMO) (see 4.00D4e). Consider
under a disability for 1 year from the date of implantation; after
that, evaluate any residual impairment(s) under the criteria for the
affected body system.
2. Continuous intravenous administration of inotropic medication
(for example, milrinone) for at least 30 consecutive days. Consider
under a disability for 1 year from the date of initiation of the
treatment; after that, evaluate any residual impairment(s) under the
criteria for the affected body system.
4.03 [Reserved]
4.04 Ischemic heart disease (see 4.00E), with symptoms due to
myocardial ischemia, while on a regimen of prescribed treatment (see
4.00B3 if there is no regimen of prescribed treatment), with A, B,
C, D, or E:
A. Inability to perform on an exercise tolerance test at a
workload equivalent to 5 METs or less with findings interpreted by
an acceptable medical source as positive for ischemia (see 4.00E9b).
OR
B. Ischemic response with exercise or pharmacological (drug-
induced) stress testing (see 4.00C14) on medically appropriate
imaging, with either 1 or 2:
1. At least two reversible or fixed regional myocardial
perfusion defects and either a or b:
a. Transient ischemic dilatation; or
b. Resting left ventricular ejection fraction of less than 50
percent; or
2. At least two reversible or fixed regional wall motion
abnormalities and either a or b:
a. Decrease in left ventricular ejection fraction during
testing; or
b. Resting left ventricular ejection fraction of less than 50
percent.
OR
C. Documentation of three separate ischemic episodes (see
4.00E9c) requiring unplanned hospitalization (inpatient or
observation status) within a consecutive 12-month period (see
4.00A3e).
OR
D. Coronary artery disease, documented by coronary angiography
(obtained independently of Social Security disability evaluation)
with 1, 2, 3 or 4:
1. Fractional flow reserve (FFR) (see 4.00E9e) measurement of
equal to or less than 0.80 of a proximal segment or mid segment
coronary artery not amenable to revascularization (see 4.00E9c(ii)).
2. Instantaneous wave-free ratio (iFR) (see 4.00E9f) measurement
of equal to or less than 0.89 of a proximal segment or mid segment
coronary artery not amenable to revascularization (see 4.00E9c(ii)).
3. History of coronary artery bypass graft surgery with
manifestations of ischemia, as described in 4.00E3-4.00E7, while on
a regimen of prescribed treatment (see 4.00B3 if there is no regimen
of prescribed treatment) with a, b, c, or d:
a. 50 percent or more stenosis of a nonbypassed left main
coronary artery; or
b. 70 percent or more stenosis in the proximal segment or mid
segment of another nonbypassed coronary artery; or
c. 50 percent or more stenosis in the proximal segment or mid
segment of at least two nonbypassed coronary arteries; or
d. 70 percent or more stenosis of a bypass graft vessel.
4. Resting left ventricular ejection fraction of less than 50
percent while medically stable (see 4.00B4) with manifestations of
ischemia, as described in 4.00E3-4.00E7, while on a regimen of
prescribed treatment (see 4.00B3 if there is no regimen of
prescribed treatment) with a, b, or c:
a. 50 percent or more stenosis of a nonbypassed left main
coronary artery; or
b. 70 percent stenosis in the proximal segment or mid segment of
another nonbypassed coronary artery; or
c. 50 percent or more stenosis in the proximal segment or mid
segment of at least two nonbypassed coronary arteries.
OR
E. Exacerbations or complications of ischemic heart disease (see
4.00E2-4.00E7) requiring three hospitalizations within a consecutive
12-month period (see 4.00A3e) and at least 30 days apart. Each
hospitalization must last at least 48 hours, including hours in a
hospital emergency department immediately before the hospitalization
(see 4.00J3).
4.05 Recurrent arrhythmias (see 4.00F), not related to
reversible causes such as electrolyte abnormalities or digitalis
glycoside or antiarrhythmic drug toxicity, while on a regimen of
prescribed treatment (see 4.00B3 if there is no prescribed
treatment), demonstrated by both A and B:
A. Coincident with recurrent (see 4.00A3c) episodes of cardiac
syncope or near syncope (see 4.00F3b).
AND
B. Documented by either 1 or 2:
1. Resting or ambulatory (Holter) electrocardiography; or
2. Other appropriate medically acceptable testing.
4.06 Congenital heart disease (see 4.00H), documented by
appropriate medically acceptable imaging (see 4.00A3d) or cardiac
catheterization, with A, B, C, D, or E:
A. Chronic hypoxemia, and 1, 2, or 3:
1. Hematocrit of 55 percent or greater on two evaluations at
least 90 days apart within a consecutive 12-month period (see
4.00A3e); or
2. Arterial blood gas test measurement obtained at rest while
breathing room air, as described in either a or b:
a. SaO2 (arterial oxygen saturation) less
than or equal to 89 percent; or
b. PO2 or PaO2 (partial
pressure of oxygen) less than or equal to 60 mmHg; or
3. SpO2 (percentage of oxygen saturation
of blood hemoglobin) measured by pulse oximetry either at rest,
during a 6-minute walk test (6MWT), or after a 6MWT, while breathing
room air, less than or equal to 87 percent on three evaluations at
least 30 days apart within a consecutive 12-month period (see
4.00A3e).
OR
B. Intermittent right-to-left shunting (for example, Eisenmenger
syndrome; see 4.00H2) during cardiopulmonary exercise testing while
breathing room air, resulting in oxygen desaturation on exertion at
a workload equivalent to 5 METs or less, or peak VO2
(oxygen uptake) of 15.0 ml/kg/min or less, and arterial blood gas
test measurement, with either 1 or 2:
[[Page 40846]]
1. SaO2 less than or equal to 89 percent;
or
2. PO2 or PaO2 less than or
equal to 60 mmHg.
OR
C. Pulmonary hypertension documented by cardiac catheterization
while medically stable, as described in 1, 2, or 3:
1. Pulmonary arterial systolic pressure elevated to at least 70
percent of the systemic arterial systolic pressure; or
2. Pulmonary arterial systolic pressure equal to or greater than
70 mmHg; or
3. Mean pulmonary artery pressure equal to or greater than 40
mmHg.
OR
D. Single ventricle (with or without Fontan procedures) (see
4.00H3).
OR
E. Exacerbations or complications of congenital heart disease
(see 4.00J3) requiring three hospitalizations within a consecutive
12-month period (see 4.00A3e) and at least 30 days apart. Each
hospitalization must last at least 48 hours, including hours in a
hospital emergency department immediately before the hospitalization
(see 4.00J3).
4.07 Aortic valvular disease (see 4.00I3), with symptoms due to
stenosis, determined by appropriate test or tests showing an aortic
valve area of less than 1.0 cm\2\.
4.08 Cardiomyopathy (see 4.00I2) while on a regimen of
prescribed treatment, with A, B, C, or D:
A. Hypertrophic cardiomyopathy documented by appropriate
medically acceptable imaging, with left ventricular or septal wall
thickness equal to or greater than 20 mm in the absence of other
causes of left ventricular hypertrophy (for example, hypertension or
aortic valvular disease) and either 1 or 2:
1. Recurrent (see 4.00A3c) symptoms of cardiomyopathy, resulting
in both a and b:
a. A medical source (see 4.00I2c) has concluded that the
performance of an exercise tolerance test would present a
significant risk to the person; and
b. Very serious limitation in the ability to perform activities
of daily living independently, appropriately, effectively, and on a
sustained basis; or
2. Inability to perform on an exercise tolerance test at a
workload equivalent to 5 METs or less if using a standard treadmill
(or bicycle) test without gas exchange, or at 15 ml/kg/min peak
VO2 (oxygen consumption) on a cardiopulmonary exercise
test.
OR
B. Endomyocardial fibrosis documented by appropriate medically
acceptable imaging, with 1, 2, and 3:
1. Loss of chamber volume due to fibrosis of the endocardium of
at least one ventricle; and
2. Right or left atrial dilatation (chamber enlargement); and
3. Regurgitant (backward) blood flow through the mitral or
tricuspid valve.
OR
C. Cardiac amyloidosis AL (light-chain) type documented by
biopsy.
OR
D. Exacerbations or complications of cardiomyopathy requiring
three hospitalizations within a consecutive 12-month period (see
4.00A3e) and at least 30 days apart. Each hospitalization must last
at least 48 hours, including hours in a hospital emergency
department immediately before the hospitalization (see 4.00J3).
4.09 Heart transplantation (see 4.00I4). Consider under a
disability for 1 year from the date of the transplant; after that,
evaluate the residual impairment(s).
4.10 Dissecting aneurysm of the aorta or major branches (see
4.00I6), due to any cause (for example, atherosclerosis, cystic
medial necrosis, Marfan syndrome, or trauma), with A and B:
A. Documented by appropriate medically acceptable imaging.
AND
B. Dissection not controlled by prescribed treatment.
4.11 Chronic venous insufficiency (see 4.00G) of a lower
extremity with reflux or obstruction of the venous system documented
by duplex ultrasound or other appropriate diagnostic technique, with
A or B:
A. Extensive trophic changes of skin (for example,
hyperpigmentation, lipodermatosclerosis, brawny edema) involving at
least two-thirds of the leg below the knee, on two evaluations at
least 90 days apart within a consecutive 12-month period (see
4.00A3e), with both 1 and 2:
1. Consistent with chronic venous insufficiency; and
2. Unresponsive to compression therapy.
OR
B. Two or more episodes of ulceration that have not healed
following at least 6 months of prescribed treatment.
4.12 Peripheral artery disease (see 4.00G7) while on a regimen
of prescribed treatment resulting in intermittent claudication or
leg pain that interferes with mobility (see 4.00G1), with A, B, C,
or D, as determined by an appropriate test(s) (see 4.00G5-4.00G6):
A. Resting ankle-brachial index of less than 0.50 (see 4.00G7b).
OR
B. Decrease in systolic blood pressure at the ankle on exercise
test (see 4.00G7a) of 50 percent or more of the pre-exercise level
and requiring 10 minutes or more to return to pre-exercise level.
OR
C. Resting toe systolic pressure of less than 30 mmHg (see
4.00G7c and 4.00G8).
OR
D. Resting toe-brachial index of less than 0.40 (see 4.00G7c).
4.13-4.15 [Reserved]
4.16 Cardiac allograft vasculopathy (see 4.00I5), documented by
appropriate medically acceptable imaging (for example, intravascular
ultrasonography or coronary angiography) (see 4.00A3d), with A, B,
C, or D:
A. Cardiac index (CI) or cardiac output (CO) less than 2 l/min/
m\2\.
OR
B. Left ventricular ejection fraction equal to or less than 45
percent.
OR
C. Right atrial pressure (RAP) greater than 12 mmHg.
OR
D. Pulmonary capillary wedge pressure (PCWP) greater than 15
mmHg.
* * * * *
Part B
* * * * *
Sec.
* * * * *
104.00 Cardiovascular Disorders
* * * * *
104.00 CARDIOVASCULAR DISORDERS
A. How do we define cardiovascular disorders and cardiovascular
terms?
1. What do we mean by a cardiovascular disorder?
a. We mean any disorder that affects the proper functioning of
the heart or the circulatory system (that is, arteries, veins,
capillaries, and the lymphatic drainage). The disorder can be
congenital or acquired.
b. Cardiovascular disorders result from one or more of four
consequences of heart disease:
(i) Chronic heart failure (chronic HF) or ventricular
dysfunction.
(ii) Discomfort or pain due to myocardial ischemia, with or
without necrosis of the heart muscle.
(iii) 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.
(iv) Hypoxemia (reduced oxygen concentration in the blood) due
to right-to-left shunt, or pulmonary vascular disease.
c. Disorders of the veins or arteries (for example, obstruction,
rupture, or aneurysm) may cause impairments of the lower extremities
(peripheral vascular disease), the central nervous system, the eyes,
the kidneys, and other organs. We will evaluate peripheral vascular
disease under 4.11 or 4.12 in part A, and impairments of another
body system(s) under the listings for that body system(s).
2. What do we consider in evaluating cardiovascular disorders?
The listings in this section describe cardiovascular disorders based
on the medical and other evidence, including response to a regimen
of prescribed treatment and functional limitations.
3. What do the following terms or phrases mean in these
listings?
a. Medical consultant is a person defined in Sec. 416.1016(a)
of this chapter. This term does not include medical sources who
provide consultative examinations for us. We use the abbreviation
``MC'' throughout this section to designate a medical consultant.
b. Persistent means that the longitudinal clinical record shows
that, with few exceptions, the required finding(s) has been present,
or is expected to be present, for a continuous period of at least 12
months, such that a pattern of continuing severity is established.
By ``exceptions,'' we mean brief periods when the required
finding(s) is greatly reduced or gone. These periods are so brief or
inconsequential, the required finding(s) remains a factor in the
person's condition.
c. Recurrent means that the longitudinal clinical record shows
that, within a
[[Page 40847]]
consecutive 12-month period, the finding(s) occurs at least three
times, with intervening periods of improvement of sufficient
duration that it is clear that separate events are involved. By
``improvement of sufficient duration,'' we mean the finding is
greatly reduced or not present for long enough that the required
finding(s) is no longer a factor in the person's condition.
d. Appropriate medically acceptable imaging means that the
technique used is the proper one to evaluate and diagnose the
impairment and is commonly recognized as accurate for assessing the
cited finding.
e. A consecutive 12-month period means a period of 12
consecutive months, all or part of which must occur within the
period we are considering in connection with an application or
continuing disability review.
B. What documentation do we need to evaluate cardiovascular
disorders?
1. What basic documentation do we need? We need sufficiently
detailed reports of history, physical examinations, laboratory
studies, and any prescribed treatment and response to allow us to
assess the severity and duration of your cardiovascular disorder. A
longitudinal clinical record covering a period of not less than 3
months of observations and treatment is usually necessary, unless we
can make a determination or decision based on the current evidence
we already have.
2. Why is a longitudinal clinical record important? We will
usually need a longitudinal clinical record to assess the severity
and expected duration of your impairment(s). If you have a listing-
level impairment, you probably will have received medically
prescribed treatment. Whenever there is evidence of such treatment,
your longitudinal clinical record should include a description of
the ongoing management and evaluation provided by your medical
source(s). It should also include your response to this medical
management, as well as information about the nature and severity of
your impairment. The record will provide us with information on your
functional status over an extended period of time and show whether
your ability to function is improving, worsening, or unchanging.
3. What if you have not received ongoing medical treatment?
a. You may not have received ongoing treatment or have an
ongoing relationship with the medical community despite the
existence of a severe impairment(s). In this situation, we will base
our evaluation on the current evidence we have. If you do not
receive treatment, you cannot show an impairment that meets the
criteria of these listings. However, we may find you disabled
because you have another impairment(s) that, in combination with
your cardiovascular disorder, medically equals a listing or
functionally equals the listings.
b. Unless we can decide your claim favorably on the basis of the
current evidence we already have, a longitudinal record is still
important. In instances when there is no or insufficient
longitudinal evidence, we may purchase a consultative examination(s)
to help us establish the existence, severity, and duration of your
impairment.
4. When will we wait before we ask for more evidence?
a. We will wait when we have information showing that your
impairment is not yet stable and the expected change in your
impairment might affect our determination or decision. In these
situations, we need to wait to properly evaluate the severity and
duration of your impairment during a stable period. Examples of when
we might wait are:
(i) If you have had a recent acute event; for example, acute
heart failure.
(ii) If you have recently had a corrective cardiac procedure;
for example, open-heart surgery.
(iii) If you have started new drug therapy and your response to
this treatment has not yet been established; for example, beta-
blocker therapy for dilated cardiomyopathy.
b. In these situations, we will obtain more evidence 3 months
following the event before we evaluate your impairment. However, we
will not wait if we have enough information to make a determination
or decision based on all of the relevant evidence in your case.
5. Will we purchase any studies? In appropriate situations, we
may purchase studies necessary to substantiate the existence of a
medically determinable impairment or to document the severity of
your impairment, generally after we have evaluated the evidence we
already have. We will not purchase studies involving exercise
testing if there is significant risk involved or if there is another
medical reason not to perform the test. We will follow sections
4.00C6, 4.00C7, 4.00C8 in part A, and 104.00B7, when we decide
whether to purchase exercise testing. We will make a reasonable
effort to obtain any additional studies from a qualified medical
source in an office or center experienced in pediatric cardiac
assessment. (See Sec. 416.919g of this chapter.)
6. What studies will we not purchase? We will not purchase any
studies involving cardiac catheterization, such as coronary
angiography, arteriograms, or electrophysiological studies. However,
if the results of a catheterization are part of the existing
evidence we have, we will consider them together with the other
relevant evidence. See 4.00C15a in part A.
7. Will we use exercise tolerance tests (ETT) for evaluating
children with cardiovascular disorders?
a. ETTs, though increasingly used, are still less frequently
indicated in children than in adults, and can rarely be performed
successfully by children under 6 years of age. An ETT may be of
value in the assessment of some arrhythmias, and may be considered
in 104.05B2. ETTs may also be used in the assessment of the severity
of chronic heart failure and in the assessment of recovery of
function following cardiac surgery or other treatment.
b. We will purchase an ETT only if we cannot make a
determination or decision based on the evidence we have and an MC,
preferably one with experience in the care of children with
cardiovascular disorders, has determined that an ETT is needed to
evaluate your impairment. We will not purchase an ETT if you are
less than 6 years of age. If we do purchase an ETT for a child age
12 or younger, it must be performed by a qualified medical source in
a specialty center for pediatric cardiology or other facility
qualified to perform exercise tests of children.
c. For full details on ETT requirements and usage, see 4.00C3 in
part A.
C. How do we evaluate chronic heart failure?
1. What is chronic heart failure (chronic HF)?
a. Heart failure is the inability of the heart to pump enough
oxygenated blood to body tissues. This syndrome is characterized by
symptoms and signs of pulmonary or systemic congestion (fluid
retention) or limited cardiac output. Certain laboratory findings of
cardiac functional and structural abnormality support the diagnosis
of chronic HF. Heart failure can range from low ejection fraction
due to muscle dysfunction to preserved ejection fraction with
impaired relaxation of the left ventricle. We consider heart failure
to be chronic when the condition persists or recurs over time
despite treatment.
b. Chronic HF is considered in these listings as a single
category whether due to atherosclerosis (narrowing of the arteries),
cardiomyopathy, hypertension, congenital, or other heart disease. If
the chronic HF is the result of primary pulmonary hypertension
secondary to disease of the lung, we will evaluate your impairment
under the listings in 3.00 (for example, 3.09) or 4.00 in part A, as
appropriate.
2. What evidence of chronic HF do we need?
a. Cardiomegaly or ventricular dysfunction must be present and
demonstrated by appropriate medically acceptable imaging, such as
cardiac magnetic resonance imaging (MRI), chest x-ray,
echocardiography (M-Mode, 2-dimensional, and Doppler), radionuclide
studies, or cardiac catheterization. Other findings on appropriate
medically acceptable imaging may include increased ventricular
volume, increased pulmonary vascular markings, pleural effusion, and
pulmonary edema.
b. Your medical history and physical examination should describe
characteristic symptoms and signs of pulmonary or systemic
congestion (fluid retention) or of limited cardiac output associated
with the abnormal findings on appropriate medically acceptable
imaging. When an acute episode of heart failure is triggered by a
remediable factor, such as an arrhythmia, dietary sodium overload,
or high altitude, cardiac function may be restored and a chronic
impairment may not be present.
(i) Symptoms of congestion or of limited cardiac output include
easy fatigue, weakness, shortness of breath (dyspnea), cough,
feeding intolerance, gastrointestinal distress, or chest discomfort
at rest or with activity. Children with chronic HF may also
experience shortness of breath on lying flat (orthopnea) or episodes
of shortness of breath that wake them from sleep (paroxysmal
nocturnal dyspnea). They may also experience cardiac arrhythmias
resulting in palpitations, lightheadedness, or fainting. Fatigue or
exercise intolerance in an infant may result in prolonged feeding
time or tube
[[Page 40848]]
feeding, often associated with excessive respiratory effort and
sweating.
(ii) Other manifestations of chronic HF may include repeated
lower respiratory tract infections, wheezing, or growth failure
(failure to thrive).
(iii) Signs of congestion may include hepatomegaly, ascites,
increased jugular venous distention or pressure, rales, peripheral
edema, rapid shallow breathing (tachypnea), or rapid weight gain.
However, these signs need not be found on all examinations because
congestion may be controlled by prescribed treatment or may not be
present at the time of evaluation.
3. How do we evaluate growth failure due to chronic HF?
a. To evaluate growth failure due to chronic HF, we require
documentation of the clinical findings of chronic HF described in
104.00C2 and the growth measurements in 104.02C within the same
consecutive 12-month period. The dates of clinical findings may be
different from the dates of growth measurements.
b. Under 104.02C, we use the appropriate table(s) under 105.08B
in the digestive system to determine whether your growth is less
than the third percentile.
(i) If you have not attained age 2, we use the weight-for-length
table corresponding to your sex (Table I or Table II).
(ii) If you have attained age 2 but have not attained age 18, we
use the body mass index (BMI)-for-age table corresponding to your
sex (Table III or Table IV).
(iii) BMI is the ratio of your weight to the square of your
height. We calculate BMI using the formulas in the digestive
disorders body system (105.00).
4. How do we evaluate chronic HF treated with a mechanical
circulatory support device? We use 104.02D to evaluate chronic HF
treated with an implanted mechanical circulatory support device
(MCSD), such as a left ventricle assistive device (LVAD) or a right
ventricle assistive device (RVAD). Implanted MCSDs are intended for
long-term circulatory support in helping the heart pump blood. For
the purposes of 104.02D, an MCSD does not include extracorporeal
membrane oxygenation (ECMO) or devices using Impella technology.
Although these are forms of mechanical circulatory support, we do
not include them in 104.02D because they are intended only for
short-term circulatory support (maximum 30 days), used in a setting
of imminent or actual cardiac arrest.
D. How do we evaluate congenital heart disease?
1. What is congenital heart disease? Congenital heart disease is
any abnormality of the heart or the major blood vessels that is
present at birth. Congenital heart disease includes abnormal
structure of the individual heart chambers, valves, and blood
vessels, and abnormal relative relationship of the chambers to each
other that alters the normal pattern of blood flow. Surgery or an
interventional catheterization procedure is the usual treatment, and
with improving surgical techniques and medical management, more
children with congenital heart disease are surviving into adulthood.
Examples of congenital heart disease include:
a. Abnormalities of cardiac septation, including atrial or
ventricular septal defect or atrioventricular canal;
b. Abnormalities resulting in cyanotic heart disease, including
tetralogy of Fallot, transposition of the great arteries, truncus
arteriosus, total anomalous pulmonary venous return, or Epstein
malformation;
c. Valvular defects with obstructions or regurgitation to
ventricular inflow or outflow, including pulmonary or aortic
stenosis, pulmonary atresia, or coarctation of the aorta; and
d. Major abnormalities of ventricular development, including
hypoplastic left heart syndrome or tricuspid atresia with
hypoplastic right ventricle.
2. How do we evaluate conditions associated with congenital
heart disease?
a. We will evaluate congenital heart disease that results in
chronic HF with evidence of ventricular dysfunction or in recurrent
arrhythmias under 104.02 or 104.05, respectively. Otherwise, we will
evaluate your impairment under 104.06.
b. We need pulse oximetry measurements documented by medical
sources using methods consistent with the prevailing state of
medical knowledge and clinical practice to evaluate chronic
hypoxemia in congenital heart disease under 104.06A3. These pulse
oximetry measurements also must be consistent with the other
evidence in the case record.
c. For 104.06D, life-threatening congenital heart disease does
not include single ventricle; we evaluate single ventricle
physiology separately under 104.06C. When we evaluate life-
threatening congenital heart disease under 104.06D, we consider
whether it responds to surgical treatment and, therefore, may not
meet the 12-month duration requirement. Examples of impairments that
in most instances will require life-saving surgery or a combination
of surgery and other major interventional procedures (for example,
multiple ``balloon'' catheter procedures) before age 1 include, but
are not limited to, the following:
(i) Critical aortic stenosis with neonatal heart failure;
(ii) Critical coarctation of the aorta, with associated
anomalies;
(iii) Complete atrioventricular canal defects;
(iv) Transposition of the great arteries;
(v) Tetralogy of Fallot; and
(vi) Multiple ventricular septal defects.
3. What is Eisenmenger syndrome? Eisenmenger syndrome refers to
any surgically untreated congenital heart defect with intracardiac
communication that over time leads to pulmonary hypertension,
reversal of blood flow, and hypoxemia.
a. Lesions in Eisenmenger syndrome, such as large septal
defects, are characterized by elevated pulmonary pressures or a high
pulmonary flow rate. In response, the pulmonary blood vessels
pathologically change, leading eventually to pulmonary hypertension.
Development of Eisenmenger syndrome represents a point at which
pulmonary hypertension is irreversible and the cardiac lesion is
likely inoperable.
b. Examples of congenital heart disease that if untreated may
cause pulmonary vascular disease leading to Eisenmenger syndrome
include atrial septal defect (ASD), ventricular septal defect (VSD),
and large patent ductus arteriosus (PDA). We evaluate Eisenmenger
syndrome under 104.06A or 104.06B.
4. What is single ventricle? The term ``single ventricle'' (also
known as ``single ventricle physiology'' or ``functional single
ventricle'') describes a diverse group of congenital cardiac
anomalies sharing the common feature that only one of the two heart
ventricles is adequately developed. At birth, one ventricle must
functionally do the work of two, pumping blood for both the body
(systemic) and the lungs (pulmonary). Because of this feature, the
ultimate plan for cardiac reconstruction is similar for most of
these anomalies. People with single ventricle will generally undergo
staged reconstructive ``Fontan procedures,'' ultimately resulting in
a ``Fontan circulation.'' Fontan circulation describes the
hemodynamic state in which virtually all systemic venous return-
blood passively flows directly into the pulmonary arteries via
surgical or catheter-placed shunts, without (the blood) passing
through a ventricle. The Fontan circulation results in difficulties
augmenting, and sometimes maintaining, cardiac output. Some of the
anomalies described as single ventricle include the following:
(a) Hypoplastic left heart syndrome;
(b) Hypoplastic right ventricle;
(c) Tricuspid valve atresia;
(d) Pulmonary atresia with intact ventricular septum;
(e) Double inlet left ventricle; and
(f) Some variations of double outlet right ventricle.
E. How do we evaluate arrhythmias?
1. What is an arrhythmia? An arrhythmia is a change in the
regular beat of the heart. Your heart may seem to skip a beat or
beat irregularly, very quickly (tachycardia), or very slowly
(bradycardia). Although we use the term ``arrhythmia'' in the
listings, the term ``dysrhythmia'' may also be used in the medical
evidence to describe this condition.
2. What are the different types of arrhythmias?
a. There are many types of arrhythmias. Arrhythmias are
identified by where they occur in the heart (atria or ventricles)
and by what happens to the heart's rhythm when they occur.
b. Arrhythmias arising in the cardiac atria (upper chambers of
the heart) are called atrial or supraventricular arrhythmias.
Ventricular arrhythmias begin in the ventricles (lower chambers). In
general, ventricular arrhythmias caused by heart disease are the
most serious.
3. How do we evaluate arrhythmias using 104.05?
a. We will use 104.05 when you have arrhythmias that are not
fully controlled by medication, an implanted pacemaker, or an
implanted cardiac defibrillator, and you have recurrent episodes of
syncope or near syncope. If your arrhythmias are controlled, we will
evaluate your underlying heart disease using the appropriate
listing. For other considerations when we evaluate arrhythmias in
the presence of an implanted cardiac defibrillator, see 104.00E4.
[[Page 40849]]
b. We consider near syncope to be a period of altered
consciousness, since syncope is a loss of consciousness or a faint.
It is not merely a feeling of light-headedness, momentary weakness,
or dizziness.
c. For purposes of 104.05, there must be a documented
association between the syncope or near syncope and the recurrent
arrhythmia. The recurrent arrhythmia, not some other cardiac or non-
cardiac disorder, must be established as the cause of the associated
symptom. This documentation of the association between the symptoms
and the arrhythmia may come from the usual diagnostic methods,
including Holter monitoring (also called ambulatory
electrocardiography) and tilt-table testing with a concurrent ECG.
Although an arrhythmia may be a coincidental finding on an ETT, we
will not purchase an ETT to document the presence of a cardiac
arrhythmia.
4. What do we consider when you have an implanted cardiac
defibrillator and you do not have arrhythmias that meet the
requirements of 104.05?
a. Implanted cardiac defibrillators are used to prevent sudden
cardiac death in children who have had, or are at high risk for,
cardiac arrest from life-threatening ventricular arrhythmias. The
largest group of children at risk for sudden cardiac death consists
of children with cardiomyopathy (ischemic or non-ischemic) and
reduced ventricular function. However, life-threatening ventricular
arrhythmias can also occur in children with little or no ventricular
dysfunction. The shock from the implanted cardiac defibrillator
rescues a child from what may have been cardiac arrest. However, as
a consequence of the shock(s), similar to the effects of treatments
for other cardiovascular disease, a child may experience
psychological distress, which we may evaluate under the listings in
112.00.
b. Most implantable cardiac defibrillators have rhythm-
correcting and pacemaker capabilities. In some children, these
functions may result in the termination of ventricular arrhythmias
without an otherwise painful shock. (The shock is like being kicked
in the chest.) Implanted cardiac defibrillators may deliver
inappropriate shocks, often repeatedly, in response to benign
arrhythmias or electrical malfunction. Also, exposure to strong
electrical or magnetic fields, such as from magnetic resonance
imaging, can trigger or reprogram an implanted cardiac
defibrillator, resulting in inappropriate shocks. We must consider
the frequency of, and the reason(s) for, the shocks when evaluating
the severity and duration of your impairment.
c. In general, the exercise limitations imposed on children with
an implanted cardiac defibrillator are those dictated by the
underlying heart impairment. However, the exercise limitations may
be greater when the implanted cardiac defibrillator delivers an
inappropriate shock in response to the increase in heart rate with
exercise, or when there is exercise-induced ventricular arrhythmia.
F. How do we evaluate other cardiovascular disorders?
1. What is ischemic heart disease (IHD) and how do we evaluate
it in children? IHD results when one or more of your coronary
arteries is narrowed or obstructed or, in rare situations,
constricted due to vasospasm, interfering with the normal flow of
blood to your heart muscle (ischemia). The obstruction may be the
result of an embolus, a thrombus, or plaque. When heart muscle
tissue dies as a result of the reduced blood supply, it is called a
myocardial infarction (heart attack). Ischemia is rare in children,
but when it occurs, its effects on children are the same as on
adults. If you have IHD, we evaluate it under 4.04 in part A.
2. How do we evaluate hypertension? Hypertension (high blood
pressure) generally causes disability in children through its
effects on other body systems, such as the brain, kidneys, or eyes,
and we will evaluate these impairments by reference to the specific
body system(s) that is affected. We also consider any limitations
imposed by your hypertension when we consider whether you have an
impairment that functionally equals the listings.
3. What is cardiomyopathy and how do we evaluate it?
a. Cardiomyopathy is a disease of the heart muscle. The heart
loses its ability to pump blood (heart failure), and in some
instances, heart rhythm is disturbed, leading to irregular
heartbeats (arrhythmias). Usually, the exact cause of the muscle
damage is never found (idiopathic cardiomyopathy).
b. There are various types of cardiomyopathy, which fall into
two major categories: ischemic and nonischemic cardiomyopathy.
Ischemic cardiomyopathy typically refers to heart muscle damage that
results from coronary artery disease, including heart attacks.
Nonischemic cardiomyopathy includes, but is not limited to several
types: dilated, hypertrophic, restrictive, and arrhythmogenic.
c. We evaluate cardiomyopathy under 4.04 in part A, 104.02, or
104.05, depending on its effects on you.
4. How do we evaluate valvular heart disease? We evaluate aortic
valvular disease under 4.07 in part A. We may also evaluate aortic
valvular disease, as well as other forms of valvular disease, under
4.04 in part A, 104.02, 104.05, 104.06, or a listing in 111.00,
depending on its effects on you.
5. What do we consider when we evaluate heart transplant
recipients?
a. After your heart transplant, we consider you disabled under
104.09 for 1 year following the surgery because there is a greater
likelihood of rejection of the organ and infection during the first
year. If you develop cardiac allograft vasculopathy after your
transplant, we will evaluate this impairment under 104.16.
b. However, heart transplant patients generally meet our
definition of disability before they undergo transplantation. We
will determine the onset of your disability based on the facts in
your case.
c. We will not assume that you became disabled when your name
was placed on a transplant waiting list. This is because you may be
placed on a waiting list soon after diagnosis of the cardiac
disorder that may eventually require a transplant. Physicians
recognize that candidates for transplantation often have to wait
months or even years before a suitable donor heart is found, so they
place their patients on the list as soon as permitted.
d. When we do a continuing disability review to determine
whether you are still disabled, we will evaluate your residual
impairment(s), as shown by the evidence in your case record,
including any side effects of medication. We will consider all
evidence indicative of cardiac dysfunction in deciding whether
medical improvement (as defined in Sec. 416.994a of this chapter)
has occurred.
6. How do we evaluate chronic rheumatic fever or rheumatic heart
disease? We evaluate rheumatic fever or rheumatic heart disease
under the listing appropriate to its effects on you, which may
include heart failure or recurrent arrhythmias. If you have evidence
of chronic heart failure or recurrent arrhythmias associated with
rheumatic heart disease, we evaluate these disorders under 104.02 or
104.05, respectively.
7. What is hyperlipidemia and how do we evaluate it?
Hyperlipidemia is the general term for an elevation of any or all of
the lipids (fats or cholesterol) in the blood; for example,
hypertriglyceridemia, hypercholesterolemia, and
hyperlipoproteinemia. These disorders of lipoprotein metabolism and
transport can cause defects throughout the body. The effects most
likely to interfere with function are those produced by
atherosclerosis (narrowing of the arteries) and coronary artery
disease. We evaluate your lipoprotein disorder by considering its
effects on you.
8. How do we evaluate Kawasaki disease? We evaluate Kawasaki
disease under the listing appropriate to its effects on you, which
may include major coronary artery aneurysm or heart failure. A major
coronary artery aneurysm may cause ischemia or arrhythmia, which we
evaluate under 4.04 in part A or 104.05. We will evaluate chronic
heart failure under 104.02.
9. What is lymphedema and how do we evaluate it?
a. Lymphedema is edema of the extremities due to a disorder of
the lymphatic circulation; at its worst, it is called elephantiasis.
Primary lymphedema is caused by abnormal development of lymph
vessels and may be present at birth (congenital lymphedema), but
more often develops during the teens (lymphedema praecox). Secondary
lymphedema is due to obstruction or destruction of normal lymphatic
channels due to tumor, surgery, repeated infections, or parasitic
infection such as filariasis. Lymphedema most commonly affects one
extremity. Symptoms of lymphedema include but are not limited to
swelling in an extremity, changes in skin, and pain or sensory
changes in the affected area. The symptoms may limit movement in
affected joints.
b. Lymphedema does not meet the requirements of 4.11 in part A,
although it may medically equal the listing. We evaluate lymphedema
by considering whether the underlying cause meets or medically
equals any listing or whether the lymphedema
[[Page 40850]]
medically equals a cardiovascular disorders listing, such as 4.11 in
part A, or a listing in 101.00 or 114.00. If no listing is met or
medically equaled, we evaluate any functional limitations imposed by
your lymphedema when we consider whether you have an impairment(s)
that functionally equals the listings.
10. What is Marfan syndrome and how do we evaluate it?
a. Marfan syndrome is a genetic connective tissue disorder that
affects multiple body systems, including the skeleton, eyes, heart,
blood vessels, nervous system, skin, and lungs. There is no specific
laboratory test to diagnose Marfan syndrome. The diagnosis is
generally made by medical history (including family history),
physical examination (including an evaluation of the ratio of arm/
leg size to trunk size), a slit lamp eye examination, and a heart
test(s) (such as an echocardiogram). In some cases, a genetic
analysis may be useful, but such analyses may not provide any
additional helpful information.
b. The effects of Marfan syndrome can range from mild to severe.
In most cases, the disorder progresses as you age. Most people with
Marfan syndrome have abnormalities associated with the heart and
blood vessels. Your heart's mitral valve may leak, causing a heart
murmur. Small leaks may not cause symptoms, but larger ones may
cause shortness of breath, fatigue, and palpitations. Another effect
is that the wall of the aorta may be weakened and stretch (aortic
dilation). This aortic dilation may tear, dissect, or rupture,
causing serious heart problems or sometimes sudden death. We
evaluate the cardiovascular manifestations of your Marfan syndrome
under the appropriate criteria, such as 4.10 in part A, or, if
necessary, consider the functional limitations imposed by your
impairment.
c. Other genetic connective tissue disorders, such as Loeys-
Dietz syndrome or Ehlers-Danlos syndrome, may have abnormalities
associated with the heart and blood vessels. We evaluate the
cardiovascular manifestations of your genetic connective tissue
disorder under the appropriate criteria, such as 4.10 in part A, or,
if necessary, consider the functional limitations imposed by your
impairment.
11. What is cardiac allograft vasculopathy and how do we
evaluate it? Cardiac allograft vasculopathy (CAV) may affect a
person who has received a heart transplant and involves thickening
in the walls of the coronary arteries that may progress quickly into
serious vascular stenosis and heart dysfunction. Stenosis in CAV is
caused by a pathological process different from classic
atherosclerosis and treatment often is only palliative. We evaluate
CAV under 104.16.
G. How do we evaluate other issues that affect the cardiovascular
system?
1. How do we consider the effects of obesity when we evaluate
your cardiovascular disorder? Obesity is a medically determinable
impairment that may be associated with cardiovascular disorders. The
additional body mass may make it harder for the chest and lungs to
expand or may cause the heart to work harder to pump blood to carry
oxygen to the body. The combined effects of obesity with a
cardiovascular disorder can be greater than the effects of each of
the impairments considered separately. We consider the additional
and cumulative effects of obesity when we determine whether you have
a severe cardiovascular disorder, a listing-level cardiovascular
disorder, a combination of impairments that medically equals the
severity of a listed impairment, and when we determine whether your
impairment(s) functionally equals the listings.
2. How do we relate treatment to functional status? In general,
conclusions about the severity of a cardiovascular disorder cannot
be made on the basis of the type of treatment rendered or
anticipated. 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, we may need to defer evaluation of
the impairment for a period of up to 3 months from the date
treatment began to permit consideration of treatment effects, unless
we can make a determination or decision using the evidence we have.
See 104.00B4.
3. How do we consider hospitalizations? The hospitalizations in
104.02E and 104.06E do not all have to be for the same exacerbation
or complication of your cardiovascular disorder(s). They may be for
three different exacerbations or complications resulting from your
cardiovascular disorder. The hospitalizations must be at least 30
days apart, and each one must last at least 48 hours, including
hours in a hospital emergency department immediately before the
hospitalization.
H. How do we evaluate cardiovascular disorders that do not meet one
of these listings?
1. These listings are only examples of common cardiovascular
disorders that we consider severe enough to result in marked and
severe functional limitations. If your impairment(s) does not meet
the criteria of any of these listings, we must also consider whether
you have an impairment(s) that satisfies the criteria of a listing
in another body system.
2. If you have a severe medically determinable impairment(s)
that does not meet a listing, we will determine whether your
impairment(s) medically equals a listing. See Sec. 416.926 of this
chapter. If your impairment(s) does not meet or medically equal a
listing, we will also consider whether it functionally equals the
listings. See Sec. 416.926a of this chapter. We will use the rules
in Sec. 416.994a of this chapter when we decide whether you
continue to be disabled.
104.01 Category of Impairments, Cardiovascular Disorders
104.02 Chronic heart failure (see 104.00C) while on a regimen of
prescribed treatment with symptoms and signs described in 104.00C2,
and with A, B, C, D, or E:
A. Persistent tachycardia at rest measured at least twice within
a consecutive 12-month period and at least 90 days apart documented
by apical heart rate greater than or equal to the value in Table I.
Table I--Tachycardia--At Rest
------------------------------------------------------------------------
Apical heart
Age rate (beats
per minute)
------------------------------------------------------------------------
Under 1 year............................................ 150
1 through 3 years....................................... 130
4 through 9 years....................................... 120
10 through 15 years..................................... 110
Over 15 years........................................... 100
------------------------------------------------------------------------
OR
B. Persistent tachypnea at rest measured at least twice within a
consecutive 12-month period and at least 90 days apart documented by
respiratory rate greater than or equal to the value in Table II or
markedly decreased exercise tolerance (see 104.00C2b).
Table II--Tachypnea--At Rest
------------------------------------------------------------------------
Respiratory
Age rate (per
minute)
------------------------------------------------------------------------
Under 1 year............................................ 40
1 through 5 years....................................... 35
6 through 9 years....................................... 30
Over 9 years............................................ 25
------------------------------------------------------------------------
OR
C. Growth failure as required in 1 or 2:
1. For children from birth to attainment of age 2, three weight-
for-length measurements that are:
a. Within a consecutive 12-month period; and
b. At least 60 days apart; and
c. Less than the third percentile on the appropriate weight-for-
length table under 105.08B1; or
2. For children age 2 to attainment of age 18, three BMI-for-age
measurements that are:
a. Within a consecutive 12-month period; and
b. At least 60 days apart; and
c. Less than the third percentile on the appropriate BMI-for-age
table under 105.08B2.
OR
D. An implanted mechanical circulatory support device (except an
extracorporeal membrane oxygenation (ECMO) while hospitalized, at
home, or both (see 104.00C4). Consider under a disability for 1 year
from the date of implantation; after that, evaluate any residual
impairment(s) under the criteria for the affected body system.
OR
E. Exacerbations or complications of chronic heart failure (see
104.00C1b) requiring three hospitalizations within a consecutive 12-
month period and at least 30 days apart. Each hospitalization must
last at least 48 hours, including hours in a hospital emergency
department immediately before the hospitalization (see 104.00G3).
[[Page 40851]]
104.03-104.04 [Reserved]
104.05 Recurrent arrhythmias (see 104.00E), not related to
reversible causes such as electrolyte abnormalities or digitalis
glycoside or antiarrhythmic drug toxicity, while on a regimen of
prescribed treatment (see 104.00B3 if there is no prescribed
treatment), demonstrated by both A and B:
A. Coincident with recurrent (see 104.00A3c) episodes of cardiac
syncope or near syncope (see 104.00E3b).
AND
B. Documented by either 1 or 2:
1. Resting or ambulatory (Holter) electrocardiography; or
2. Other appropriate medically acceptable testing.
104.06 Congenital heart disease (see 104.00D), documented by
appropriate medically acceptable imaging (see 104.00A3d) or cardiac
catheterization, with A, B, C, D, or E:
A. Chronic hypoxemia, and 1, 2, or 3:
1. Hematocrit of 55 percent or greater on two evaluations at
least 90 days apart within a consecutive 12-month period (see
104.00A3e); or
2. Arterial blood gas test measurement obtained at rest while
breathing room air, as described in either a or b:
a. SaO2 (arterial oxygen saturation) less
than or equal to 89 percent; or
b. PO2 or PaO2 (partial
pressure of oxygen) less than or equal to 60 mmHg; or
3. SpO2 (percentage of oxygen saturation
of blood hemoglobin) measured by pulse oximetry either at rest, or
after activity, while breathing room air, less than or equal to 87
percent on three evaluations at least 30 days apart within a
consecutive 12-month period (see 104.00A3e).
OR
B. Pulmonary hypertension documented by cardiac catheterization
while medically stable, as described in 1, 2, or 3:
1. Pulmonary arterial systolic pressure elevated to at least 70
percent of the systemic arterial systolic pressure; or
2. Pulmonary arterial systolic pressure equal to or greater than
70 mmHg; or
3. Mean pulmonary artery pressure equal to or greater than 40
mmHg.
OR
C. Single ventricle (for example, hypoplastic left or right
ventricle) that has or will require Fontan procedures (see
104.00D4).
OR
D. For infants under 1 year of age at the time of filing, with
life-threatening congenital heart disease (see 104.00D2c) that will
require or already has required surgical treatment in the first year
of life, and the impairment is expected to be disabling (because of
residual impairment following surgery, or the recovery time
required, or both) until the attainment of at least 1 year of age,
consider under a disability until the attainment of at least age 1;
after that, evaluate impairment severity with the appropriate
listing.
OR
E. Exacerbations or complications of congenital heart disease
(see 104.00D) requiring three hospitalizations within a consecutive
12-month period (see 104.00A3e) and at least 30 days apart. Each
hospitalization must last at least 48 hours, including hours in a
hospital emergency department immediately before the hospitalization
(see 104.00G3).
104.07-104.08 [Reserved]
104.09 Heart transplantation (see 104.00F5). Consider under a
disability for 1 year from the date of the transplant; after that,
evaluate the residual impairment(s).
104.10-104.15 [Reserved]
104.16 Cardiac allograft vasculopathy (see 104.00F11),
documented by appropriate medically acceptable imaging (for example,
intravascular ultrasonography or coronary angiography).
* * * * *
114.00 Immune System Disorders
* * * * *
J. * * *
2. * * *
m. Syphilis or neurosyphilis under the criteria for the affected
body system; for example, 102.00 Special senses and speech, 104.00
Cardiovascular disorders, or 111.00 Neurological.
* * * * *
[FR Doc. 2026-13420 Filed 7-1-26; 8:45 am]
BILLING CODE 4191-02-P