[Federal Register Volume 87, Number 124 (Wednesday, June 29, 2022)]
[Proposed Rules]
[Pages 38838-38867]
From the Federal Register Online via the Government Publishing Office [www.gpo.gov]
[FR Doc No: 2022-12980]
[[Page 38837]]
Vol. 87
Wednesday,
No. 124
June 29, 2022
Part II
Social Security Administration
-----------------------------------------------------------------------
20 CFR Part 404
Revised Medical Criteria for Evaluating Cardiovascular Disorders;
Proposed Rule
Federal Register / Vol. 87, No. 124 / Wednesday, June 29, 2022 /
Proposed Rules
[[Page 38838]]
-----------------------------------------------------------------------
SOCIAL SECURITY ADMINISTRATION
20 CFR Part 404
[Docket No. SSA-2019-0013]
RIN 0960-AI43
Revised Medical Criteria for Evaluating Cardiovascular Disorders
AGENCY: Social Security Administration.
ACTION: Notice of proposed rulemaking (NPRM).
-----------------------------------------------------------------------
SUMMARY: We propose to revise some of 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 proposed revisions reflect
advances in medical knowledge, our adjudicative experience, and
comments we received from experts and the public in response to an
advance notice of proposed rulemaking (ANPRM), and at an outreach
policy conference.
DATES: To ensure that your comments are considered, we must receive
them by no later than August 29, 2022.
ADDRESSES: You may submit comments by any one of three methods--
internet, fax, or mail. Do not submit the same comments multiple times
or by more than one method. Regardless of which method you choose,
please state that your comments refer to Docket No. SSA-2019-0013, so
that we may associate your comments with the correct regulation.
Caution: You should be careful to include in your comments only
information that you wish to make publicly available. We strongly urge
you not to include in your comments any personal information, such as
Social Security numbers or medical information.
1. Internet: We strongly recommend that you submit your comments
via the internet. Please visit the Federal eRulemaking portal at http://www.regulations.gov. Use the search function to find docket number
SSA-2019-0013. The system will issue a tracking number to confirm your
submission. You will not be able to view your comment immediately
because we must post each comment manually. It may take up to a week
for your comment to be viewable.
2. Fax: Fax comments to (410) 966-2830.
3. Mail: Address your comments to the Office of Regulations and
Reports Clearance, Social Security Administration, 3100 West High Rise,
6401 Security Boulevard, Baltimore, Maryland 21235-6401.
Comments are available for public viewing on the Federal
eRulemaking portal at http://www.regulations.gov or in person, during
regular business hours, by arranging with the contact person identified
below.
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
For adults, the listings describe, for each of the major body
systems, impairments that we consider to be severe enough to prevent an
individual from doing any gainful activity regardless of his or her
age, education, or work experience.\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 in which the
individual is clearly disabled under our rules.\3\ However, we do not
deny any claim solely because a person's medical impairment(s) does not
satisfy the criteria of a listing.
---------------------------------------------------------------------------
\1\ 20 CFR 404.1525(a) and 20 CFR 416.925(a).
\2\ 20 CFR 416.925(a).
\3\ 20 CFR 404.1520, 20 CFR 416.920, and 20 CFR 416.924.
---------------------------------------------------------------------------
Why are we proposing to revise the listings for cardiovascular
disorders?
We last published final rules that comprehensively revised the
cardiovascular disorders listings on January 13, 2006, and the rules
became effective on April 13, 2006.\4\ We are now proposing targeted
revisions to the cardiovascular disorders listings, as previously
mentioned, to reflect advances in medical knowledge, our adjudicative
experience, and comments we received from experts and the public in
response to an ANPRM, and at an outreach policy conference.
---------------------------------------------------------------------------
\4\ 71 FR 2312 (2006).
---------------------------------------------------------------------------
How did we develop this proposed rule?
In developing this proposed rule:
We published an ANPRM for cardiovascular disorders in the
Federal Register on April 16, 2008.\5\ We invited the public to send us
written comments and suggestions about whether and how we should revise
the cardiovascular disorders listings. We received five comments on the
ANPRM. The commenters made several suggestions that we incorporated
into the proposals, such as consideration for people with a single
ventricle; clarifying in proposed 4.00D(4)(c)(ii) (How do we evaluate
CHF using 4.02?) and 4.02B1 (Chronic heart failure) that when we
evaluate a person's ability to perform activities of daily living, we
will also consider the person's ability to perform them effectively;
\6\ and providing more examples of skin examination findings that might
accompany venous insufficiency. Two commenters asked us to base our
proposals on the American College of Cardiology/American Heart
Association (ACC/AHA) clinical practice guidelines and one suggested we
review and incorporate the American Society of Echocardiography (ASE)
guidelines. As a result, we tasked a committee of medical experts with
reviewing and analyzing the ACC/AHA and ASE guidelines to inform the
recommendations in the Institute of Medicine (IOM) \7\ report titled,
``Cardiovascular Disability: Updating the Social Security Listings.''
\8\ This report, which is discussed in more detail below, informed some
of the proposed changes in this NPRM.
---------------------------------------------------------------------------
\5\ 73 FR 20564 (2008).
\6\ In current listing 4.02B1 (Chronic heart failure), we
require persistent symptoms of heart failure ``which very seriously
limit the person's ability to independently initiate, sustain or
complete activities of daily living.'' Consistent with the
commenter's suggestion and how we assess functional limitations in
the adult mental disorders listings (12.00), in proposed 4.02B1, we
require ``a very serious limitation in the ability to perform
activities of daily living independently, appropriately,
effectively, and on a sustained basis.''
\7\ On April 28, 2015, the membership of the National Academy of
Sciences 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.
\8\ Institute of Medicine (IOM). (2010). Cardiovascular
Disability: Updating the Social Security Listings. Washington, DC:
The National Academies Press.
---------------------------------------------------------------------------
On September 24 and 25, 2008, we hosted a policy
conference titled ``Cardiovascular Disorders in the Disability
Programs'' in Baltimore, Maryland. At this conference, we received
public comments and suggestions from physicians and advocacy groups for
updating and revising our criteria for evaluating cardiovascular
disorders. Physicians and advocacy groups specifically discussed the
evaluation of chronic heart failure, ischemic heart disease, peripheral
artery disease, and chronic
[[Page 38839]]
venous insufficiency. Participants made several suggestions that we
researched and incorporated into the proposals, such as distinguishing
cardiovascular disorders from pulmonary disorders by using biomarkers
such as B-type natriuretic peptide (BNP).
In 2009, we commissioned a study by an ad hoc committee of
medical experts appointed by the Institute of Medicine (IOM). The
committee: (1) conducted a comprehensive review of the relevant
research literature and current professional practice guidelines
developed jointly by the ACC/AHA; (2) assessed the current criteria in
light of current research knowledge and evidence-based medical
practice; and (3) produced a report with specific recommendations for
revision of the criteria based on evidence and professional judgment.
The committee provided its recommendations in a 2010 report titled,
``Cardiovascular Disability: Updating the Social Security Listings.''
\9\ We recently sought guidance from our cardiologists and other
medical experts, reviewed disability claims involving cardiovascular
disorders, and reviewed current research to ensure the IOM
recommendations are still relevant.
---------------------------------------------------------------------------
\9\ IOM. (2010).
---------------------------------------------------------------------------
Recommendations we received from the IOM report, responses to the
ANPRM, and the ``Cardiovascular Disorders in the Disability Programs''
policy conference informed the proposed changes in this NPRM. As with
the IOM report, we have conducted independent medical research,
consulted with agency cardiologists, and reviewed disability claims
involving cardiovascular disorders to ensure the accuracy and relevance
of these stated resources. In developing this proposed rule, we also
considered information from several other sources, including:
Medical experts in cardiology from SSA's Office of Medical
Assistance \10\ who assist in the development and evaluation of policy
and whom we regularly consulted with in drafting these proposals;
---------------------------------------------------------------------------
\10\ SSA's Office of Medical Assistance (OMA) provides medical
and analytical support to ensure accurate and consistent disability
policy and procedure application. In addition to a full complement
of subject matter experts who are permanent SSA staff, OMA contracts
with medical and psychological consultants to provide medical
expertise in the development and evaluation of policy.
---------------------------------------------------------------------------
Advocacy groups for people with cardiovascular disorders
and individuals with cardiovascular disorders and their families who
submitted comments on the ANPRM or participated in the 2008
``Cardiovascular Disorders in the Disability Program'' policy
conference;
Individuals who make and review disability determinations
and decisions for us in State agencies; in our Office of Hearings
Operations; and in our Office of Analytics, Review, and Oversight; and
The published sources of medical literature and research
we list in the references section at the end of this preamble.
What revisions are we proposing for cardiovascular disorders?
We propose to:
Change the name of the body system from ``Cardiovascular
System'' to ``Cardiovascular Disorders'' to be consistent with the
nomenclature of all body systems in our listings;
Reorganize and revise the introductory text (section 4.00
for adults and 104.00 for children) to provide guidance for using the
revised criteria in the listings;
Revise the adult and childhood listings for chronic heart
failure (4.02 and 104.02), recurrent arrhythmias (4.05 and 104.05),
symptomatic congenital heart disease (4.06) and congenital heart
disease (104.06), and heart transplant (4.09 and 104.09);
Revise the adult listings for ischemic heart disease (IHD)
(4.04), chronic venous insufficiency (4.11), and peripheral arterial
disease (4.12);
Add adult listings for aortic valvular disease (4.07) and
cardiomyopathy (4.08);
Add adult and childhood listings for cardiac allograft
vasculopathy (4.16 and 104.16);
Remove childhood listing for rheumatic heart disease
(104.13) and reserve listing number 104.13; and
Make minor editorial revisions, including changes to
conform to revised rules for evaluating medical evidence,\11\ to the
introductory text and to the listings for clarity.
---------------------------------------------------------------------------
\11\ 82 FR 5844 (2017).
---------------------------------------------------------------------------
Proposed Changes to the Adult Cardiovascular Disorders Introductory
Text
The following table shows the heading of the current and proposed
sections of the adult introductory text for cardiovascular disorders:
Introductory Text 4.00
------------------------------------------------------------------------
------------------------------------------------------------------------
Current Sections of the Adult Proposed Sections of the Adult
Introductory Text for Cardiovascular Introductory Text for
System. Cardiovascular Disorders.
4.00 Cardiovascular System............. 4.00 Cardiovascular Disorders.
A. General............................. A. How do we define
cardiovascular disorders and
cardiovascular terms?
B. Documenting Cardiovascular B. What documentation do we
Impairment. need to evaluate
cardiovascular disorders?
C. Using Cardiovascular Test Results... C. How do we use cardiovascular
test results?
D. Evaluating Chronic Heart Failure.... D. How do we evaluate chronic
heart failure?
E. Evaluating Ischemic Heart Disease... E. How do we evaluate ischemic
heart disease?
F. Evaluating Arrhythmias.............. F. How do we evaluate
arrhythmias?
G. Evaluating Peripheral Vascular G. How do we evaluate
Disease. peripheral vascular disease?
H. Evaluating Other Cardiovascular H. How do we evaluate
Impairments. congenital heart disease?
I. Other Evaluation Issues............. I. How do we evaluate other
cardiovascular disorders?
J. How do we evaluate issues
that affect the cardiovascular
system?
K. How do we evaluate
cardiovascular disorders that
do not meet one of these
listings?
------------------------------------------------------------------------
[[Page 38840]]
Proposed 4.00--Introductory Text to the Adult Cardiovascular Disorders
Listings
The following is a detailed description of the primary changes we
are proposing to the introductory text. In addition to the changes we
describe below, we are proposing minor changes to the introductory text
to clarify how we use the proposed listings to evaluate cardiovascular
disorders, changes to be consistent with current medical terminology,
the language we use in other body system listings, and the revised
rules for evaluating medical evidence.\12\ We repeat much of the
introductory text of proposed 4.00 in the introductory text of proposed
104.00 (the introductory text to the childhood cardiovascular disorders
listings), making distinctions where needed. This is necessary because
the same basic criteria for evaluating cardiovascular disorders apply
to both adults and children.
---------------------------------------------------------------------------
\12\ Id.
---------------------------------------------------------------------------
Proposed 4.00A--How do we define cardiovascular disorders and
cardiovascular terms?
To improve clarity and promote consistent understanding of the
terms we use in these listings, we propose:
In 4.00A3b (Persistent), to clarify that ``exceptions''
means 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;
In 4.00A3c (Recurrent), to clarify in our definition of
``recurrent'' that the term ``improvement of sufficient duration''
means the finding is greatly reduced (for example, treatment reduced a
grade 3 chronic venous insufficiency (CVI) skin ulcer to a grade 1 CVI
skin ulcer) or not present for long enough that the required finding(s)
is no longer a factor in the person's condition.
In 4.00A3f (Uncontrolled) we would remove the definition
for the term ``uncontrolled'' because we propose to eliminate the term
as a descriptor for recurrent episodes of cardiac syncope. The
definition of ``uncontrolled'' was redundant after describing
reoccurring episodes of cardiac syncope despite treatment.
Proposed 4.00C--How do we use cardiovascular test results?
We propose:
In 4.00C8d(iv) (When will we not purchase an exercise test
or wait before we purchase an exercise test?), to include the procedure
percutaneous coronary intervention (PCI) to be consistent with medical
advancements that indicate a PCI is a nonsurgical procedure to improve
blood flow to the heart.\13\
---------------------------------------------------------------------------
\13\ IOM. (2010), 109.
---------------------------------------------------------------------------
In 4.00C15 (How do we evaluate cardiac catheterization
evidence?), to remove 4.00C15b and 4.00C15c and create a new 4.00C15b
to simplify our explanation of cardiac catheterization reports and to
include information about fractional flow reserve (FFR), which we use
in the proposed 4.04D1 (Ischemic heart disease).
Proposed 4.00D--How do we evaluate chronic heart failure?
We propose:
In 4.00D1a (What is chronic heart failure (CHF)?), to
provide a more descriptive definition of ``ejection fraction'' for
clarity;
In 4.00D1b (What is chronic heart failure (CHF)?), to
explain that high blood levels of the proteins B-type natriuretic
peptide (BNP) and N-terminal pro-BNP (NT-pro-BNP) may help identify
chronic heart failure as the cause of a person's symptoms (for example,
shortness of breath);Sec. 14 15 16 17
---------------------------------------------------------------------------
\14\ Cohen-Solal, A., Laribi, S., Ishihara, S., Vergaro, G.,
Baudet, M., Logeart, D., . . . Seronde, M.-F. (2015). Prognostic
markers of acute decompensated heart failure: The emerging roles of
cardiac biomarkers and prognostic scores. Archives of Cardiovascular
Disease, 108(1), 64-74. doi:10.1016/j.acvd.2014.10.002.
\15\ Desai, A.S. (2013). Are serial BNP measurements useful in
heart failure management? Serial natriuretic peptide measurements
are not useful in heart failure management: The art of medicine
remains long. Circulation, 127(4), 509-516. doi:10.1161/
CIRCULATIONAHA.112.120493.
\16\ Patterson, C.C., Blankenberg, S., Ben-Shlomo, Y., Heslop,
L., Bayer, A., Lowe, G., . . . Yarnell, J. (2015). Which biomarkers
are predictive specifically for cardiovascular or for non-
cardiovascular mortality in men? Evidence from the Caerphilly
Prospective Study (CaPS). International Journal of Cardiology, 201,
113-118. doi:10.1016/j.ijcard.2015.07.106.
\17\ Uszko-Lencer, N.H., Frankenstein, L., Spruit, M.A., Maeder,
M.T., Gutmann, M., Muzzarelli, S., . . . Brunner-La Rocca, H.-P.
(2017). Predicting hospitalization and mortality in patients with
heart failure: The BARDICHE-index. International Journal of
Cardiology, 227, 901-907. doi:10.1016/j.ijcard.2016.11.122.
---------------------------------------------------------------------------
In 4.00D2a(i) (What evidence of CHF do we need?) and
4.00D2a(iii) (What evidence of CHF do we need?), to explain left atrial
volume index (LAVi) and how it is calculated. LAVi is a new measurement
used in criterion A2 of proposed listing 4.02 (Chronic heart failure)
to provide a more precise representation of increased left atrial
pressure;
In 4.00D4c (How do we evaluate CHF using 4.02? ),to
describe in more detail the two-part process we use in criteria B1 of
proposed listing 4.02 (Chronic heart failure) to evaluate chronic heart
failure if a person cannot perform an exercise tolerance test (ETT);
\18\ and
---------------------------------------------------------------------------
\18\ We define an exercise tolerance test at 4.00C3b (What are
exercise tests and what are they used for?) as ``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.'' This is an existing
definition coming from existing regulation.
---------------------------------------------------------------------------
Add 4.00D4e (How do we evaluate CHF treated with a
mechanical circulatory support device?) to explain mechanical
circulatory support devices (MCSD) and clarify how we would evaluate
individuals treated for heart failure with a MCSD.\19\ We propose to
evaluate MCSDs under proposed 4.02D1 (Chronic heart failure )to account
for cardiac bridge treatment which we currently evaluate under listing
4.09 (Heart transplantation).
---------------------------------------------------------------------------
\19\ Ciarka, A., Edwards, L., Nilsson, J., Stehlik, J., & Lund,
L.H. (2017). Trends in the use of mechanical circulatory support as
a bridge to heart transplantation across different age groups.
International Journal of Cardiology, 231, 225-227. doi:10.1016/
j.ijcard.2016.10.049.
---------------------------------------------------------------------------
Proposed 4.00E--How do we evaluate ischemic heart disease?
We propose:
In 4.00E9b (How do we evaluate IHD using 4.04?), to
consolidate and revise the guidance that is currently in 4.00E9b, c, d,
and e for ease of reference;
In 4.00E9b (How do we evaluate IHD using 4.04?), to
explain that we will use an interpretation of electrocardiogram (ECG)
findings by an acceptable medical source (AMS) \20\ if the
interpretation concludes that the ECG findings are positive for IHD.
Interpreting ECG results requires a systematic review and analysis of
several components, including relevant clinical details and raw data.
Relying on an interpretation by an AMS is consistent with our rules for
establishing a medically determinable impairment (MDI) and will ensure
the accuracy of adjudication for claims involving IHD because
interpretation of ECG findings requires medical judgment; \21\
---------------------------------------------------------------------------
\20\ 20 CFR 404.1502(a) and 416.902(a).
\21\ 20 CFR 404.1521 and 416.921.
---------------------------------------------------------------------------
In 4.00E9c (How do we evaluate IHD using 4.04?), to
clarify that revascularizations that result from unplanned
hospitalizations must be an emergency and unplanned; \22\
---------------------------------------------------------------------------
\22\ As we explain in 4.00E9c, revascularization means
angioplasty (with or without stent placement) or bypass surgery.
---------------------------------------------------------------------------
To redesignate current 4.00E9f, g, and h (How do we
evaluate IHD using 4.04?) as proposed new sections 4.00E9c, d, and f,
respectively; and
In 4.00E9e ( How do we evaluate IHD using 4.04?), to add a
definition for
[[Page 38841]]
the term ``fractional flow reserve (FFR),'' which is an objective
measure of flow access across an obstruction that we use in criterion
D1 of proposed listing 4.04 (Ischemic heart disease).\23\
---------------------------------------------------------------------------
\23\ Fearon, W.F. (2014). Percutaneous coronary intervention
should be guided by fractional flow reserve measurement.
Circulation, 129(18), 1860-1870. doi:10.1161/
CIRCULATIONAHA.113.004300.
---------------------------------------------------------------------------
Proposed 4.00G--How do we evaluate peripheral vascular disease?
In 4.00G6 (Are there any other studies that are helpful in
evaluating PAD?), as IOM advised, we propose to provide more
information about imaging and other tests used to diagnose peripheral
arterial disease (PAD) to clearly convey our intent of the listing,
which is to tie PAD to mobility.\24\
---------------------------------------------------------------------------
\24\ IOM. (2010), 151.
---------------------------------------------------------------------------
Proposed 4.00H--How do we evaluate congenital heart disease?
We propose:
To redesignate and rename some paragraphs for ease of
reference;
In 4.00H (How do we evaluate congenital heart disease?),
to add a definition for the term ``single ventricle,'' and include more
detailed guidance on how we evaluate congenital heart disease as
recommended by the IOM; and
To add 4.00H1 (What is congenital heart disease?), 4.00H2
(What is Eisenmenger syndrome?), 4.00H3 (What is single ventricle?),
and 4.004H4 (How do we evaluate conditions associated with congenital
heart disease?).
Proposed 4.00I--How do we evaluate other cardiovascular disorders?
We propose:
To rename and rearrange the content of 4.00I (How do we
evaluate other cardiovascular disorders?), including redesignating some
paragraphs, for ease of reference;
In 4.00I2 (What is cardiomyopathy and how will we evaluate
it?), to explain how we would evaluate cardiomyopathy under proposed
new 4.08 (Cardiomyopathy);
In 4.00I3 (How do we evaluate valvular heart disease?), to
explain that we would evaluate valvular heart disease under the
proposed new 4.07 (Aortic valvular disease);
In 4.00I4 (What do we consider when we evaluate heart
transplant recipients?), to explain that we would evaluate cardiac
allograft vasculopathy under proposed new 4.16 (Cardiac allograft
vasculopathy); and
To add 4.00I5 (What is cardiac allograft vasculopathy and
how do we evaluate it?), to explain proposed new 4.16 (Cardiac
allograft vasculopathy).
Proposed 4.00J--How do we evaluate issues that affect the
cardiovascular system?
We propose:
To redesignate and rename some paragraphs for ease of
reference;
In 4.00J1 (How do we consider the effects of obesity when
we evaluate your cardiovascular disorder?), to simplify and refocus our
discussion of how we consider the effects of obesity more specifically
on cardiovascular disorders;
To add 4.00J3 (How do we consider hospitalizations?) to
explain how we would evaluate hospitalizations for repeated
exacerbations and complications of cardiovascular disorders under
proposed 4.02B3 (Chronic heart failure), 4.04E (Ischemic heart
disease), 4.06E (Congenital heart disease), and 4.08D (Cardiomyopathy).
Proposed 4.00K--How do we evaluate cardiovascular disorders that do
not meet one of these listings?
We propose to rename and redesignate 4.00I3 (How do we
evaluate impairments that do not meet one of the cardiovascular
listings?) as 4.00K and redesignate 4.00I3a and 4.00I3b as 4.00K1 and
4.00K2 for ease of reference.
Proposed Changes to the Adult Cardiovascular Disorders Listings
The following table shows the heading of the current and proposed
sections of the adult listings for cardiovascular disorders:
Adult Cardiovascular Disorders Listings
------------------------------------------------------------------------
Current Proposed
------------------------------------------------------------------------
4.02 Chronic heart failure............. 4.02 Chronic heart failure.
4.04 Ischemic heart disease............ 4.03 [Reserved].
4.05 Recurrent arrhythmias............. 4.04 Ischemic heart disease.
4.06 Symptomatic congenital heart 4.05 Recurrent arrhythmias.
disease.
4.09 Heart transplant.................. 4.06 Congenital heart disease.
4.10 Aneurysm of aorta or major 4.07 Aortic valvular disease.
branches.
4.11 Chronic venous insufficiency...... 4.08 Cardiomyopathy.
4.12 Peripheral arterial disease....... 4.09 Heart transplantation.
4.10 Dissecting aneurysm of the
aorta or major branches.
4.11 Chronic venous
insufficiency.
4.12 Peripheral arterial
disease.
4.13 [Reserved].
4.14 [Reserved].
4.15 [Reserved].
4.16 Cardiac allograft
vasculopathy.
------------------------------------------------------------------------
[[Page 38842]]
The following table shows our proposed changes to the adult
cardiovascular disorders listings criteria that involve changes to
healthcare utilization and condition/episode requirements, the
rationale for each change, and supporting resource. Following this
table, we discuss all of our proposed changes to the adult
cardiovascular disorders listings in more detail.
Adult Cardiovascular Disorders Listings Criteria--Changes in Healthcare Utilization and Condition/Episode
Requirements
----------------------------------------------------------------------------------------------------------------
Proposed listing
Current listing criterion criterion Rationale Resources
----------------------------------------------------------------------------------------------------------------
Listing 4.02 Chronic heart failure
----------------------------------------------------------------------------------------------------------------
4.02 A1--A. Medically documented A. Medically Proposed criterion 4.02A1 IOM. (2010), 88, 89.
presence of one of the following: documented presence requires an increased
1. Systolic failure (see of one of the left ventricular end
4.00D1a(i)), with left following: diastolic dimension
ventricular end diastolic 1. Systolic failure (LVEDD) equal to or
dimensions greater than 6.0 cm or documented by greater than 7.0
ejection fraction of 30 percent appropriate centimeters (cm) instead
or less during a period of medically acceptable of the current criterion
stability (not during an episode imaging during a of an LVEDD greater than
of acute heart failure); or. period of stability 6.0 cm. We followed the
(not during an IOM's recommendation in
episode of determining that an
exacerbation of increased LVEDD of
heart failure), with greater than 6.0 cm but
left ventricular end less than 7.0 cm
diastolic dimension indicates only a
equal to or greater moderately enlarged
than 7.0 cm; or heart, and an increased
ejection fraction of LVEDD of at least 7.0 cm
30 percent or less more clearly establishes
during a period of an enlarged heart with
stability (not signs and symptoms
during an episode of indicating listing-level
acute heart failure). heart failure.
4.02B2--Three or more separate B.3. Exacerbations or We propose to remove IOM. (2010), 89, 91.
episodes of acute congestive complications of current 4.02B2 ``three or
heart failure within a chronic heart more separate episodes of
consecutive 12-month period (see failure (see acute congestive heart
4.00A3e), with evidence of fluid 4.00D1b) requiring failure'' because we
retention (see 4.00D2b(ii)) from three would evaluate these
clinical and imaging assessments hospitalizations episodes under proposed
at the time of the episodes, within a consecutive 4.02B3. As recommended by
requiring acute extended 12-month period (see the IOM, proposed 4.02B3
physician intervention such as 4.00A3e) and at would evaluate
hospitalization or emergency room least 30 days apart. exacerbations or
treatment for 12 hours or more, Each hospitalization complications of CHF,
separated by periods of must last at least requiring three
stabilization (see 4.00D4c); 48 hours, including hospitalizations within a
hours in a hospital consecutive 12-month
emergency department period and at least 30
immediately before days apart. An impairment
the hospitalization resulting in
(see 4.00J3). exacerbations or
complications that
require this many
hospitalizations in 12
months is a very severe
impairment. We would
require these
hospitalizations to be at
least 30 days apart to
ensure we are evaluating
separate episodes of
exacerbations or
complications.
No current listing criteria....... C. Heart failure with The IOM recommended a IOM. (2010), 84, 89.
left ventricular criterion for chronic Desai, R.V., Guichard,
ejection fraction of heart failure with an EF J.L., Mujib, M., Ahmed,
20 percent or less on a sustained basis of M.I., Feller, M.A.,
while on a regimen 20 percent or less. An EF Fonarow, G.C., . . .
of prescribed of only 20 percent means Ahmed, A. (2013).
therapy, on two the heart's pumping Reduced right
evaluations at least action is less than a ventricular ejection
90 days apart within third of normal, and fraction and increased
a consecutive 12- critically affects a mortality in chronic
month period (see person's ability to systolic heart failure
4.00A3e) during a perform gainful activity. patients receiving beta-
period of stability blockers: Insights from
(not during an the BEST trial.
episode of International Journal of
exacerbation of Cardiology, 163(1), 61-
heart failure); 67. doi:10.1016/
j.ijcard.2011.05.051.
Runge, M.S., Patterson,
C., Stouffer, G.A., &
Netter, F.H. (2010).
Netter's Cardiology (2nd
ed.). Philadelphia, PA:
Saunders Elsevier.
[[Page 38843]]
No current listing criteria....... D. One of the Implanted MCSDs help the Malotte, K., Saguros, A.,
following while heart pump blood and may & Groninger, H. (2018).
hospitalized, at be used as a ``bridge'' Continuous cardiac
home, or both: while a person waits for inotropes in patients
1. Mechanical a heart transplant. We with end-stage heart
circulatory support currently use our medical failure: An evolving
device except equivalence rules to find experience. Journal of
extracorporeal someone with heart Pain Symptom Management,
membrane oxygenation failure and an implanted 55(1), 159-163.
(ECMO) (see MCSD disabled under doi:10.1016/
4.00D4e). Consider listing 4.09 (Heart j.jpainsymman.2017.09.02
under a disability transplantation). Adding 6.
for 1 year from the this new criterion will Bistola, V., Arfaras-
date of ensure that people with Melainis, A.,
implantation; after heart failure treated Polyzogopoulou, E.,
that, evaluate any with MCSD are Ikonomidis, I., &
residual consistently identified. Parissis, J. (2019).
impairment(s) under People who require Inotropes in acute heart
the criteria for the continuous intravenous failure: From guidelines
affected body system. administration of to practical use:
2. Continuous inotropic medication (for Therapeutic options and
intravenous example, milrinone) have clinical practice.
administration of very serious heart Cardiac Failure Review,
inotropic medication failure, and the length 5(3), 133-139.
(for example, of this treatment can be doi:10.15420/
milrinone) for at an accurate predictor of cfr.2019.11.2.
least 30 consecutive impairment severity.
days. Consider under Accordingly, proposed
a disability for 1 4.02D2 would find people
year from the date disabled if they require
of initiation of the continuous intravenous
treatment; after inotropic medications for
that, evaluate any 30 or more consecutive
residual days.
impairment(s) under
the criteria for the
affected body system.
----------------------------------------------------------------------------------------------------------------
Listing 4.04 Ischemic heart disease
----------------------------------------------------------------------------------------------------------------
4.04B--Three separate ischemic 4.04C--Documentation We would evaluate Hua, M., Gong, M.N.,
episodes, each requiring of three separate unplanned Brady, J., & Wunsch, H.
revascularization or not amenable ischemic episodes hospitalizations under (2015). Early and late
to revascularization (see (see 4.00E9c) this section to ensure we unplanned
4.00E9f), within a consecutive 12- requiring unplanned are only evaluating rehospitalizations for
month period (see 4.00A3e). hospitalization urgent ischemic episodes. survivors of critical
(inpatient or Individuals who have illness. Critical Care
observation status) ischemic episodes that Medicine, 43(2), 430-
within a consecutive result in unplanned 438. doi:10.1097/
12-month period (see hospitalizations may need CCM.0000000000000717.
4.00A3e). intensive care, can have
long hospital stays, may
require multiple
procedures, and can be at
high risk for post-
discharge morbidity and
mortality.
No current criterion for repeated 4.04E--Exacerbations An impairment resulting in Hua, M., Gong, M.N.,
exacerbations or complications or complications of exacerbations or Brady, J., & Wunsch, H.
ischemic heart disease, 4.04B ischemic heart complications that (2015). Early and late
(above) only considers episodes disease (see 4.00E2- require three or more unplanned
requiring revascularization or 4.00E7) requiring hospitalizations in 12 rehospitalizations for
that are not amenable to three months is a very severe survivors of critical
revascularization. hospitalizations impairment. We would illness. Critical Care
within a consecutive require these Medicine, 43(2), 430-
12-month period (see hospitalizations to be at 438. doi:10.1097/
4.00A3e) and at least 30 days apart to CCM.0000000000000717.
least 30 days apart. ensure we are evaluating
Each hospitalization separate episodes of
must last at least exacerbations or
48 hours, including complications of ischemic
hours in a hospital heart disease.
emergency department
immediately before
the hospitalization
(see 4.00E9c).
[[Page 38844]]
Listing 4.06 Congenital heart disease
----------------------------------------------------------------------------------------------------------------
4.06A--A. Cyanosis at rest, and: A. Chronic hypoxemia, We propose to revise Stout, K.K., Daniels,
1. Hematocrit of 55 percent or and 1, 2, or 3: current 4.06A to require C.J., Aboulhosn, J.A.,
greater; or. 1. Hematocrit of 55 ``hypoxemia'' rather than Bozkurt, B., Broberg,
2. Arterial O2 saturation of less percent or greater ``cyanosis or C.S., Colman, J.M., . .
than 90 percent in room air or on two evaluations acyanosis''. Cyanosis is . Van Hare, G.F. (2019).
resting arterial PO2 of 60 Torr at least 90 days a more subjective 2018 AHA/ACC Guideline
or less. apart within a assessment subject to for the management of
consecutive 12-month misinterpretation due to adults with congenital
period (see by many factors, heart disease: A report
4.00A3e); or. including skin of the American College
2. Arterial blood gas complexion. Thus, the of Cardiology/American
test measurement term ``hypoxemia'' Heart Association Task
obtained at rest relates more to the Force on Clinical
while breathing room laboratory and pulse Practice Guidelines.
air, as described in oximetry findings than Journal of the American
either a or b:. the term ``cyanosis. College of Cardiology,
a. SaO2 (arterial We would require two 73(12), e81-e192.
oxygen saturation) hematocrit measurements doi:10.1016/
less than or equal instead of the current j.jacc.2018.08.1029.
to 89 percent; or. listing's single Stack, S.W, . . . &
b. PO2 or PaO2 measurement. Two Berger, S.A. (2009). The
(partial pressure of measurements, at least 90 effects of high
oxygen) less than or days apart within a hematocrit arterial
equal to 60 mmHg;. consecutive 12-month flow--A phenomenological
3. SpO2 (percentage period will help ensure study of health risk
of oxygen saturation the person's hematocrit implications. Chemical
of blood hemoglobin) level is associated with Engineering Science,
measured by pulse chronic hypoxemia and not 64(22), 4701-4706.
oximetry either at the result of a doi:10/1016/
rest, during a 6- reversible condition. j.ces.2009.07.017.
minute walk test Proposed 4.06A3 would IOM. (2010), 178.
(6MWT), or after a require three SpO2 Oster, M.E, . . . &
6MWT, while measurements 30 days Kochilas, L.K. (2016).
breathing room air, apart within a Screening for critical
less than or equal consecutive 12-month congenital heart
to 87 percent on period showing hypoxemia. disease. Clinics in
three evaluations at This will document that Perinatology, 43(1), 73-
least 30 days apart the condition is chronic 80. doi:10.1016/
within a consecutive and persistent, and the j.clp.2015.11.005.
12-month period (see measurements are not Mechem, C.C. (2014).
4.00A3e). related to a reversible Pulse oximetry. In P.E.
condition or an Parsons (Ed.), UpToDate
inaccurate reading. (Jan. 2014). Retrieved
We would add a criterion from https://
for SpO2 (percentage of www.uptodate.com/
oxygen saturation of contents/pulse-oximetry.
blood hemoglobin)
measured by pulse
oximetry, including
measurements taken while
the person is at rest or
while doing a six-minute
walk test (6MWT). Pulse
oximetry measurements are
a non-invasive
alternative to invasive
testing. A person's
medical evidence often
provides SpO2 findings,
and SpO2 measured by
pulse oximetry reflects
an advance in medical
technology that provides
another way to establish
listing-level severity.
No current criteria............... 4.06E--Exacerbations An impairment resulting in Hua, M., Gong, M.N.,
or complications of exacerbations or Brady, J., & Wunsch, H.
congenital heart complications that (2015). Early and late
disease (see 4.00J3) require three or more unplanned
requiring three hospitalizations in 12 rehospitalizations for
hospitalizations months is a very severe survivors of critical
within a consecutive impairment. We would illness. Critical Care
12-month period (see require these Medicine, 43(2), 430-
4.00A3e) and at hospitalizations to be at 438. doi:10.1097/
least 30 days apart. least 30 days apart to CCM.0000000000000717.
Each hospitalization ensure we are evaluating
must last at least separate episodes of
48 hours, including exacerbations or
hours in a hospital complications.
emergency department
immediately before
the hospitalization
(see 4.00J3).
----------------------------------------------------------------------------------------------------------------
Listing 4.08 Cardiomyopathy
----------------------------------------------------------------------------------------------------------------
No current listing................ 4.08D--D. Consistent with IOM IOM. (2010), 80.
Exacerbations or recommendations, we In addition, SSA has
complications of created this new designated
cardiomyopathy cardiomyopathy listing to endomyocardial fibrosis
requiring three specifically address and cardiac amyloidosis
hospitalizations hypertrophic AL type as Compassionate
within a consecutive cardiomyopathy, Allowance (CAL)
12-month period (see endomyocardial fibrosis, conditions. See
4.00A3e) and at and cardiac amyloidosis Compassionate Allowances
least 30 days apart. AL type, which are more Website Home Page
Each hospitalization serious types of (ssa.gov).
must last at least cardiomyopathy.
48 hours, including
hours in a hospital
emergency department
immediately before
the hospitalization
(see 4.00J3).
[[Page 38845]]
Listing 4.11 Chronic venous insufficiency
----------------------------------------------------------------------------------------------------------------
4.11--Chronic venous insufficiency 4.11--Chronic venous As recommended by IOM, we IOM. (2010), 161.
of a lower extremity with insufficiency (see would require
incompetency or obstruction of 4.00G) of a lower confirmation of CVI by
the deep venous system and one of extremity with duplex ultrasound or
the following: reflux or other appropriate
obstruction of the diagnostic technique. The
venous system medical community
documented by duplex considers the use of
ultrasound or other duplex ultrasound to be
appropriate the best method for
diagnostic detecting reflux or
technique, with A or obstruction.
B:
4.11A--A. Extensive brawny edema A. Extensive trophic We would adopt IOM IOM. (2010), 157-161.
(see 4.00G3) involving at least changes of skin (for recommendations and
two-thirds of the leg between the example, broaden the listing
ankle and knee or the distal one- hyperpigmentation, criteria we apply to
third of the lower extremity lipodermatosclerosis trophic changes of the
between the ankle and hip. , brawny edema) skin. For example, in
involving at least addition to brawny edema,
two-thirds of the trophic changes evaluated
leg below the knee, under the proposed
on two evaluations listing would include
at least 90 days hyperpigmentation and
apart within a lipodermatosclerosis
consecutive 12-month We would revise the
period (see current requirement that
4.00A3e), with both these skin changes
1 and 2: involve ``at least two-
thirds of the leg between
the ankle and knee or the
distal one-third of the
lower extremity between
the ankle and hip.''
Instead, we would require
extensive skin changes
involving at least two-
thirds of the leg below
the knee, to make the
requirement simpler to
understand and apply.
This revision is
consistent with IOM's
recommendation to require
skin changes below the
knee.
We would require the skin
changes under proposed
4.11A to be consistent
with CVI, and we would
document the skin changes
over a period of at least
90 days to ensure they
are chronic.
4.11B--Superficial varicosities, 4.11B--Two or more This requirement is more IOM. (2010), 161.
stasis dermatitis, and either episodes of conclusive than the
recurrent ulceration or ulceration that has current requirement of 3
persistent ulceration that has not healed following months of unsuccessful
not healed following at least 3 at least 6 months of prescribed treatment as
months of prescribed treatment. prescribed treatment. it demonstrates the
condition has persisted
despite treatment for a
longer period of time.
The CVI must be
unresponsive to
compression therapy,
because this therapy
usually enables people to
return to a good level of
functioning.
----------------------------------------------------------------------------------------------------------------
Proposed Listing 4.02--Chronic Heart Failure
We propose to revise the listing criteria for chronic heart failure
(CHF) in 4.02A and 4.02B and add new listing criteria 4.02C and 4.02D.
Proposed listing-level severity for CHF would be met when the person's
CHF satisfies the criteria in 4.02A and 4.02B. Listing-level severity
for CHF would also be met when the person's CHF satisfies either
proposed 4.02C or 4.02D.
Proposed criterion 4.02A1 requires an increased left ventricular
end diastolic dimension (LVEDD) equal to or greater than 7.0
centimeters (cm) instead of the current criterion of an LVEDD greater
than 6.0 cm, because an LVEDD less than 5.6 cm is normal. We followed
the IOM's recommendation in determining that an increased LVEDD of
greater than 6.0 cm but less than 7.0 cm indicates only a moderately
enlarged heart, and an increased LVEDD of at least 7.0 cm more clearly
establishes an enlarged heart with signs and symptoms indicating
listing-level heart failure and is comparable to an ejection fraction
(EF) of 30 percent or less.\25\
---------------------------------------------------------------------------
\25\ IOM. (2010), 88, 89.
---------------------------------------------------------------------------
In proposed 4.02A2, we would consider an elevated left atrial
volume index (LAVi) measurement. An LAVi measurement provides a precise
representation of increased left atrial pressure, making it a more
accurate indicator of heart failure than considering left atrium size
alone.\26\
---------------------------------------------------------------------------
\26\ Cacciapuoti, Fu., Scognamiglio, A., Paoli, V.D., Romano,
C., & Cacciapuoti, Fe. (2012). Left atrial volume index as indicator
of left ventricular diastolic dysfunction: Comparation between left
atrial volume index and tissue myocardial performance index. Journal
of Cardiovascular Ultrasound, 20(1), 25-29. doi:10.4250/
jcu.2012.20.1.25.
---------------------------------------------------------------------------
To establish listing-level severity for CHF, in addition to
satisfying the criteria in proposed 4.02A, a person's CHF must satisfy
the criteria in proposed 4.02B. The criteria in proposed 4.02B are
similar to the criteria in current 4.02B1 and 4.02B3, respectively,
with some important changes. For proposed 4.02B1a, we can use a
conclusion by a medical source that an exercise tolerance test (ETT)
presents a significant risk to a person; for example, the person's
cardiologist stating that an ETT would cause cardiac instability or
injury. For proposed 4.02B1b, we would require findings showing that a
person is very seriously limited in his or her ability to perform an
ETT, similar to current 4.02B3. Consistent with the IOM's
recommendations, proposed 4.02B2 would also include findings showing
the inability to perform on an ETT at 15 milliliters/kilograms/minute
(ml/kg/min) peak VO2 (oxygen consumption).\27\ Peak
VO2 at this level is comparable to the requirement in
current 4.02B3 for an inability to perform on an ETT at a workload
equivalent to 5 metabolic equivalents (METs) of task.
---------------------------------------------------------------------------
\27\ IOM. (2010), 85, 93.
---------------------------------------------------------------------------
We propose to remove current 4.02B2 ``three or more separate
episodes of acute congestive heart failure'' because we would evaluate
these episodes under proposed 4.02B3. As recommended by the IOM,
proposed 4.02B3 would evaluate exacerbations or complications
[[Page 38846]]
of CHF, requiring three hospitalizations within a consecutive 12-month
period and at least 30 days apart.\28\
---------------------------------------------------------------------------
\28\ IOM. (2010), 89, 91.
---------------------------------------------------------------------------
Additionally, proposed 4.02B2 would not include the requirement in
current 4.02B3b of frequent premature ventricular contractions (PVCs).
Frequent PVCs do not necessarily reflect an inability to perform an
ETT.29 30 31 The proposed listing also would no longer
include the criterion in current 4.02B3d requiring signs attributable
to inadequate cerebral perfusion, such as ataxic gait or mental
confusion. Such manifestations rarely occur during an ETT, even if the
person has very serious CHF.\32\
---------------------------------------------------------------------------
\29\ Cha, Y.-M., Lee, G.K., Klarich, K.W., & Grogan, M. (2012).
Premature ventricular contraction-induced cardiomyopathy: A
treatable condition. Circulation: Arrhythmia and Electrophysiology,
5(1), 229-236. doi:10.1161/CIRCEP.111.963348.
\30\ Dukes, J.W., Dewland, T.A., Vittinghoff, E., Mandyam, M.C.,
Heckbert, S.R., Siscovick, D.S., . . . Marcus, G.M. (2015).
Ventricular ectopy as a predictor of heart failure and death.
Journal of the American College of Cardiology, 66(2), 101-109.
doi:10.1016/j.jacc.2015.04.062.
\31\ IOM. (2010), 87.
\32\ IOM. (2010), 87, 88.
---------------------------------------------------------------------------
The IOM recommended a criterion for chronic heart failure in people
who are stable and receiving treatment but have an EF on a sustained
basis of 20 percent or less. An EF of only 20 percent means the heart's
pumping action is less than a third of normal, and critically affects a
person's ability to perform gainful activity.33 34 35 Most
individuals with disease this advanced have greater risk of mortality
and major functional limitations, such as shortness of breath or
fatigue, even during mild exertion.\36\ We propose 4.02C consistent
with the IOM's recommendation. We would require at least two EF
measurements equal to or less than 20 percent at least 90 days apart
within a consecutive 12-month period to document chronic disease and to
exclude heart failure resulting from reversible causes.
---------------------------------------------------------------------------
\33\ Desai, R.V., Guichard, J.L., Mujib, M., Ahmed, M.I.,
Feller, M.A., Fonarow, G.C., . . . Ahmed, A. (2013). Reduced right
ventricular ejection fraction and increased mortality in chronic
systolic heart failure patients receiving beta-blockers: Insights
from the BEST trial. International Journal of Cardiology, 163(1),
61-67. doi:10.1016/j.ijcard.2011.05.051.
\34\ IOM. (2010), 84, 89.
\35\ Runge, M.S., Patterson, C., Stouffer, G.A., & Netter, F.H.
(2010). Netter's Cardiology (2nd ed.). Philadelphia, PA: Saunders
Elsevier.
\36\ IOM. (2010), 78, 81, 82, 94.
---------------------------------------------------------------------------
Under proposed 4.02D1, we would include a new criterion for heart
failure treated with a mechanical circulatory support device (MCSD).
Implanted MCSDs, such as a left ventricle assistive device (LVAD) or a
right ventricle assistive device (RVAD), help the heart pump blood and
may be used as a ``bridge'' while a person waits for a heart
transplant. We currently use our medical equivalence rules \37\ to find
someone with heart failure and an implanted MCSD disabled under listing
4.09 (Heart transplantation). Adding this new criterion will ensure
that people with heart failure treated with MCSD are consistently
identified.
---------------------------------------------------------------------------
\37\ 20 CFR 404.1526 and 416.926.
---------------------------------------------------------------------------
People who require continuous intravenous administration of
inotropic medication (for example, milrinone) have very serious heart
failure, and the length of this treatment can be an accurate predictor
of impairment severity.38 39 Accordingly, proposed 4.02D2
would find people disabled if they require continuous intravenous
inotropic medications for 30 or more consecutive days.
---------------------------------------------------------------------------
\38\ Malotte, K., Saguros, A., & Groninger, H. (2018).
Continuous cardiac inotropes in patients with end-stage heart
failure: An evolving experience. Journal of Pain Symptom Management,
55(1), 159-163. doi:10.1016/j.jpainsymman.2017.09.026.
\39\ Bistola, V., Arfaras-Melainis, A., Polyzogopoulou, E.,
Ikonomidis, I., & Parissis, J. (2019). Inotropes in acute heart
failure: From guidelines to practical use: Therapeutic options and
clinical practice. Cardiac Failure Review, 5(3), 133-139.
doi:10.15420/cfr.2019.11.2.
---------------------------------------------------------------------------
Proposed Listing 4.04--Ischemic Heart Disease
As noted earlier in this preamble, we propose to use reports from
AMSs under proposed 4.04A to determine whether ECG findings are
positive for IHD. We would also rely on such reports to determine
whether systolic blood pressure measurements during ETTs are positive
for IHD. Based on our program experience and consultation with agency
medical experts, we expect that relying on these reports from AMSs will
ensure the accuracy of disability claims adjudication.
We would replace current 4.04A4 with proposed 4.04A. Proposed 4.04B
adds the requirement to consider imaging results derived from
pharmacologic stress testing. The new proposed requirement will provide
more specific findings than the current criterion, which generally
requires only ``documented ischemia.'' \40\ Because of these changes,
we would redesignate current 4.04B as proposed 4.04C and revise the
introductory text accordingly.
---------------------------------------------------------------------------
\40\ IOM. (2010), 120.
---------------------------------------------------------------------------
Proposed 4.04C (current 4.04B) would require three separate
ischemic episodes that result in unplanned hospitalizations within a
consecutive 12-month period (see 4.00A3e (What do the following terms
or phrases mean in these listings?), including episodes requiring
unplanned revascularization or treatment for myocardial infarction
(heart attack), unstable angina, or an irregular heartbeat. We would
evaluate unplanned hospitalizations under this section to ensure we are
only evaluating urgent ischemic episodes. Individuals who have ischemic
episodes that result in unplanned hospitalizations may need intensive
care, can have long hospital stays, may require multiple procedures,
and can be at high risk for post-discharge morbidity and
mortality.41 42 43 44 Many also have serious co-occurring
medical conditions (for example, heart failure, chronic kidney disease,
and chronic obstructive pulmonary disease).45 46 47
---------------------------------------------------------------------------
\41\ Hua, M., Gong, M.N., Brady, J., & Wunsch, H. (2015). Early
and late unplanned rehospitalizations for survivors of critical
illness. Critical Care Medicine, 43(2), 430-438. doi:10.1097/
CCM.0000000000000717.
\42\ Kim, Y., Gani, F., Canner, J.K., Margonis G.A., Makary,
M.A., Schneider, E.B., & Pawlik, T.M. (2016). Hospital readmission
after multiple major operative procedures among patients with
employer provided health insurance. Surgery, 160(1), 178-190.
doi:10.1016/j.surg.2016.01.025.
\43\ Reynolds, K., Butler, M.G., Kimes, T.M., Rosales, A.G.,
Chan, W., & Nichols, G.A. (2015). Relation of acute heart failure
hospital length of stay to subsequent readmission and all-cause
mortality. American Journal of Cardiology, 116(3), 400-405.
doi:10.1016/j.amjcard.2015.04.052.
\44\ Yu, P.-J., Cassiere, H.A., Fishbein, J., Esposito, R.A., &
Hartman, A.R. (2016). Outcomes of patients with prolonged intensive
care unit length of stay after cardiac surgery. Journal of
Cardiothoracic and Vascular Anesthesia, 30(6), 1550-1554.
doi:10.1053/j.jvca.2016.03.145.
\45\ Frigola-Capell, E., Comin-Colet, J., Davins-Miralles, J.,
Gich-Saladich, I., Wensing, M., & Verd[uacute]-Rotellar, J.M.
(2012). Trends and predictors of hospitalization, readmissions and
length of stay in ambulatory patients with heart failure. Revista
Clinica Espanola, 213(1), 1-7. doi:10.1016/j.rce.2012.10.006.
\46\ Nombela-Franco, L., del Trigo, M., Morrison-Polo, G.,
Veiga, G., Jimenez-Quevedo, P., Altisent, O.A.-J., . . .
Rod[eacute]s-Cabau, J. (2015). Incidence, causes, and predictors of
early (<30 days) and late unplanned hospital readmissions after
transcatheter aortic valve replacement. Journal of the American
College of Cardiology: Cardiovascular Interventions, 8(13), 1748-
1757. doi:10.1016/j.jcin.2015.07.022.
\47\ Versteeg, H., Hoogwegt, M.T., Hansen, T.B., Pedersen, S.S.,
Zwisler, A.-D., & Thygesen, L.C. (2013). Depression, not anxiety, is
independently associated with 5-year hospitalizations and mortality
in patients with ischemic heart disease. Journal of Psychosomatic
Research, 75(6), 518-525. doi:10.1016/j.jpsychores.2013.10.005.
---------------------------------------------------------------------------
We would redesignate current 4.04C as proposed 4.04D. We would
reorganize the criteria in current 4.04C and follow IOM's
recommendation to add new criteria to evaluate the severity of a
person's IHD regardless of whether he or she has had a timely ETT or
pharmacologic stress test or whether such tests are contraindicated.
ETTs and pharmacologic stress test are commonly performed for
diagnostic and prognostic purposes, and applicable to determine
[[Page 38847]]
functional capacity in persons with IHD.\48\
---------------------------------------------------------------------------
\48\ IOM. (2010), 70.
---------------------------------------------------------------------------
We would create proposed new 4.04D1 based upon blood flow in the
coronary arteries expressed as fractional flow reserve (FFR). Updated
medical science has shown FFR is a more objective and medically updated
measure of the severity of stenosis.\49\ A clinician may measure FFR in
a stenotic (obstructed) artery to determine whether it requires
revascularization. A normal (patent) artery has an FFR equal to 1.0. If
a stenotic artery has an FFR equal to or less than 0.80, and the artery
is amenable to revascularization, the clinician will use
revascularization to restore the vessel, because the obstructed artery
is causing significant ischemia. Accordingly, an FFR measurement less
than or equal to 0.80 in the proximal or mid segment of an artery that
is not amenable to revascularization is consistent with the
requirements of proposed 4.04D1.50 51 52
---------------------------------------------------------------------------
\49\ Shlofmitz, E., and Jeremias, A. (2017). FFR in 2017:
Current Status in PCI Management--American College of Cardiology.
Available online at: https://www.acc.org/latest-in-cardiology/articles/2017/05/25/08/34/ffr-in-2017-current-status-in-pci-management (accessed September 17, 2021).
\50\ Fearon, W.F. (2014). Percutaneous coronary intervention
should be guided by fractional flow reserve measurement.
Circulation, 129(18), 1860-1870. doi:10.1161/
CIRCULATIONAHA.113.004300.
\51\ Fearon, W.F., De Bruyne, B., & Pijlis, N.H. (2016).
Fractional flow reserve in acute coronary syndromes. Journal of the
American College of Cardiology, 68(11), 1192-1194. doi:10.1016/
j.jacc.2016.07.713.
\52\ Tebaldi, M., Biscaglia, S., Pecoraro, A., Fineschi, M., &
Campo, G. (2016). Fractional flow reserve implementation in daily
clinical practice: A European survey. International Journal of
Cardiology, 207, 206-207. doi:10.1016/j.ijcard.2016.01.097.
---------------------------------------------------------------------------
In proposed 4.04D2 we would evaluate IHD by taking into
consideration a person's history of coronary artery bypass graft
surgery. In proposed 4.04D3, we would evaluate IHD by taking into
consideration that a person has a decreased EF (i.e., EF of less than
50 percent while medically stable and on a regimen of prescribed
treatment).
In both proposed 4.04D2 and 4.04D3, we would require the person to
have symptoms of myocardial ischemia, as described in current 4.00E3
through 4.00E7 and be on a regimen of prescribed treatment. Proposed
4.04D2 and 4.04D3 would include the criteria in current 4.04C1a, b, and
d with minor editorial changes. Proposed 4.04D2 includes criteria in
current 4.04C1e. We would not include the criteria in current 4.04C1c
in the proposed listings 4.04D2 and 4.04D3 because based on our program
experience, we determined that these criteria do not consistently
correlate with listing-level severity.
We would remove current 4.04C2 with its requirement that a person's
IHD result in very serious limitations in his or her ability to
independently initiate, sustain, or complete activities of daily
living. The criteria in proposed listings 4.04D1, 4.04D2, and 4.04D3
alone provide a description of listing-level IHD.
We are proposing new 4.04E to evaluate exacerbations or
complications of IHD requiring three hospitalizations within a
consecutive 12-month period and at least 30 days apart. These
hospitalizations may be planned or unplanned. The hospitalizations
required under 4.04E differ from those required in proposed 4.04C,
which requires that hospitalizations be unplanned.
Proposed Listing 4.05--Recurrent Arrhythmias
We propose to reorganize the basic structure and presentation of
current 4.05 (Recurrent arrhythmias) to improve its clarity and ease of
reference. We would breakdown the current criteria into two parts:
recurrent episodes of syncope (or near syncope) and findings and
documentation by a medically acceptable test, to demonstrate that both
parts must be satisfied to document listing-level severity. We would
also remove ``uncontrolled'' as a descriptor for recurrent episodes of
cardiac syncope because, inherently, these episodes are uncontrolled if
they recur while a person is on a regimen of prescribed treatment.
Because we would remove ``uncontrolled'' as a descriptor in the
listing, we would also remove the definition for this term from current
4.00A3 in the introductory text. Symptoms associated with arrhythmia
include: anxiety, chest pain, fatigue, sweating, near-syncope, and
fainting (syncope). Syncope and near-syncope are two of the more
serious symptoms; individuals with arrhythmias and recurrent episodes
of syncope have a higher risk of mortality and sudden cardiac death.
Furthermore, syncope and near-syncope are more quantifiable and
objective than other symptoms like anxiety and fatigue. For these
reasons, recurrent episodes of syncope and near syncope continue to be
an appropriate indicator of listing-level severity for individuals with
recurrent arrhythmias.\53\
---------------------------------------------------------------------------
\53\ Koene, R.J., Adkisson, W.O., & Benditt, D.G. (2017).
Syncope and the risk of sudden cardiac death: Evaluation,
management, and prevention. Journal of arrhythmia, 33(6), 533-544.
https://doi.org/10.1016/j.joa.2017.07.005.
---------------------------------------------------------------------------
Proposed Listing 4.06--Congenital Heart Disease
For the reason discussed below, we propose to remove the
parenthetical reference to ``cyanotic or acyanotic'' congenital heart
disease from the heading in current 4.06 in order to focus on
hypoxemia.
Accordingly, we propose to revise current 4.06A to require
``hypoxemia'' rather than ``cyanosis or acyanosis.'' ``Hypoxemia''
reflects abnormalities in the blood, such as an increased hematocrit
level or a low blood oxygen level, which are detected through
laboratory analysis or pulse oximetry. On the other hand, the term
``cyanosis'' refers to skin discoloration observed during a physical
examination. Cyanosis is a more subjective assessment subject to
misinterpretation due to by many factors, including skin complexion.
Thus, the term ``hypoxemia'' relates more to the laboratory and pulse
oximetry findings than the term ``cyanosis.'' \54\
---------------------------------------------------------------------------
\54\ Stout, K.K. (2019).
---------------------------------------------------------------------------
To establish listing-level severity for individuals with congenital
heart disease, IOM recommended documentation of chronic and persistent
hypoxemia. Therefore to demonstrate the chronic and persistent nature,
in proposed 4.06A1, we would require two hematocrit measurements
instead of the current listing's single measurement. Two measurements,
at least 90 days apart within a consecutive 12-month period will help
ensure the person's hematocrit level is associated with chronic
hypoxemia and not the result of a reversible condition, such as
dehydration.\55\ The proposed requirement that the two measurements be
90 days apart is consistent with the time period requirement used in
our other body system listings, and consistent with instructions
providers receive for scheduling patients \56\ and established check-up
intervals for adults with congenital heart disease.\57\
[[Page 38848]]
Furthermore, requiring two measurements at least 90 days apart is
consistent with the current (and proposed) childhood congenital heart
disease criterion (104.06A1) and will assist with documenting duration
and establishing that the persistent nature of the person's
condition.\58\
---------------------------------------------------------------------------
\55\ Stack, S.W. . . ., & Berger, S.A. (2009). The effects of
high hematocrit arterial flow--A phenomenological study of health
risk implications. Chemical Engineering Science, 64(22), 4701-4706.
doi:10/1016/j.ces.2009.07.017.
\56\ Bavafa, H., Savin, S., & Terwiesch, C. (2019). Redesigning
Primary Care Delivery: Customized Office Revisit Intervals and E-
Visits. https://dx.doi.org/10.21con39/ssrn.2363685.
Paper referenced by Bavafa: Schectman, G., G. Barnas, P. Laud,
L. Cantwell, M. Horton, E.J. Zarling. 2005. Prolonging the return
visit interval in primary care. The American Journal of Medicine,
118(4) 393-39.
\57\ According to the University of Washington's Adult
Congenital Heart Disease Clinic's Information for Patients and
Families (2016), most people with congenital heart disease require
regular check-ups with their cardiologist ``at intervals ranging
from every several months to every several years.'' Accessed May 4,
2022, from HeartInstitute_AdultCongenitalHeartDiseaseClinic.pdf
(uwmedicine.org).
People with listing-level congenital heart disease are expected
to require more frequent checkups than those who are asymptomatic or
have less severe disease.
\58\ See 20 CFR 404.1505(a) and 416.905(a). The law defines
disability as the inability to do any substantial gainful activity
by reason of any medically determinable physical or mental
impairment which can be expected to result in death or which has
lasted or can be expected to last for a continuous period of not
less than 12 months.
---------------------------------------------------------------------------
We would include the medical abbreviation
``SaO2'' in 4.06A2. This abbreviation frequently
appears in the medical evidence to indicate arterial oxygen
(O2) saturation determined by arterial blood gas testing. We
would also include the medical abbreviation
``PaO2'' (partial pressure of O2),
because medical reports may use it interchangeably with the
abbreviation ``PO2'' that we use in current 4.06A for
arterial partial pressure of oxygen. Additionally, we would express
PaO2 and PO2 in millimeters of mercury
(mmHg) instead of Torr units to make the listing consistent with
current medical practice and terminology.\59\
---------------------------------------------------------------------------
\59\ Castro D, Patil SM, Keenaghan M. Arterial Blood Gas. 2021
Jan 27. In: StatPearls [internet]. Treasure Island (FL): StatPearls
Publishing; 2021 Jan-. PMID: 30725604.
---------------------------------------------------------------------------
In proposed 4.06A3, we would add a criterion for
SpO2 (percentage of oxygen saturation of blood
hemoglobin) measured by pulse oximetry, including measurements taken
while the person is at rest or while doing a six-minute walk test
(6MWT). Pulse oximetry measurements are a non-invasive alternative to
invasive testing. A person's medical evidence often provides
SpO2 findings, and SpO2
measured by pulse oximetry reflects an advance in medical technology
that provides another way to establish listing-level
severity.60 61 62
---------------------------------------------------------------------------
\60\ IOM. (2010), 178.
\61\ Oster, M. E. . . ., & Kochilas, L.K. (2016). Screening for
critical congenital heart disease. Clinics in Perinatology, 43(1),
73-80. doi:10.1016/j.clp.2015.11.005.
\62\ Mechem, C.C. (2014). Pulse oximetry. In P.E. Parsons (Ed.),
UpToDate (Jan. 2014). Retrieved from https://www.uptodate.com/contents/pulse-oximetry.
---------------------------------------------------------------------------
Proposed 4.06A3 would require three SpO2
measurements 30 days apart within a consecutive 12-month period showing
hypoxemia. We explain in the introductory text of proposed 4.00H4c that
these measurements must be documented by a medical source using methods
consistent with the prevailing state of medical knowledge and clinical
practice, and also must be consistent with the other evidence in the
person's case record. We would require an SpO2 of
87 percent or less because this finding is comparable in severity to an
SaO2 of less than 90 percent in current
4.06A2.\63\ By requiring several measurements at least 30 days apart,
we ensure that the required findings span a period of at least 90 days.
Similar to the requirement for repeated hematocrit measurements under
4.06A1, this will document that the condition is chronic and
persistent, and the measurements are not related to a reversible
condition or an inaccurate reading.
---------------------------------------------------------------------------
\63\ IOM. (2010), 178.
---------------------------------------------------------------------------
In proposed 4.06B, we would include an additional option of taking
an SaO2 measurement for determining the level of
hypoxemia during exertion. This change would provide an additional way
of evaluating hypoxemia. Similarly, we would include an oxygen uptake
measurement as another option.
We are proposing several changes to current 4.06C. We would use the
term ``pulmonary hypertension'' to describe the impairment instead of
the term ``pulmonary vascular obstructive disease.'' ``Pulmonary
hypertension'' is the term more commonly used by clinicians and,
therefore, the most likely to appear in the medical evidence.\64\ We
are also proposing to delete ``secondary'' from the listing's heading,
because pulmonary hypertension can be disabling regardless of whether
it is a ``primary'' or ``secondary'' condition.
---------------------------------------------------------------------------
\64\ 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 exact term ``pulmonary hypertension'' came back with more
than 800 results. A search for the exact term ``pulmonary vascular
obstructive disease'' came back with zero results. The search was
conducted on September 8, 2021.
---------------------------------------------------------------------------
We would include medical findings in proposed 4.06C that are
expressed in millimeters of mercury (mmHg). Findings of pulmonary
artery pressure are expressed in mmHg more often than they are
expressed as a percentage of ``systemic arterial systolic pressure,''
as in current 4.06C. Pulmonary hypertension may be reported in the
medical evidence as either pulmonary artery pressure or mean pulmonary
artery pressure, so we would include both types of findings in the
proposed listing.
We would add a new criterion--proposed 4.06D--to evaluate adults
with ``single ventricle,'' which is also known as ``single ventricle
physiology'' or ``functional single ventricle.'' Children born with
single ventricle have a severe, medically determinable impairment (MDI)
that will usually need to be corrected by staged surgery called
``Fontan procedures.'' \65\ These procedures enable an increasing
percentage of affected children to survive into adulthood. As adults,
they have significantly reduced functional capacities that steadily
decline. We would find adults disabled under proposed 4.06D if
objective medical evidence shows the person has single ventricle,
regardless of whether or not they had Fontan or other surgical
procedures.66 67 We provide information in the introductory
text in proposed 4.00H3 (What is single ventricle?) about single
ventricle and these surgical procedures.
---------------------------------------------------------------------------
\65\ People with single ventricle will generally undergo staged
reconstructive ``Fontan procedures,'' ultimately resulting in a
``Fontan circulation.'' Fontan circulation describes the 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.
\66\ Cohen, S., & Marelli, A. (2016). Evolving heart
transplantation across the lifespan: A growing population of adults
with congenital heart disease. Archives of Cardiovascular Disease,
109(10), 511-513. doi:10.1016/j.acvd.2016.05.001.
\67\ IOM. (2010), 169, 178.
---------------------------------------------------------------------------
Proposed Listing 4.07--Aortic Valvular Disease
We currently evaluate aortic valvular disease under other
cardiovascular disorders listings, which include requirements for ETT
or repeated hospitalization. According to the IOM report, due to the
risk associated with exercise testing for individuals with symptomatic
aortic stenosis, ETT is not advised.\68\ Furthermore, very serious
symptomatic aortic stenosis is ``universally fatal'' if left untreated
and there are few effective, long-term medical therapies for
individuals with this level of disease.\69\ Therefore, we followed IOM
recommendations to provide evaluation criteria for aortic valvular
disease and propose to add new listing 4.07 to evaluate aortic valvular
disease. The medical community considers an aortic valve area equal to
or less than 1.0 cm\2\ indicative of advanced stenotic disease
associated with significant dyspnea, fatigue, angina, and other serious
---------------------------------------------------------------------------
\68\ IOM. (2010), 195.
\69\ IOM. (2010), 194, 195.
---------------------------------------------------------------------------
[[Page 38849]]
symptoms.70 71 72 73 Proposed 4.07 would require appropriate
testing that documents the aortic valve area.
---------------------------------------------------------------------------
\70\ Berthelot-Richer, M., Pibarot, P., Capoulade, R., Dumesnil,
J.G., Dahou, A., Thebault, C., . . . Clavel, M.-A. (2016).
Discordant grading of aortic stenosis severity: Echocardiographic
predictors of survival benefit associated with aortic valve
replacement. Journal of the American College of Cardiology:
Cardiovascular Imaging, 9(7), 797-805. doi:10.1016/
j.jcmg.2015.09.026.
\71\ IOM. (2010), 195.
\72\ Nombela-Franco, L. (2015).
\73\ Ziberszac, R., Gabriel, H., Schemper, M., Laufer, G.,
Maurer, G., & Rosenhek, R. (2017). Asymptomatic severe aortic
stenosis in the elderly. Journal of the American College of
Cardiology: Cardiovascular Imaging, 10(1), 43-50. doi:10.1016/
j.jcmg.2016.05.015.
---------------------------------------------------------------------------
Proposed Listing 4.08--Cardiomyopathy
Consistent with IOM recommendations, we would add 4.08 to evaluate
cardiomyopathies, such as hypertrophic cardiomyopathy (HCM). We
currently evaluate cardiomyopathy under 4.02 (Chronic heart failure),
4.04 (Ischemic heart disease), 4.05 (Recurrent arrhythmias), or 11.04
(Vascular insult to the brain), depending on its effects. Depending on
the underlying cause of the person's cardiomyopathy or its effects, we
may continue to evaluate cardiomyopathy under 4.02, 4.04, 4.05, and
11.04. We created this new cardiomyopathy listing to specifically
address HCM, endomyocardial fibrosis, and cardiac amyloidosis AL type,
which are more serious types of cardiomyopathy.\74\
---------------------------------------------------------------------------
\74\ SSA has designated endomyocardial fibrosis and cardiac
amyloidosis AL type as Compassionate Allowance (CAL) conditions. See
Compassionate Allowances website Home Page (ssa.gov).
---------------------------------------------------------------------------
HCM with severe left ventricular or septal wall thickness can cause
serious problems, including chest pain, dyspnea, syncope, and
arrhythmias.\75\ As recommended by IOM, we would evaluate HCM under
proposed 4.08A by requiring the heart to have a left ventricular or
septal wall thickness equal to or greater than 20 millimeters. Proposed
4.08 also requires the individual to be seriously limited in the
ability to perform an ETT, or a medical source has concluded that the
performance of an ETT would present a significant risk.
---------------------------------------------------------------------------
\75\ IOM. (2010), 80.
---------------------------------------------------------------------------
Proposed 4.08B would evaluate endomyocardial fibrosis, a form of
cardiomyopathy with a generally poor prognosis despite treatment. Under
proposed 4.08B, we would require endomyocardial fibrosis resulting in a
loss of heart chamber volume, atrial dilatation, and mitral or
tricuspid valve regurgitation.
Proposed 4.08C would evaluate cardiac amyloidosis AL (light-chain)
type, another form of cardiomyopathy with a poor prognosis. We would
need objective medical evidence, such as biopsy findings,
echocardiogram, cardiac MRI, and PET scan to establish listing-level
severity.
Proposed 4.08D would evaluate exacerbations or complications of
cardiomyopathy, requiring three hospitalizations within a consecutive
12-month period and at least 30 days apart.
Proposed Listing 4.09--Heart Transplantation
We are proposing editorial changes in the heading and text of
current 4.09, which would not be substantive but would clarify the
guidance. We have changed from ``1 year following surgery'' to ``1 year
from the date of the transplant'' consistent with transplantation
listings in other body systems such as 6.04 (Chronic kidney disease)
and 7.17 (Hematological disorders treated by bone marrow or stem cell
transplantation).
Proposed Listing 4.10--Dissecting Aneurysm of the Aorta or Major
Branches
We propose to revise the heading for listing 4.10 to specify that
we evaluate only ``dissecting'' aneurysms under the listing consistent
with IOM recommendations.\76\
---------------------------------------------------------------------------
\76\ IOM. (2010), 217.
---------------------------------------------------------------------------
Proposed Listing 4.11--Chronic Venous Insufficiency
We propose to revise the heading in current listing 4.11 by
replacing the outdated term ``incompetency'' with the term ``reflux''--
the term the medical community currently uses to describe decrease
blood flow and pooling of blood in the veins.\77\ We would delete the
word ``deep'' in the heading so that the listing covers reflux or
obstruction associated with superficial and perforating veins. Reflux
or obstruction in these veins may result in the required level of
CVI.\78\ Additionally, as recommended by IOM, we would require
confirmation of CVI by duplex ultrasound or other appropriate
diagnostic technique. The medical community considers the use of duplex
ultrasound to be the best method for detecting reflux or
obstruction.\79\
---------------------------------------------------------------------------
\77\ IOM. (2010), 160.
\78\ IOM. (2010), 161.
\79\ Id.
---------------------------------------------------------------------------
In proposed 4.11A, we would adopt IOM recommendations and broaden
the listing criteria we apply to trophic changes (changes resulting
from interruption of nerve supply) of the skin. For example, in
addition to brawny edema, trophic changes evaluated under the proposed
listing would include hyperpigmentation and lipodermatosclerosis.
We would revise the current requirement that these skin changes
involve ``at least two-thirds of the leg between the ankle and knee or
the distal one-third of the lower extremity between the ankle and
hip.'' Instead, we would require extensive skin changes involving at
least two-thirds of the leg below the knee, to make the requirement
simpler to understand and apply. This revision is consistent with IOM's
recommendation to require skin changes below the knee.\80\
---------------------------------------------------------------------------
\80\ IOM. (2010), 157-161.
---------------------------------------------------------------------------
We would require the skin changes under proposed 4.11A to be
consistent with CVI, and we would document the skin changes over a
period of at least 90 days to ensure they are chronic. Additionally,
the CVI must be unresponsive to compression therapy, because this
therapy usually enables people to return to a good level of
functioning.\81\
---------------------------------------------------------------------------
\81\ IOM. (2010), 159.
---------------------------------------------------------------------------
In proposed 4.11B, we would remove findings in the current listing
that no longer demonstrate required severity. For example, we would
remove superficial varicosities, which indicate venous disease but not
necessarily CVI. We would follow IOM's recommendation and also remove
stasis dermatitis, because it is ``a generic term referring to the
trophic changes,'' and it is unreliable because it may be a sign of
other unrelated conditions including aging.\82\
---------------------------------------------------------------------------
\82\ IOM. (2010), 161.
---------------------------------------------------------------------------
Proposed 4.11B would require recurrent or persistent skin
ulceration that has not healed after 6 or more months of prescribed
treatment. In regard to documenting duration and severity of CVI, this
requirement is more conclusive than the current requirement of 3 months
of unsuccessful prescribed treatment as it demonstrates the condition
has persisted despite treatment for a longer period of time.\83\
---------------------------------------------------------------------------
\83\ IOM. (2010), 161.
---------------------------------------------------------------------------
Proposed Listing 4.12--Peripheral Arterial Disease
We propose to revise the heading of the current listing to evaluate
peripheral arterial disease (PAD) while the person is on a regimen of
prescribed treatment. PAD often improves with angioplasty, supervised
physical rehabilitation, and other prescribed therapies.\84\
---------------------------------------------------------------------------
\84\ Poredo[scaron] P, Jezovnik M, Kalodiki E. Medical
management of patients with peripheral arterial disease. Int Angiol.
2015 Feb;34(1):75-93. Epub 2014 Jun 11. PMID: 24916346.
---------------------------------------------------------------------------
[[Page 38850]]
We would add leg pain in the heading as a serious and potentially
debilitating symptom of PAD. People who have PAD with intermittent leg
pain may be impaired to a similar extent as a person with PAD with
intermittent claudication.\85\
---------------------------------------------------------------------------
\85\ IOM. (2010), 151.
---------------------------------------------------------------------------
Consistent with IOM recommendations, we would require a person's
intermittent leg pain or claudication to interfere with his or her
mobility. This proposed change clarifies our original intent in the
listing, which is to tie PAD to functioning. Finally, we would replace
the term ``appropriate medically acceptable imaging'' in the listing
heading with ``appropriate test(s)'' (4.00G6--Are there any other
studies that are helpful in evaluating PAD?) to acknowledge that non-
imaging procedures such as physical examination and blood tests may
also help detect PAD.\86\
---------------------------------------------------------------------------
\86\ Id.
---------------------------------------------------------------------------
Proposed Listing 4.16--Cardiac Allograft Vasculopathy
We propose to add new listing 4.16 to evaluate a person who
received a heart transplant (allograft) and subsequently developed
cardiac allograft vasculopathy (CAV). Currently, we evaluate CAV
through medical equivalence to listing 4.09 (Heart transplant). CAV
results in stenosis of the heart's blood vessels that may progress
quickly and cause significant heart dysfunction. CAV with moderate
stenosis, as defined in the medical literature,\87\ may also result in
a listing-level impairment, depending on the extent and seriousness of
dysfunction. To establish the required level of CAV, we would require a
cardiac index (cardiac output) of less than 2 liters/minute/meter
square (L/min/m\2\), an ejection fraction equal to or less than 45
percent, right atrial pressure greater than 12 mmHg, or pulmonary
capillary wedge pressure greater than 15 mmHg. Individuals who have any
of these findings have a poor prognosis and are limited in their
activities and ability to work.88 89 90 91
---------------------------------------------------------------------------
\87\ The International Society of Heart and Lung
Transplantation's grading classification defines mild cardiac
allograft vasculopathy (CAV1) as having left main artery
stenosis of less than 50 percent, primary vessel stenosis greater
than 70 percent (including the right coronary artery), or any branch
stenosis greater than 70 percent (see Mehra, M.R., Crespo-Leiro,
M.G., Dipchand, A., Ensminger, S.M., Hiemann, N.E., Kobashigawa,
J.A., . . . Uber, P.A. (2010). International Society of Heart and
Lung Transplantation working formulation of a standardized
nomenclature for cardiac allograft vasculopathy--2010. Journal of
Heart and Lung Transplantation, 29(7), 717-727. doi:10.1016/
j.healun.2010.05.017).
\88\ Kindel, S.J., & Pahl, E. (2011). Cardiac allograft
vasculopathy in children--treatment challenges. Progress in
Pediatric Cardiology, 32(1), 37-42. doi:10.1016/
j.ppedcard.2011.06.008.
\89\ Kobashingawa, J.A. (2015). The changing face of first-year
intravascular ultrasonography in heart transplantation. Journal of
the American College of Cardiology: Heart Failure, 3(12), 954-955.
doi:10.1016/j.jchf.2015.09.004.
\90\ Mehra, M.R. (2010).
\91\ Okada, K., Kitahara, H., Yang, H., Tanaka, S., Kobayashi,
Y., Kimura, T., . . . Fearon, W.F. (2015). Paradoxical vessel
remodeling of the proximal segment of the left anterior descending
artery predicts long-term mortality after heart transplantation.
Journal of the American College of Cardiology: Heart Failure, 3(12),
945-952. doi:10.1016/j.jchf.2015.07.013.
---------------------------------------------------------------------------
Other Proposed Changes
As mentioned, we are proposing new criteria to evaluate
exacerbations or complications of several categories of cardiovascular
disorders. These new criteria include proposed 4.02B3 for evaluating
chronic heart failure, proposed 4.04E for evaluating ischemic heart
disease, proposed 4.06E for evaluating congenital heart disease, and
proposed 4.08D for evaluating cardiomyopathies. Consistent with IOM
recommendations, we are proposing these new criteria for evaluating
chronic heart failure and cardiomyopathies.\92\ In addition, we are
proposing these new criteria for evaluating ischemic heart disease
(4.04) and congenital heart disease (4.06). Our adjudicative experience
shows that these cardiovascular disorders are prone to exacerbations
and serious complications. These proposed criteria would require
exacerbations or complications causing a person to be hospitalized
three or more times within a consecutive 12-month period.\93\ An
impairment resulting in exacerbations or complications that require
this many hospitalizations in 12 months will prevent a person from
engaging in any gainful activity.94 95 96 97 98 99 100 We
would require these hospitalizations to be at least 30 days apart and
to last at least 48 hours, including hours in a hospital emergency
department immediately before the hospitalization, to ensure we are
evaluating separate listing-level episodes of exacerbations or
complications. Our proposal to require that each hospitalization last
at least 48 hours is generally consistent with data showing that the
average length of hospital stays for serious cardiac conditions like
primary heart failure and adult congenital heart disease is at least 48
hours.101 102
---------------------------------------------------------------------------
\92\ IOM. (2010), 89, 172.
\93\ Id.
\94\ Hua, M. (2015).
\95\ IOM. (2010), 30, 90, 196.
\96\ Kim, Y. (2016).
\97\ Kyriakou, M., & Kiff, P.F. (2016). Prognosis of the
comorbid heart failure and anemia: A systematic review and meta-
analysis. Clinical Trials and Regulatory Science in Cardiology, 16,
12-21. doi:10.1016/j.ctrsc.2016.01.008.
\98\ Reynolds, K. (2015).
\99\ Versteeg, H. (2013).
\100\ Yu, P.-J. (2016).
\101\ Jackson SL, Tong X, King RJ, et al. National Burden of
Heart Failure Events in the United States, 2006 to 2014.
Circulation. Heart Failure. 2018 Dec;11(12):e004873. DOI: 10.1161/
circheartfailure.117.004873. PMID: 30562099; PMCID: PMC6424109.
\102\ Cedars, A., Benjamin, L., Burns, S.V., Novak, E., & Amin,
A. (2017). Clinical predictors of length of stay in adults with
congenital heart disease. Heart (British Cardiac Society), 103(16),
1258-1263. https://doi.org/10.1136/heartjnl-2016-310841.
---------------------------------------------------------------------------
Another revision we are proposing would affect chronic venous
insufficiency evaluated under 4.11. Under our proposed changes, we
would follow IOM's recommendations and require documentation that
certain manifestations of chronic venous insufficiency (for example,
trophic changes of the skin) occurred at least twice within a
consecutive 12-month period, instead of only once under the current
listings.\103\ This change is based on the IOM recommendation that two
occurrences per year more accurately and consistently demonstrates
listing-level severity. We would also require documentation that these
manifestations occurred at least 90 days apart. These requirements
ensure we are appropriately documenting the chronicity and persistence
of these conditions and evaluating people who have very serious chronic
conditions.
---------------------------------------------------------------------------
\103\ IOM. (2010), 161.
---------------------------------------------------------------------------
Proposed Changes to the Childhood Cardiovascular Disorders Introductory
Text
Proposed 104.00--Introductory Text to the Childhood Cardiovascular
Disorders Listings
We repeat much of the introductory text of proposed 4.00 in the
introductory text of proposed 104.00, because the same basic criteria
for evaluating cardiovascular disorders apply to both adults and
children. Because we have already described these proposed criteria
above, the following discussion describes only those criteria that are
unique to children or that require further explanation in how they will
be applied to children.
The following table shows the heading of the current and proposed
sections of the childhood introductory text for cardiovascular
disorders:
[[Page 38851]]
------------------------------------------------------------------------
Proposed sections of the
Current sections of the childhood childhood introductory text and
introductory text and listings for listings for cardiovascular
cardiovascular system disorders
------------------------------------------------------------------------
104.00 Cardiovascular System........... 104.00 Cardiovascular
Disorders.
A. General............................. A. How do we define
cardiovascular disorder and
cardiovascular terms?
B. Documenting Cardiovascular B. What documentation do we
Impairment. need to evaluate
cardiovascular disorders?
C. Evaluating Chronic Heart Failure.... C. How do we evaluate chronic
heart failure?
D. Evaluating Congenital Heart Disease. D. How do we evaluate
congenital heart disease?
E. Evaluating Arrhythmias.............. E. How do we evaluate
arrhythmias?
F. Evaluating Other Cardiovascular F. How do we evaluate other
Impairments. cardiovascular disorders?
G. Other Evaluation Issues............. G. How do we evaluate issues
that affect the cardiovascular
system?
H. How do we evaluate
cardiovascular disorders that
do not meet one of these
listings?
------------------------------------------------------------------------
Proposed 104.00C--How do we evaluate chronic heart failure?
We are proposing changes in current 104.00C consistent with changes
we are proposing in the adult listings for chronic heart failure. We
would extensively revise current 104.00C2 by removing specific findings
for documenting cardiomegaly. These findings are often not provided in
a child's case record and, therefore, have presented difficulty in
adjudication. Proposed 104.00C2a would describe the types of imaging
provided in a child's case record for documenting cardiomegaly. We
explain at 104.00C2b(iii) that signs of congestion 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. We have
added 104.00C4 to explain how we propose to evaluate chronic heart
failure treated with a mechanical circulatory support device under
proposed 104.02D (Chronic heart failure).
Proposed 104.00D--How do we evaluate congenital heart disease?
We plan to significantly expand the information in current 104.00D.
In proposed 104.00D2 (How do we evaluate conditions associated with
congenital heart disease?), we would explain how we evaluate conditions
associated with congenital heart disease. Proposed 104.00D2 includes
additional means of measuring oxygen saturation in 104.06 (Congenital
heart disease), because these measurements are readily found in the
medical evidence. We are proposing new 104.00D3 (What is Eisenmenger
syndrome?) to explain Eisenmenger syndrome in children, and we propose
new 104.00D4 (What is a single ventricle?) to include a definition for
the term ``single ventricle.''
Proposed 104.00F--How do we evaluate other cardiovascular disorders?
We propose revisions to 104.00F6 (How will we evaluate chronic
rheumatic fever or rheumatic heart disease?) consistent with the
removal of current listing 104.13, rheumatic heart disease. These
revisions would explain that we evaluate rheumatic heart disease under
104.02 (Chronic heart failure) or 104.05 (Recurrent arrhythmias). We
propose adding 104.00F11 (What is cardiac allograft vasculopathy and
how do we evaluate it?) consistent with proposed 104.16 (Cardiac
allograft vasculopathy).
Proposed 104.00G--How do we evaluate issues that affect the
cardiovascular system?
We propose revisions to 104.00G consistent with those proposed to
the adult listings. We propose to revise 104.00G1 (How do we consider
the effects of obesity when we evaluate your cardiovascular disorder?)
to simplify and refocus our discussion of how we consider the effects
of obesity more specifically on cardiovascular disorders. We propose
adding 104.00G3 (How do we consider hospitalizations?), consistent with
new 104.02E (Chronic heart failure) and 104.06E (Congenital heart
disease). We propose to redesignate current 104.00G3 (How do we
evaluate impairments that do not meet one of the cardiovascular
listings?) as 104.00H (How do we evaluate cardiovascular disorders that
do not meet one of these listings?).
Proposed Changes to the Childhood Cardiovascular Disorders Listings
We are proposing some changes to the childhood listings that
correspond with changes we are proposing to the adult listings. Other
changes are specific to how we evaluate cardiovascular disorders in
children. The reasons provided above for changing or removing current
criteria for adults also apply to the criteria for children. Because we
have already described these proposed criteria above, the following
discussion describes only those criteria that are unique to children or
that require further explanation in how we will specifically apply them
to children. Additionally, the numbering of the childhood listings
would conform to that of the adult listings.
The following table shows the heading of the current and proposed
sections of the childhood listings for cardiovascular disorders:
Childhood Cardiovascular Disorders Listings
------------------------------------------------------------------------
Current Proposed
------------------------------------------------------------------------
104.02 Chronic heart failure........... 104.02 Chronic heart failure.
104.05 Recurrent arrhythmias........... 104.03 [Reserved].
104.06 Congenital heart disease........ 104.04 [Reserved].
104.09 Heart transplant................ 104.05 Recurrent arrhythmias.
104.13 Rheumatic heart disease......... 104.06 Congenital heart
disease.
104.07 [Reserved].
104.08 [Reserved].
104.09 Heart transplantation.
104.10 [Reserved].
104.11 [Reserved].
104.12 [Reserved].
[[Page 38852]]
104.13 [Reserved].
104.14 [Reserved].
104.15 [Reserved].
104.16 Cardiac allograft
vasculopathy.
------------------------------------------------------------------------
The following table shows our proposed changes to the childhood
cardiovascular disorders listings criteria that involve changes to
healthcare utilization and condition/episode requirements, the
rationale for each change, and supporting resource. Following this
table, we discuss all of our proposed changes to the childhood
cardiovascular disorders listings in more detail.
Childhood Cardiovascular Disorders Listings Criteria--Changes in Healthcare Utilization and Condition/Episode
Requirements
----------------------------------------------------------------------------------------------------------------
Proposed listing
Current listing criterion criterion Rationale Resources
----------------------------------------------------------------------------------------------------------------
Listing 104.02 Chronic heart failure
----------------------------------------------------------------------------------------------------------------
104.02A Persistent tachycardia at A. Persistent We would clarify that IOM. (2010), 171,173,
rest (see Table I); tachycardia at rest to satisfy 104.02A, we 176.
measured at least would require two or
twice within a more tachycardia
consecutive 12-month measurements in a
period and at least 90 consecutive 12-month
days apart documented period. Our intent is
by apical heart rate to ensure that the
greater than or equal child has persistent
to the value in Table tachycardia despite
I. treatment. We would
also require that
readings of
tachycardia occur at
least 90 days apart to
further document
chronic disease.
104.02B.............................. B. Persistent tachypnea To satisfy 104.02B, we IOM. (2010), 171,173,
Persistent tachypnea at rest (see at rest measured at would require two or 176.
Table II) or markedly decreased least twice within a more tachypnea
exercise tolerance (see 104.00C2b);. consecutive12-month measurements in a
period and at least 90 consecutive 12-month
days apart documented period. Our intent is
by respiratory rate to ensure that the
greater than or equal child has persistent
to the value in Table tachypnea, despite
II or markedly treatment. We would
decreased exercise also require that
tolerance (see readings of tachypnea
104.00C2b). occur at least 90 days
apart to further
document chronic
disease.
----------------------------------------------------------------------------------------------------------------
Listing 104.06 Congenital heart disease
----------------------------------------------------------------------------------------------------------------
104.06............................... 104.06................. In 104.06A2, we would Based on SSA
A. 2. Arterial O2 saturation of less A. 2. Arterial blood use the measurement of administrative data
than 90 percent in room air, or gas test measurement millimeters of from FY 2019-2021, of
resting arterial PO2 of 60 Torr or obtained at rest while mercury, ``mmHg,'' all childhood claims
less; or. breathing room air, as instead of the with a primary
3. Hypercyanotic spells, syncope, described in either a measurement of impairment of
characteristic squatting, or other or b:. ``Torr'' that is used congenital heart
incapacitating symptoms directly a. SaO2 (arterial in current 104.06A2, disease that met or
related to documented cyanotic heart oxygen saturation) and we would note that medically equaled
disease; or. less than or equal to arterial PO2 is listing 104.06,
4. Exercise intolerance with 89 percent; or. normally measured in approximately .2
increased hypoxemia on exertion.. b. PO2 or PaO2 (partial room air. percent cited 104.06A3
pressure of oxygen) We would remove current or 104.06A4 criteria.
less than or equal to 104.06A3 and 104.06A4, See Table A and B in
60 mmHg; or. because Agency medical supporting and related
3. SpO2 (percentage of experts indicated they materials to this
oxygen saturation of are less objective and Docket for more
blood hemoglobin) more difficult to information.
measured by pulse document than the
oximetry either at other criteria and
rest, or after they are used
activity, while infrequently..
breathing room air, We would add another
less than or equal to criterion to 104.06A
87 percent on three by adding SpO2
evaluations at least (percentage of oxygen
30 days apart within a saturation of blood
consecutive 12-month hemoglobin), measured
period (see by pulse oximetry
104.00A3e).. equal to or less than
87 percent. Consistent
with the proposed
adult listing (4.06),
this criterion would
become the new
104.06A3..
[[Page 38853]]
No current criteria.................. E. Exacerbations or We would add 104.06E to IOM. (2010), 179.
complications of evaluate exacerbations
congenital heart or complications of
disease (see 104.00D) congenital heart
requiring three disease occurring at
hospitalizations least 30 days apart
within a consecutive and resulting in at
12-month period (see least three
104.00A3e) and at hospitalizations
least 30 days apart. within a consecutive
Each hospitalization 12-month period. An
must last at least 48 impairment resulting
hours, including hours in exacerbations or
in a hospital complications that
emergency department require this many
immediately before the hospitalizations in 12
hospitalization (see months will result in
104.00G3). marked and severe
functional limitations
for children. We would
require these
hospitalizations to be
at least 30 days apart
to ensure we are
evaluating separate
episodes of
exacerbations or
complications.
----------------------------------------------------------------------------------------------------------------
Proposed Listing 104.02--Chronic Heart Failure
We would clarify that to satisfy 104.02A, we would require two or
more tachycardia measurements in a consecutive 12-month period, and to
satisfy 104.02B, we would require two or more tachypnea measurements in
a consecutive 12-month period.\104\ Our intent is to ensure that the
child has persistent tachycardia or persistent tachypnea, despite
treatment. We would also require that readings of tachycardia or
tachypnea occur at least 90 days apart to further document chronic
disease.\105\
---------------------------------------------------------------------------
\104\ IOM. (2010), 171, 176.
\105\ IOM. (2010), 173.
---------------------------------------------------------------------------
When we last published final rules for growth disorders and weight
loss in children,\106\ we inadvertently removed Table I for tachycardia
at rest and Table II for tachypnea at rest in listing 104.02. We would
restore these tables to the proposed 104.02.
---------------------------------------------------------------------------
\106\ 80 FR 19522 (2015).
---------------------------------------------------------------------------
We would add new 104.02D to describe how we will evaluate chronic
heart failure treated with a mechanical circulatory support device.
We would add new 104.02E(), to describe how we will evaluate
exacerbations and complications of heart failure requiring extended
medical intervention in the hospital or emergency department, as
explained above.
Proposed Listing 104.06--Congenital Heart Disease
In 104.06A2, we would use the measurement of millimeters of
mercury, ``mmHg,'' instead of the measurement of ``Torr'' that is used
in current 104.06A2, and we would note that arterial PO2 is
normally measured in room air.
We would add another criterion to 104.06A by adding
SpO2 (percentage of oxygen saturation of blood
hemoglobin), measured by pulse oximetry equal to or less than 87
percent. This criterion would become the new criterion 104.06A3. As we
are proposing in the adult criteria, we would explain in the
introductory text to the childhood listings that we need pulse oximetry
measurements documented by medical sources using methods consistent
with the prevailing state of medical knowledge and clinical practice.
These measurements must be consistent with the other evidence in the
case record.
We would remove current 104.06A3 and 104.06A4, because they are
used infrequently.\107\ Our adjudicative experience shows that children
with impairments meeting these listings would be evaluated under
current and proposed 104.06.
---------------------------------------------------------------------------
\107\ Based on SSA administrative data from FY 2019-2021, of all
childhood claims with a primary impairment of congenital heart
disease that met or medically equaled listing 104.06, approximately
.2 percent cited these criteria. See Table A and B in supporting and
related materials to this Docket for more information.
---------------------------------------------------------------------------
We would add multiple medical readings for pulmonary hypertension
in 104.06B. We propose adding laboratory findings expressed in
millimeters of mercury (mmHG) in 104.06B2, and we would add mean
pulmonary artery pressure readings in 104.06B3.
We are proposing 104.06C, similar to the adult criterion 4.06D
(Congenital heart disease), to evaluate children born with a single
ventricle. Adding consideration of single ventricle to listing 104.06
enables seriously limited children to be identified earlier in the
sequential evaluation process.
We would add 104.06E to evaluate exacerbations or complications of
congenital heart disease occurring at least 30 days apart and resulting
in at least three hospitalizations within a consecutive 12-month
period. An impairment resulting in exacerbations or complications that
require this many hospitalizations in 12 months will result in marked
and severe functional limitations for children.\108\ We would require
these hospitalizations to be at least 30 days apart to ensure we are
evaluating separate episodes of exacerbations or complications.
---------------------------------------------------------------------------
\108\ IOM. (2010), 179.
---------------------------------------------------------------------------
Proposed Removal of Listing 104.13--Rheumatic Heart Disease
We would remove and reserve listing 104.13 because rheumatic heart
disease is a complication of rheumatic fever, which is rare in the
United States due to widely available treatment with
antibiotics.109 110 When complications of rheumatic fever
result in rheumatic heart disease, and these complications last for 12
months or more, we would evaluate the complications under other
cardiovascular listings, such as 104.02 (Chronic heart failure) or
104.05
[[Page 38854]]
(Recurrent arrhythmias). Rheumatic heart disease will still be
addressed in the introductory text under 104.00F6 (How will we evaluate
chronic rheumatic fever or rheumatic heart disease?).
---------------------------------------------------------------------------
\109\ Beaudoin, A., Edison, L., Introcaso, C.E., Goh, L.,
Marrone, J., Mejia, A. . . ., & Van Beneden, C. (2015). Acute
rheumatic fever and rheumatic heart disease among children--America
Samoa, 2011-2012. Morbidity and Mortality Weekly Report, 64(20),
555-558. Retrieved from https://www.cdc.gov/mmwr/pdf/wk/mm6420.pdf.
\110\ Yandrapalli, S., Tariq, S., Vuddanda, V.L.K., Sanaani, A.,
Solangi, Z., Anugu, V.R., . . . Aronow, W. (2017). In-hospital
outcomes and hospitalizations for acute rheumatic heart disease: A
United States national study. Journal of the American College of
Cardiology, 69(11)(Suppl.), 1742. doi:10.1016/S0735-1097(17)35131-8.
---------------------------------------------------------------------------
Proposed Listing 104.16--Cardiac Allograft Vasculopathy
We propose to add listing 104.16 (Cardiac allograft vasculopathy)
to evaluate a child who received a heart transplant and developed
cardiac allograft vasculopathy (CAV). CAV may develop after heart
transplantation and progress to a very serious condition with
significant functional effects. The medical literature indicates that
CAV is a leading cause of graft failure and mortality in pediatric
heart transplant recipients.\111\ To establish listing-level CAV for
children, we would require only CAV documented by appropriate medically
acceptable imaging, because pediatric CAV alone is disabling enough to
result in marked and severe functional limitations for children
---------------------------------------------------------------------------
\111\ Kindel, S.J. (2011).
---------------------------------------------------------------------------
Specific Questions for the Public
While the public is welcome to comment on any aspect of this
proposed rule, we are also seeking input on the following topics:
Should any of the proposed listings for cardiovascular
disorders be combined into one listing, or divided into multiple
listings, to enable our adjudicators to more easily identify adults or
children with impairments that are of listing-level severity? If you
believe our listing categories create unnecessary administrative
barriers for impairments that meet listing level severity, please tell
us by submitting your comments and any supporting research or data.
Are there changes in the medical terminology related to
cardiovascular disorders that we should consider incorporating or
clarifying in future revisions to the cardiovascular disorders
listings? If you believe we should consider updating the medical
terminology we use in our cardiovascular disorders listings, please
tell us by submitting your comments and any supporting research or
data.
Do the frequencies and durations of exacerbations of
cardiovascular disorders in this proposed rule adequately represent
listing level severity for cardiovascular disorders? Are there other
treatments and evidence we should consider when assessing listing-level
severity including additional objective medical tests, for any of the
proposed cardiovascular disorders listings? We encourage you to cite
relevant research or data to support your comments.
Are the proposed functional criteria for cardiovascular
disorders sufficient for assessing listing level severity? Please
provide specific suggestions along with supporting research and data
for different criteria you would like SSA to consider.
Did we not include any valuable information that should be
included in the introductory text of the cardiovascular disorders
listings? This text is intended to ease administrative burden for
adjudicators, claimants, claimant representatives, and the public.
Please submit specific comments, along with supporting research or
data, about additional information to include in the introductory text.
In proposed 4.02A1 (Chronic heart failure), we require
systolic failure documented by appropriate medically acceptable imaging
during a period of stability (not during an episode of exacerbation of
heart failure), with left ventricular end diastolic dimension equal to
or greater than 7.0 cm; or ejection fraction of 30 percent or less. If
you believe we should require more than one evaluation to document the
duration of an individual's chronic heart failure, please tell us by
submitting your comments and any supporting research or data.
In proposed 4.02A2 (Chronic heart failure), we require
diastolic failure documented by appropriate medically acceptable
imaging during a period of stability (not during an episode of
exacerbation of heart failure), with 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 left atrial volume index
(LAVi) greater than or equal to 40 ml, BSA/m2 (milliliters to body
surface area in squared meters). If you believe we should consider
other measurements of chronic heart failure, please tell us by
submitting your comments and any supporting research or data.
In proposed 104.02A (Chronic heart failure), we require
persistent tachycardia at rest measured 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. In proposed 104.02B, we
require persistent tachypnea 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. If you believe our proposed
requirement for at least two measurements of apical heart rate and
respiratory rate under this listing is inconsistent with current
medical practice or standards of care (i.e., medical providers do not
routinely repeat these measurements), please tell us by submitting your
comments and any supporting research or data.
In proposed 4.06A1 (Congenital heart disease), we require
two measurements of hematocrit at least 90 days apart within a
consecutive 12-month period instead of the current requirement for one
measurement. If you would like to propose a different time frame during
which these measures should occur, please submit comments and any
supporting research or data.
Are there alternatives to pulse oximetry testing that are
reliable, non-invasive, and commonly used to measure chronic hypoxemia
that we should consider incorporating into proposed listing criterion
4.06A3 (Congenital heart disease) and 104.06A3 (Congenital heart
disease)? If you believe there are tests that fit into this category,
please tell us by submitting your comments and any supporting research
or data.
At IOM's recommendation, we are proposing to add listing
4.07 (Aortic valvular disease) to provide evaluation criteria for
symptomatic adult individuals with aortic valvular disease.\112\ We
currently evaluate aortic valvular disease under other cardiovascular
disorders listings, which include requirements for exercise testing or
repeated hospitalizations. If you disagree with proposed 4.07 (Aortic
valvular disease), please tell us by submitting your comments and any
supporting research or data.
---------------------------------------------------------------------------
\112\ IOM. (2010), 195.
---------------------------------------------------------------------------
What is our authority to make rules and set procedures for determining
whether a person is disabled under the statutory definition?
The Act authorizes us to make rules and regulations and to
establish necessary and appropriate procedures to implement them.\113\
---------------------------------------------------------------------------
\113\ Sections 205(a), 702(a)(5), and 1631(d)(1) of the Social
Security Act.
---------------------------------------------------------------------------
How long would this proposed rule be effective?
If we publish this proposed rule as a final rule, it will remain in
effect for five years after the date it becomes effective, unless we
extend it, or revise and issue it again.
Rulemaking Analyses and Notices
We will consider all comments we receive on or before the close of
[[Page 38855]]
business on the comment closing date indicated above. The comments will
be available for examination in the rulemaking docket for this rule at
the above address. We will file comments received after the comment
closing date in the docket and will consider those comments to the
extent practicable. However, we will not address untimely comments. We
may publish a final rule at any time after close of the comment period.
Clarity of This Proposed Rule
Executive Order 12866, as supplemented by Executive Order 13563,
requires each agency to write all rules in plain language. In addition
to your substantive comments on this proposed rule, we invite your
comments on how to make them easier to understand.
For example:
Would more, but shorter, sections be better?
Are the requirements in the rule clearly stated?
Have we organized the material to suit your needs?
Could we improve clarity by adding tables, lists, or
diagrams?
What else could we do to make the rule easier to
understand?
Does the rule contain technical language or jargon that is
not clear?
Would a different format make the rule easier to
understand such as using different groupings and order of sections,
headings, or paragraphing?
When will we start to use this rule?
We will not use this proposed rule until we evaluate public
comments and publish a final rule in the Federal Register. All final
rules we issue include an effective date. We will continue to use our
current rule until that date. If we publish a final rule, we will
include a summary of the relevant comments we received and an
explanation of how we will apply the new rule.
Regulatory Procedures
Executive Order 12866, as Supplemented by Executive Order 13563
We consulted with the Office of Management and Budget (OMB) and
determined that this proposed rule meets the criteria for a significant
regulatory action under Executive Order 12866, as supplemented by
Executive Order 13563. Therefore, OMB reviewed the rule.
We also determined that this proposed rule meets the plain language
requirement of Executive Order 12866.
Executive Order 13132 (Federalism)
We analyzed this proposed rule in accordance with the principles
and criteria established by Executive Order 13132 and determined that
this proposed rule will not have sufficient federalism implications to
warrant the preparation of a federalism assessment. We also determined
that this proposed rule will not preempt any State law or State
regulation or affect the States' abilities to discharge traditional
State governmental functions.
Regulatory Flexibility Act
We certify that this proposed rule would not have a significant
economic impact on a substantial number of small entities because it
affects individuals only. Therefore, a regulatory flexibility analysis
is not required under the Regulatory Flexibility Act, as amended.
Executive Order 13771
Based upon the criteria established in Executive Order 13771 and M-
17-21 (Guidance Implementing E.O. 13771), we consider this rule a
transfer rule with no more than de minimis costs. As such, it is exempt
from requirements under E.O. 13771.
Anticipated Accounting Costs of This Proposed Rule
Anticipated Costs to Our Programs
Our Office of the Chief Actuary has developed estimates of the
effects of implementing this proposed rule, which are presented in a
memorandum attached to this NPRM as a supplementary document. The
memorandum indicates the estimated annual changes in Old-Age, Survivors
and Disability Insurance (OASDI) benefit payments and Federal
Supplemental Security Income (SSI) payments over the 10-year period of
fiscal years (FY) 2022-2031. The memorandum also provides details about
the case study developed for the purpose of making these estimates, as
well as changes since the time the case study was originally developed
and conducted, that may have impacted the case study results.
In summary, based on the best available data, our Office of the
Chief Actuary estimates that this proposed rule, assuming it is
finalized and implemented for all disability decisions completed on or
after April 1, 2023, would result in net increases of $308 million in
scheduled OASDI benefit payments and $71 million in Federal SSI
payments over the 10-year period of fiscal years (FY) 2022-2031.
Anticipated Administrative Costs to the Social Security Administration
The Office of Budget, Finance, and Management estimates a net
administrative savings of less than 15 work years and $2 million
annually.
Paperwork Reduction Act
This rule does not create any new or affect any existing
collections and, therefore, does not require OMB approval under the
Paperwork Reduction Act.
References
We consulted the following references when we developed this
proposed rule:
Adams, E.E., & Wrightson, M.L. (2018). Quality of life with an LVAD:
A misunderstood concept. Heart & Lung, 47(3), 177-183. doi:10.1016/
j.hrtlng.2018.02.003.
Agarwal, S., Sud, K., & Shishehbor, M.H. (2016). Nationwide trends
of hospital admission and outcomes among critical limb ischemia
patients: From 2003-2011. Journal of the American College of
Cardiology, 67(16), 1901-1913. doi:10.1016/j.jacc.2016.02.040.
Bavafa, H., Savin, S., & Terwiesch, C. (2019). Redesigning Primary
Care Delivery: Customized Office Revisit Intervals and E-Visits.
https://dx.doi.org/10.21con39/ssrn.2363685. Paper referenced by
Bavafa: Schectman, G., G. Barnas, P. Laud, L. Cantwell, M. Horton,
E.J. Zarling. 2005. Prolonging the return visit interval in primary
care. The American Journal of Medicine, 118(4) 393-39.
Beaudoin, A., Edison, L., Introcaso, C.E., Goh, L., Marrone, J.,
Mejia, A., & Van Beneden, C. (2015). Acute rheumatic fever and
rheumatic heart disease among children--America Samoa, 2011-2012.
Morbidity and Mortality Weekly Report, 64(20), 555-558. Retrieved
from https://www.cdc.gov/mmwr/pdf/wk/mm6420.pdf.
Berthelot-Richer, M., Pibarot, P., Capoulade, R., Dumesnil, J.G.,
Dahou, A., Thebault, C., . . . Clavel, M.-A. (2016). Discordant
grading of aortic stenosis severity: Echocardiographic predictors of
survival benefit associated with aortic valve replacement. Journal
of the American College of Cardiology: Cardiovascular Imaging, 9(7),
797-805. doi:10.1016/j.jcmg.2015.09.026.
Bistola, V., Arfaras-Melainis, A., Polyzogopoulou, E., Ikonomidis,
I., & Parissis, J. (2019). Inotropes in acute heart failure: From
guidelines to practical use: Therapeutic options and clinical
practice. Cardiac Failure Review, 5(3), 133-139. doi:10.15420/
cfr.2019.11.2.
Blume, E.D., Rosenthal, D.N., Rossano, J.W., Baldwin, J.T.,
Eghtesady, P., Morales, D.L., . . . Kirklin, J.K.. (2016). Outcomes
of children implanted with ventricular assist devices in the United
States: First analysis of the pediatric interagency registry of
mechanical circulatory support (PediMACS). Journal of Heart
[[Page 38856]]
and Lung Transplantation, 35(5), 578-584. doi:10.1016/
j.healun.2016.01.1227.
Cacciapuoti, Fu., Scognamiglio, A., Paoli, V.D., Romano, C., &
Cacciapuoti, Fe. (2012). Left atrial volume index as indicator of
left ventricular diastolic dysfunction: Comparation between left
atrial volume index and tissue myocardial performance index. Journal
of Cardiovascular Ultrasound, 20(1), 25-29. doi:10.4250/
jcu.2012.20.1.25.
Cedars, A., Benjamin, L., Burns, S.V., Novak, E., & Amin, A. (2017).
Clinical predictors of length of stay in adults with congenital
heart disease. Heart (British Cardiac Society), 103(16), 1258-1263.
https://doi.org/10.1136/heartjnl-2016-310841.
Cha, Y.-M., Lee, G.K., Klarich, K.W., & Grogan, M. (2012). Premature
ventricular contraction-induced cardiomyopathy: A treatable
condition. Circulation: Arrhythmia and Electrophysiology, 5(1), 229-
236. doi:10.1161/CIRCEP.111.963348.
Chen, J., Dharmarajan, K., Wang, Y., & Krumholz, H.M. (2013).
National trends in heart failure hospital stay rates, 2001 to 2009.
Journal of the American College of Cardiology, 61(10), 1078-1088.
doi:10.1016/j.jacc.2012.11.057.
Cheng, R.K., Cox, M., Neely, M.L., Heidenreich, P.A., Bhatt, D.L.,
Eapen, Z.J., . . . Fonarow, G.C. (2014). Outcomes in patients with
heart failure with preserved, borderline, and reduced ejection
fraction in the Medicare population. American Heart Journal, 168(5),
721-730. doi:10.1016/j.ahj.2014.07.008.
Ciarka, A., Edwards, L., Nilsson, J., Stehlik, J., & Lund, L.H.
(2017). Trends in the use of mechanical circulatory support as a
bridge to heart transplantation across different age groups.
International Journal of Cardiology, 231, 225-227. doi:10.1016/
j.ijcard.2016.10.049.
Cohen, S., & Marelli, A. (2016). Evolving heart transplantation
across the lifespan: A growing population of adults with congenital
heart disease. Archives of Cardiovascular Disease, 109(10), 511-513.
doi:10.1016/j.acvd.2016.05.001.
Cohen-Solal, A., Laribi, S., Ishihara, S., Vergaro, G., Baudet, M.,
Logeart, D., . . . Seronde, M.-F. (2015). Prognostic markers of
acute decompensated heart failure: The emerging roles of cardiac
biomarkers and prognostic scores. Archives of Cardiovascular
Disease, 108(1), 64-74. doi:10.1016/j.acvd.2014.10.002.
Desai, A.S. (2013). Are serial BNP measurements useful in heart
failure management? Serial natriuretic peptide measurements are not
useful in heart failure management: The art of medicine remains
long. Circulation, 127(4), 509-516. doi:10.1161/
CIRCULATIONAHA.112.120493.
Desai, R.V., Guichard, J.L., Mujib, M., Ahmed, M.I., Feller, M.A.,
Fonarow, G.C., . . . Ahmed, A. (2013). Reduced right ventricular
ejection fraction and increased mortality in chronic systolic heart
failure patients receiving beta-blockers: Insights from the BEST
trial. International Journal of Cardiology, 163(1), 61-67.
doi:10.1016/j.ijcard.2011.05.051.
Dukes, J.W., Dewland, T.A., Vittinghoff, E., Mandyam, M.C.,
Heckbert, S.R., Siscovick, D.S., . . . Marcus, G.M. (2015).
Ventricular ectopy as a predictor of heart failure and death.
Journal of the American College of Cardiology, 66(2), 101-109.
doi:10.1016/j.jacc.2015.04.062.
Fearon, W.F. (2014). Percutaneous coronary intervention should be
guided by fractional flow reserve measurement. Circulation, 129(18),
1860-1870. doi:10.1161/CIRCULATIONAHA.113.004300.
Fearon, W.F., De Bruyne, B., & Pijlis, N.H. (2016). Fractional flow
reserve in acute coronary syndromes. Journal of the American College
of Cardiology, 68(11), 1192-1194. doi:10.1016/j.jacc.2016.07.713.
Fihn, S.D., Gardin, J.M., Abrams, J., Berra, K., Blankenship, J.C.,
Dallas, A.P., . . . Williams, S.V. (2012). 2012 ACCF/AHA/ACP/AATS/
PCNA/SCAI/STS Guideline for the diagnosis and management of patients
with stable ischemic heart disease: Executive summary: A report of
the American College of Cardiology Foundation/American Heart
Association Task Force on Practice Guidelines, and the American
College of Physicians, American Association for Thoracic Surgery,
Preventive Cardiovascular Nurses Association, Society for
Cardiovascular Angiography and Interventions, and Society of
Thoracic Surgeons. Circulation, 126(25), 3097-3137. doi:10.1161/
CIR.0b013e3182776f83.
Fletcher, G.F., Ades, P.A., Kligfield, P., Arena, R., Balady, G.J.,
Bittner, V.A., . . . Williams, M.A. (2013). Exercise standards for
testing and training: A scientific statement from the American Heart
Association. Circulation, 128(8), 873-934. doi:10.1161/
CIR.0b013e31829b5b44.
Frigola-Capell, E., Comin-Colet, J., Davins-Miralles, J., Gich-
Saladich, I., Wensing, M., & Verd[uacute]-Rotellar, J.M. (2012).
Trends and predictors of hospitalization, readmissions and length of
stay in ambulatory patients with heart failure. Revista Clinica
Espanola, 213(1), 1-7. doi:10.1016/j.rce.2012.10.006.
Henry, T.D., Satran, D., Hodges, J.S., Johnson, R.K., Poulose, A.K.,
Campbell, A.R., . . . Traverse, J.H. (2013). Long-term survival in
patients with refractory angina. European Heart Journal, 34(34),
2683-2688. doi:10.1093/eurheartj/eht165.
H[ouml]baus, C., Roller-Wimsberger, Schernthaner, G.-H. (2016).
Peripheral arterial disease and loss of physical function: Just two
old friends? Atherosclerosis, 257, 246-247. doi:10.1016/
j.atherosclerosis.2016.12.015.
Hua, M., Gong, M.N., Brady, J., & Wunsch, H. (2015). Early and late
unplanned rehospitalizations for survivors of critical illness.
Critical Care Medicine, 43(2), 430-438. doi:10.1097/
CCM.0000000000000717.
Institute of Medicine. (2010). Cardiovascular Disability: Updating
the Social Security Listings. Washington, DC: The National Academies
Press.
Jackson SL, Tong X, King RJ, et al. (2018). National Burden of Heart
Failure Events in the United States, 2006 to 2014. Circulation:
Heart Failure. 2018 Dec;11(12):e004873. DOI: 10.1161/
circheartfailure.117.004873. PMID: 30562099; PMCID: PMC6424109.
Januzzi, J.L., Chen-Tournoux, A.A., Christenson, R.H., Doros, G.,
Hollander, J.E., Levy, P.D., . . . Gaggin, H.K. (2018). N-terminal
in pro-B-type natriuretic peptide in the emergency department: The
ICON-RELOADED Study. Journal of the American College of Cardiology,
71(11), 1191-1200. doi:10.1016/j.jacc.2018.01.021.
Koene, R.J., Adkisson, W.O., & Benditt, D.G. (2017). Syncope and the
risk of sudden cardiac death: Evaluation, management, and
prevention. Journal of arrhythmia, 33(6), 533-544. https://doi.org/10.1016/j.joa.2017.07.005.
Khan, T.Z., Pottle, A., Pennell, D.J., & Barbir, M.S. (2015). The
impact of lipoprotein apheresis in patients with refractory angina
and raised lipoprotein(a): Objectives and methods of a randomized
controlled trial. Atherosclerosis Supplements, 18, 103-108.
doi:10.1016/j.atherosclerosissup.2015.02.019.
Kim, Y., Gani, F., Canner, J.K., Margonis G.A., Makary, M.A.,
Schneider, E.B., & Pawlik, T.M. (2016). Hospital readmission after
multiple major operative procedures among patients with employer
provided health insurance. Surgery, 160(1), 178-190. doi:10.1016/
j.surg.2016.01.025.
Kindel, S.J., & Pahl, E. (2011). Cardiac allograft vasculopathy in
children--treatment challenges. Progress in Pediatric Cardiology,
32(1), 37-42. doi:10.1016/j.ppedcard.2011.06.008.
Kindel, S.J., Law, Y.M., Chin, C., Burch, M., Kirklin. J.K., Naftel,
D.C., . . . Pahl, E. (2015). Improved detection of cardiac allograft
vasculopathy: A multi-institutional analysis of functional
parameters in pediatric heart transplant recipients. Journal of the
American College of Cardiology, 66(5), 547-557. doi:10.1016/
j.jacc.2015.05.063.
Kobashingawa, J.A. (2015). The changing face of first-year
intravascular ultrasonography in heart transplantation. Journal of
the American College of Cardiology: Heart Failure, 3(12), 954-955.
doi:10.1016/j.jchf.2015.09.004.
Khoo, C., Chakrabarti, S., Arbour, L. & Krahn, A.D. (2013).
Recognizing life-threatening causes of syncope. Cardiology Clinics,
31(1), 51-66. doi:10/1016/j.cci.2012.10.005.
Kyriakou, M., & Kiff, P.F. (2016). Prognosis of the comorbid heart
failure and anemia: A systematic review and meta-analysis. Clinical
Trials and Regulatory Science in Cardiology, 16, 12-21. doi:10.1016/
j.ctrsc.2016.01.008.
Marine, J.E. (2013). ECG features that suggest a potentially life-
threatening arrhythmia as the cause of syncope. Journal of
[[Page 38857]]
Electrocardiology, 46(6), 561-568. doi:10.1016/
j.jelectrocard.2013.07.008.
Malotte, K., Saguros, A., & Groninger, H. (2018). Continuous cardiac
inotropes in patients with end-stage heart failure: An evolving
experience. Journal of Pain Symptom Management, 55(1), 159-163.
doi:10.1016/j.jpainsymman.2017.09.026.
Mechem, C.C. (2014). Pulse oximetry. In P.E. Parsons (Ed.), UpToDate
(Jan. 2014). Retrieved from https://www.uptodate.com/contents/pulse-oximetry.
Mehra, M.R., Crespo-Leiro, M.G., Dipchand, A., Ensminger, S.M.,
Hiemann, N.E., Kobashigawa, J.A., . . . Uber, P.A. (2010).
International Society of Heart and Lung Transplantation working
formulation of a standardized nomenclature for cardiac allograft
vasculopathy--2010. Journal of Heart and Lung Transplantation,
29(7), 717-727. doi:10.1016/j.healun.2010.05.017.
Nakano, Y. & Wataru, S. (2017). Syncope in patients with inherited
arrhythmias. Journal of Arrhythmia, 33(6), 572-578. doi:10.1016/
j.joa.2017.07.007.
National Academies of Sciences, Engineering, and Medicine. (2018).
Health-care utilization as a proxy in disability determination.
Washington, DC: The National Academies Press. doi:10.17226/24969.
Nieminen, M.S., Dickstein, K., Fonseca, C., Serrano, J.M., Parissis,
J., Fedele, F., . . . Zymli[nacute]ski, R. (2015). The patient
perspective: Quality of life in advanced heart failure with frequent
hospitalizations. International Journal of Cardiology, 191, 256-264.
doi:10.1016.j.ijcard.2015.04.235.
Nixon, J.L., Benuzillo, J., McCubrey, R.O., Budge, D., Rasmusson,
K., Brush, S., . . . Kfoury, A. (2013). Is 30-day readmission a
valid measure for quality care or simply an indicator of severe
disease in patients discharged after heart failure hospitalization?
Journal of Cardiac Failure, 19(8)(Suppl.), S80-S81. doi:10.1016/
j.cardfail.2013.06.257.
Nombela-Franco, L., del Trigo, M., Morrison-Polo, G., Veiga, G.,
Jimenez-Quevedo, P., Altisent, O. A.-J., . . . Rod[eacute]s-Cabau,
J. (2015). Incidence, causes, and predictors of early (<30 days) and
late unplanned hospital readmissions after transcatheter aortic
valve replacement. Journal of the American College of Cardiology:
Cardiovascular Interventions, 8(13), 1748-1757. doi:10.1016/
j.jcin.2015.07.022.
Okada, K., Kitahara, H., Yang, H., Tanaka, S., Kobayashi, Y.,
Kimura, T., . . . Fearon, W.F. (2015). Paradoxical vessel remodeling
of the proximal segment of the left anterior descending artery
predicts long-term mortality after heart transplantation. Journal of
the American College of Cardiology: Heart Failure, 3(12), 945-952.
doi:10.1016/j.jchf.2015.07.013.
Oster, M.E., & Kochilas, L.K. (2016). Screening for critical
congenital heart disease. Clinics in Perinatology, 43(1), 73-80.
doi:10.1016/j.clp.2015.11.005.
Park, S.H., Jeon, K.-H., Lee, J.M., Nam, C.-W., Doh, J.-H., Lee, B.-
K., . . . Koo, B.-K. (2015). Long-term clinical outcomes of
fractional flow reserve-guided versus routine drug-eluting stent
implantation in patients with intermediate coronary stenosis: Five-
year clinical outcomes of DEFER-DES trial. Circulation:
Cardiovascular Interventions, 8(12), e002442. doi:10.1161/
CIRCINTERVENTIONS.115.002442.
Patel, D.A., Lavie, C.J., Milani, R.V., Shah, S., & Gilliland, Y.
(2009). Clinical implications of left atrial enlargement: A review.
The Ochsner Journal, 9(4), 191-196.
Patterson, C.C., Blankenberg, S., Ben-Shlomo, Y., Heslop, L., Bayer,
A., Lowe, G., . . . Yarnell, J. (2015). Which biomarkers are
predictive specifically for cardiovascular or for non-cardiovascular
mortality in men? Evidence from the Caerphilly Prospective Study
(CaPS). International Journal of Cardiology, 201, 113-118.
doi:10.1016/j.ijcard.2015.07.106.
Pijls, N.H., Fearon, W.F., Tonino, P.A., Siebert, U., Ikeno, F.,
Bornschein, B., . . . De Bruyne, B. (2010). Fractional flow reserve
versus angiography for guiding percutaneous coronary intervention in
patients with multivessel coronary artery disease: 2-year follow-up
of the FAME (Fractional Flow Reserve Versus Angiography for
Multivessel Evaluation) study. Journal of the American College of
Cardiology, 56(3), 177-184. doi:10.1016/j.jacc.2010.04.012.
Poredo[scaron] P, Jezovnik M, Kalodiki E. Medical management of
patients with peripheral arterial disease. Int Angiol. 2015
Feb;34(1):75-93. Epub 2014 Jun 11. PMID: 24916346.
Reynolds, K., Butler, M.G., Kimes, T.M., Rosales, A.G., Chan, W., &
Nichols, G.A. (2015). Relation of acute heart failure hospital
length of stay to subsequent readmission and all-cause mortality.
American Journal of Cardiology, 116(3), 400-405. doi:10.1016/
j.amjcard.2015.04.052.
Rod[eacute]s-Cabau, J. (2015). Incidence, causes, and predictors of
early (<30 days) and late unplanned hospital readmissions after
transcatheter aortic valve replacement. Journal of the American
College of Cardiology: Cardiovascular Interventions, 8(13), 1748-
1757. doi:10.1016/j.jcin.2015.07.02.
Rosenthal, D.N., Almond, C.S., Jaquiss, R.D., Peyton, C.E.,
Auerbach, S.R., Morales, D.R., . . . Blume, E.D. (2016). Adverse
events in children implanted with ventricular assist devices in the
United States: Data from the Pediatric Interagency Registry for
Mechanical Circulatory Support (PediMACS). The Journal of Heart and
Lung Transplantation, 35(5), 569-577. doi:10.1016/
j.healun.2016.03.005.
Roston, T.M., Tran, D.T., Sanatani, S., Sandhu, R., Sheldon, R., &
Kaul, P. (2017). A population-based study of syncope in the young.
Canadian Journal of Cardiology, 34(2), 195-201. doi:10.1016/
j.cjca.2017.12.006.
Runge, M.S., Patterson, C., Stouffer, G.A., & Netter, F. H. (2010).
Netter's Cardiology (2nd ed.). Philadelphia, PA: Saunders Elsevier.
Salamanca-Bautista, P., Conde-Martel, A., Aramburu-Bodas, [Oacute].,
Formiga, F., Trull[agrave]s, J.C., Quesada-Sim[oacute]n, M.A., . . .
Montero-P[eacute]rez-Barquero, M. (2016). Precipitating factors of
heart failure admission: Differences related to age and left
ventricular ejection fraction. International Journal of Cardiology,
219, 150-155. doi:10.1016/j.ijcard.2016.06.040.
Santangeli, P., & Marchlinski, F.E. (2015). Ventricular ectopy as a
modifiable risk factor for heart failure and death:
``D[eacute]j[agrave] vu all over again'' may be a good thing.
Journal of the American College of Cardiology, 66(2), 110-112.
doi:10.1016/j.jacc.2015.05.031.
Shlofmitz, E., and Jeremias, A. (2017). FFR in 2017: Current Status
in PCI Management-American College of Cardiology. Available online
at: https://www.acc.org/latest-in-cardiology/articles/2017/05/25/08/34/ffr-in-2017-current-status-in-pci-management (accessed September
17, 2021).
Sheikh, A., Patel, N.J., Nalluri, N., Agnihotri, K., Spagnola, J.,
Patel, A., . . . Paydak, H. (2015). Trends in hospitalization for
atrial fibrillation: Epidemiology, cost, and implications for the
future. Progress in Cardiovascular Diseases, 58(2), 105-116.
doi:10.1016/j.pcad.2015.07.002.
Siracuse, J.J., Gill, H.L., Jones, D.W., Schneider, D.B, Connolly,
P.H., Parrack, I., . . . Meltzer, A.J. (2014). Risk factors for
protracted preoperative length of stay after lower extremity bypass
for critical limb ischemia. Annals of Vascular Surgery, 28(6), 1432-
1438. doi:10.1016/j.avsg.2013.12.027.
Sorensen, L.L, Liang, H.-L., Pinheiro, A., Hilser, A., Dimaano, V.,
Olsen, N.T., . . . Abraham, T.P. (2017). Safety profile and utility
of treadmill exercise in patients with high-gradient hypertrophic
cardiomyopathy. American Heart Journal, 184, 47-54. doi:10.1016/
j.ahj.2016.10.010.
Stack, S.W., & Berger, S.A. (2009). The effects of high hematocrit
arterial flow--A phenomenological study of health risk implications.
Chemical Engineering Science, 64(22), 4701-4706. doi:10/1016/
j.ces.2009.07.017.
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.
doi:10.1016/j.jacc.2018.08.1029.
Tebaldi, M., Biscaglia, S., Pecoraro, A., Fineschi, M., & Campo, G.
(2016). Fractional flow reserve implementation in daily clinical
practice: A European survey. International Journal of Cardiology,
207, 206-207. doi:10.1016/j.ijcard.2016.01.097.
Thrush, P.T., & Canter, C.E. (2015). Looking to the future of
ventricular assist devices in pediatric cardiomyopathy. Progress in
Pediatric Cardiology, 39(1), 21-23. doi:10.1016/
j.ppedcard.2015.01.003.
[[Page 38858]]
Uszko-Lencer, N.H., Frankenstein, L., Spruit, M.A., Maeder, M.T.,
Gutmann, M., Muzzarelli, S., . . . Brunner-La Rocca, H.-P. (2017).
Predicting hospitalization and mortality in patients with heart
failure: The BARDICHE-index. International Journal of Cardiology,
227, 901-907. doi:10.1016/j.ijcard.2016.11.122.
Versteeg, H., Hoogwegt, M.T., Hansen, T.B., Pedersen, S.S., Zwisler,
A.-D., & Thygesen, L.C. (2013). Depression, not anxiety, is
independently associated with 5-year hospitalizations and mortality
in patients with ischemic heart disease. Journal of Psychosomatic
Research, 75(6), 518-525. doi:10.1016/j.jpsychores.2013.10.005.
Wei, B., Qian, C., Fang, Q., & Wang, Y. (2016). The prognostic value
of peripheral artery disease in heart failure: Insights from a meta-
analysis. Heart, Lung and Circulation, 25(12), 1195-1202.
doi:10.1016/j.hlc.2016.04.002.
Yandrapalli, S., Tariq, S., Vuddanda, V.L.K., Sanaani, A., Solangi,
Z., Anugu, V.R., . . . Aronow, W. (2017). In-hospital outcomes and
hospitalizations for acute rheumatic heart disease: A United States
national study. Journal of the American College of Cardiology,
69(11)(Suppl.), 1742. doi:10.1016/S0735-1097(17)35131-8.
Yu, P.-J., Cassiere, H.A., Fishbein, J., Esposito, R.A., & Hartman,
A.R. (2016). Outcomes of patients with prolonged intensive care unit
length of stay after cardiac surgery. Journal of Cardiothoracic and
Vascular Anesthesia, 30(6), 1550-1554. doi:10.1053/
j.jvca.2016.03.145.
Ziberszac, R., Gabriel, H., Schemper, M., Laufer, G., Maurer, G., &
Rosenhek, R. (2017). Asymptomatic severe aortic stenosis in the
elderly. Journal of the American College of Cardiology:
Cardiovascular Imaging, 10(1), 43-50. doi:10.1016/
j.jcmg.2016.05.015.
We will make these references available to you for inspection if
you are interested in reading them. Please make arrangements with the
contact person shown in this preamble if you would like to review any
reference materials.
(Catalog of Federal Domestic Assistance Program Nos. 96.001, Social
Security--Disability Insurance; 96.002, Social Security--Retirement
Insurance; 96.004, Social Security--Survivors Insurance; and 96.006,
Supplemental Security Income).
List of Subjects in 20 CFR Part 404
Administrative practice and procedure; Blind, Disability benefits;
Old-age, survivors, and disability insurance; Reporting and
recordkeeping requirements; Social Security.
The Acting Commissioner of Social Security, Kilolo Kijakazi, Ph.D.,
M.S.W., having reviewed and approved this document, is delegating the
authority to electronically sign this document to Faye I. Lipsky, who
is the primary Federal Register Liaison for the Social Security
Administration, for purposes of publication in the Federal Register.
Faye I. Lipsky,
Federal Register Liaison, Office of Legislation and Congressional
Affairs, Social Security Administration.
For the reasons set forth in the preamble, we propose to amend
subpart P of part 404 of title 20 of the Code of Federal Regulations as
set forth below:
PART 404--FEDERAL OLD-AGE, SURVIVORS AND DISABILITY INSURANCE
(1950- )
0
1. The authority citation for subpart P of part 404 continues to read
as follows:
Authority: Secs. 202, 205(a)-(b), and (d)-(h), 216(i), 221(a)
and (h)-(j), 222(c), 223, 225, and 702(a)(5) of the Social Security
Act (42 U.S.C. 402, 405(a)-(b), and (d)-(h), 416(i), 421(a) and (h)-
(j), 422(c), 423, 425, and 902(a)(5)); 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 by:
0
a. Revising item 5 of the introductory text before part A;
0
b. Revising the body system name for section 4.00 in the table of
contents;
0
c. Revising the heading of 4.00A, the heading of 4.00A1, the
introductory text of 4.00A1b, and 4.00A1b(iv), 4.00A2, and the first
sentence of 4.00A3a; and adding two sentences to 4.00A3b, and two
sentences to 4.00A3c; and removing 4.00A3f;
0
d. Revising the heading of 4.00B, the first sentence of 4.00B1, the
third sentence of 4.00B2, the second and fourth sentences of 4.00B3(a),
the second sentence of 4.00B3(b), and the first sentence of 4.00B5;
0
e. Revising the heading of 4.00C, the second sentence of 4.00C3(c),
4.00C6(a)(i), 4.00C7(b) through (d), 4.00C8(d)(iv), 4.00C8(e), and
4.00C9(a); removing the third sentence of 4.00C15(a), revising
4.00C15(b), removing 4.00C15(c); and revising the first two sentences
of 4.00C16;
0
f. Revising the heading of 4.00D; adding new fourth and fifth sentences
to 4.00D1a; revising the second sentence of 4.00D1b; adding a third
sentence to 4.00D1b; revising 4.00D2(a)(i) through (iii), the first
sentence of 4.00D2(b), the second sentence of 4.00D2(b)(i), the second
sentence of 4.00D2(b)(ii), the third sentence of 4.00D3, 4.00D4(b) and
4.00D4(c) and the first, second, and fifth sentences of 4.00D4(d); and
adding 4.00D4(e);
0
g. Revising the heading of 4.00E, 4.00E2b; the first sentence of
4.00E5, 4.00E7(b)(i)-(ii); and adding 4.00E7(b)(iii);
0
h. Revising the first three sentences of 4.00E8, and4.00E9(b) through
(f), and removing 4.00E9(g) and (h);
0
i. Revising the heading of 4.00F; adding a new sentence to 4.00F1,
revising the first sentence of 4.00F3(a), 4.00F4(a), and the second and
fourth sentences of 4.00F4(b);
0
j. Revising the heading of 4.00G; adding a fourth sentence to 4.00G1,
revising 4.00G2, the first two sentences of 4.00G4(b), 4.00G6, and the
fourth sentence of 4.00G7(b);
0
k. Redesignating 4.00H and I as 4.00I and J, respectively
0
l. Adding a new 4.00H;
0
m. Revising the heading of 4.00I; 4.00I1, removing 4.00I2, and
redesignating 4.00I3 through 5 as 4.00I2 through 4;
0
n. Revising 4.00I2, 4.00I3, 4.00I4(a) and (d), adding a new 4.00I5, and
revising the third sentence of 4.00I8b;
0
o. Revising the heading of 4.00J, 4.00J1, the first sentence of 4.00J2,
redesignating 4.00J3 as 4.00K, and adding new 4.00J3;
0
p. Revising 4.00K;
0
q. Revising listings 4.01 and 4.02, adding and reserving listing 4.03,
revising listings 4.05 and 4.06, adding listing 4.07, adding listing
4.08, revising 4.09 through 4.12, adding and reserving listings 4.13
through 4.15, and adding listing 4.16.
0
r. Revising the heading of 104.00A; the heading of 104.00A1; the
introductory text of 104.00A1(b), 104.00A1(b)(iv), and 104.00A2; and
the first sentence of 104.00A3(a), and adding two sentences to
104.00A3(b), adding two sentences to 104.00A3(c), and removing
104.00A3(f) and (g);
0
s. Revising the heading of 104.00B; the first sentence of 104.00B1; the
third sentence of 104.00B2; the second and fourth sentences of
104.00B3(a); the second sentence of 104.00B3(b); 104.00B4(a)(i); the
first and third sentences of 104.00B5; the heading of 100.04B7; the
second sentence of 100.04B7(a); and the first sentence of 104.00B7(b);
0
t. Revising the heading of 104.00C; the heading of 104.00C1, and the
first sentence of 104.00C1a; adding two sentences to 104.00C1a;
revising 104.00C1b; 104.00C2(a);.00C2(b), and the second sentence of
104.00C2(b)(iii); and adding 104.00C4;
0
u. Revising the heading of 104.00D; 104.00D1, 104.00D1d, and 104.00D2;
and adding 104.00D3 and 104.00D4;
0
v. Revising the heading of 104.00E; adding a new sentence to the end of
104.00E1; revising the fourth and fifth sentences of 104.00E4(a), and
the fourth sentence of 104.00E4(b);
[[Page 38859]]
0
w. Revising the heading of 104.00F; the last sentence of 104.00F1, the
first sentence of 104.00F2; removing the fourth through seventh
sentences of 104.00F3; adding a new 104.00F3a and 104.00F3b; revising
104.00F4, 104.00F5(a), 104.00F5(d), 104.00F6, and 104.00F9b; and adding
104.00F11;
0
x. Revising the heading of 104.00G; 104.00G1, the first sentence of
104.00G2; redesignating 104.00G3 as 104.00H, 104.00G3(a) as 104.00H1,
and 104.00G3(b) as 104.00H2; and adding a new 104.00G3;
0
y. Revising 104.00H; and
0
z. Revising listings 104.01,104.02; adding and reserving 104.03 and
104.04; revising 104.05 and 104.06; adding and reserving 104.07 and
104.08; revising 104.09; adding and reserving listings 104.10 through
104.12; removing and reserving listing 104.13; adding and reserving
listings 104.14 and 104.15: and adding listing 104.16.
The additions and revision to read as follows:
Appendix 1 to Subpart P of Part 404--Listing of Impairments
* * * * *
5. Cardiovascular Disorders (4.00 and 104.00) [DATE 5 YEARS FROM
THE EFFECTIVE DATE OF THE FINAL RULE].
* * * * *
Part A
* * * * *
4.00 Cardiovascular Disorders.
* * * * *
4.00 Cardiovascular Disorders
A. How do we define cardiovascular disorders and cardiovascular
terms?
1. What do we mean by a cardiovascular disorder?
a. * * *
b. Cardiovascular disorders result from one or more of four
consequences of heart disease:
(i) * * *
(ii) * * *
(iii) * * *
(iv) Hypoxemia due to right-to-left shunt, reduced oxygen
concentration in the arterial blood, or pulmonary vascular disease.
c. * * *
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. * * *
a. Medical consultant is a person defined in Sec. Sec.
404.1616(a) and 416.1016(a) of this chapter. * * *
b. * * * 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. * * * 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.
* * * * *
f. [Removed]
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.
* * * * *
2. Why is a longitudinal clinical record important? * * *
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). * * *
3. * * *
a. * * * In this situation, we will base our evaluation on the
current evidence we have. * * * However, we may find you disabled
because you have another impairment(s) that, in combination with
your cardiovascular disorder, medically equals a listing or based on
consideration of your residual functional capacity and age,
education, and work experience.
b. * * * In rare 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.
* * * * *
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. * * *
* * * * *
C. How do we use cardiovascular test results?
* * * * *
3. * * *
c. * * * 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. * * *
* * * * *
6. * * *
a. * * *
(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 have (see 4.00C9), and we
cannot find you disabled on some other basis; or
* * * * *
7. * * *
a. * * *
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.
* * * * *
8. * * *
d. * * *
(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. * * *
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.
* * * * *
15. * * *
a. * * *
b. Cardiac catheterization reports commonly include evaluation
of coronary artery size and flow patterns, pressures in the left and
right side of the heart, and evaluation of wall motion and ejection
fraction, as well as chamber size. Also more routinely included in
the catheterization report is fractional flow reserve (FFR), which
is an objective measure of flow access across an obstruction. FFR
also helps define the adequacy of collateral flow that directly
affects function in ischemic heart disease.
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, and the reasons for stopping exercise if
the expected level of exercise was not attained. These reports 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. * * *
* * * * *
D. How do we evaluate chronic heart failure?
1. * * *
a. * * * Ejection fraction in heart failure is a continuum
ranging from low ejection
[[Page 38860]]
fraction due to muscle dysfunction to preserved ejection fraction
resulting from high intracardiac pressures. We consider heart
failure to be chronic when the condition persists or recurs over
time despite treatment. * * *
b. * * * If the CHF 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-pro-BNP) in the blood assists in differentiating chronic
heart failure from non-heart failure symptoms.
2. * * *
a. * * *
(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 equal to or greater than 7.0 cm, or an EF of 30
percent or less during a period of stability (that is, not during an
episode of acute 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.
* * * * *
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. * * *
(i) * * * People with CHF may also experience shortness of
breath upon lying flat (orthopnea) or episodes of shortness of
breath that wake them from sleep (paroxysmal nocturnal dyspnea). * *
*
(ii) * * * 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. Is it safe for you to have an ETT if you have CHF? * * * ETT
has been used safely in people with CHF. 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. * * *
4. * * *
a. * * *
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. * * * 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. * * *
e. How do we evaluate CHF treated with a mechanical circulatory
support device? We use 4.02D1 to evaluate CHF 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). Although ECMO is a form of mechanical
circulatory support, we do not include it in 4.02D1 because ECMO is
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. * * *
2. * * *
a. * * *
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.
* * * * *
5. What is anginal equivalent? Often, people with IHD will
complain of shortness of breath (dyspnea) on exertion without chest
pain or discomfort. * * *
* * * * *
7. * * *
a. * * *
b. * * *
(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.
* * * * *
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. * * *
9. * * *
a. * * *
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 last for at least 1 minute of
[[Page 38861]]
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, the term 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.
f. In 4.04D2 and 4.04D3, 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? * * * Although we use the term
``arrhythmia'' in the listings, the term ``dysrhythmia'' may also be
used in the medical evidence to describe this condition.
* * * * *
3. * * *
a. We will use 4.05 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. * * *
* * * * *
4. * * *
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. * * * In some people, these functions may result in the
termination of ventricular arrhythmias without an otherwise painful
shock. * * * 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.
* * *
* * * * *
G. How do we evaluate peripheral vascular disease?
1. What is peripheral vascular disease (PVD)? * * * Neuropathy
may mask these typical symptoms. * * *
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.
* * * * *
4. * * *
a. * * *
b. Lymphedema does not meet the requirements of 4.11, although
it may medically equal the listing. We will 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.
* * *
5. * * *
6. Are there any other studies that are helpful in evaluating
PAD? Doppler studies done using a recording ultrasonic Doppler unit
and strain-gauge 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 tibia arteries, is an evaluation tool that compares
waveforms in normal and compromised peripheral blood flow.
Qualitative analysis of the pulse wave 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. * * *
a. * * *
b. * * * The criterion in 4.12A is met when your resting ankle/
brachial systolic blood pressure ratio is less than 0.50. * * *
* * * * *
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, he or she is 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).
[[Page 38862]]
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. 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) Double inlet left ventricle; and
(e) 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 will 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 several types: dilated,
hypertensive, hypertrophic, and restrictive. Cardiomyopathy includes
hypertrophic cardiomyopathy, endomyocardial fibrosis, or cardiac
amyloidosis AL 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 4.08A2, 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 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 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. * * *
c. * * *
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.
* * * * *
8. * * *
a. * * *
b. * * * Most people with Marfan syndrome have abnormalities
associated with the heart and blood vessels. * * * * *
J. How do we evaluate 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. * * *
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,
[[Page 38863]]
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:
1. Systolic failure documented by appropriate medically
acceptable imaging during a period of stability (not during an
episode of exacerbation of heart failure), with left ventricular end
diastolic dimension equal to or greater than 7.0 cm; or ejection
fraction of 30 percent or less during a period of stability (not
during an episode of acute heart failure); OR
2. Diastolic failure documented by appropriate medically
acceptable imaging during a period of stability (not during an
episode of exacerbation of heart failure), with 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
left atrial volume index (LAVi) greater than or equal to 40 ml, BSA/
m2 (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. 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, or 3:
1. Fractional flow reserve (see 4.00E9e) measurement of less
than or equal to 0.80 of a proximal segment or mid segment coronary
artery not amenable to revascularization (see 4.00E9c(ii)).
2. 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.
3. 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;
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 38864]]
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
40mmHg.
OR
D. Single ventricle (with or without Fontan procedures) (see
4.00H4).
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. 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
2. A medical source (see 4.00I2c) has concluded that the
performance of an exercise tolerance test would present a
significant risk to the person.
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
medical necrosis Marfan syndrome, or trauma), demonstrated by
appropriate medically acceptable imaging, with 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 has not healed
following at least 6 months of prescribed treatment.
4.12 Peripheral arterial 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 systolic blood pressure ratio of less
than 0.50 (see 4.00G7a).
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 systolic blood pressure ratio 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.
* * * * *
5. Amend part B of appendix 1 to subpart P of part 404 by
revising the body system name for section 104.00 in the table of
contents to read as follows:
* * * * *
Part B
* * * * *
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. * * *
b. Cardiovascular disorders result from one or more of four
consequences of heart disease: * * *
(iv) Hypoxemia due to right-to-left shunt, reduced oxygen
concentration in the arterial blood, or pulmonary vascular disease.
* * * * *
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. * * *
b. * * * 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. * * * 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.
* * * * *
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.
* * * * *
2. Why is a longitudinal clinical record important? * * *
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). * * *
3. What if you have not received ongoing medical treatment?
a. * * * In this situation, we will base our evaluation on the
current evidence we have. * * * 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. * * * In rare 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. * * *
(i) If you have had a recent acute event; for example, acute
heart failure.
* * * * *
[[Page 38865]]
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 follow sections 4.00C6, 4.00C7, 4.00C8
in part A, and 104.00B7, when we decide whether to purchase exercise
testing. * * *
* * * * *
7. Will we use exercise tolerance tests (ETT) for evaluating
children with cardiovascular disorders?
a. * * * An ETT may be of value in the assessment of some
arrhythmias, as indicated 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. * * *
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 (CHF)?
a. Heart failure is the inability of the heart to pump enough
oxygenated blood to body tissues. * * * Ejection fraction in heart
failure is a continuum ranging from low ejection fraction due to
muscle dysfunction to preserved ejection fraction resulting from
high intracardiac pressures. We consider heart failure to be chronic
when the condition persists or recurs over time despite treatment.
b. CHF 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 CHF 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, as appropriate.
2. What evidence of CHF do we need?
a. Cardiomegaly or ventricular dysfunction must be present and
demonstrated by appropriate medically acceptable imaging, such as
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
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) * * *
(ii) * * *
(iii) * * * 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.
* * * * *
4. How do we evaluate CHF treated with a mechanical circulatory
support device? We use 104.02D to evaluate CHF 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). Although ECMO is a form of mechanical
circulatory support, we do not include it in 104.02D because ECMO is
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 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. * * *
b. * * *
c. * * *
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 heart failure 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. 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. Some
of the anomalies described as single ventricle include the
following:
(i) Hypoplastic left heart syndrome;
(ii) Hypoplastic right ventricle;
(iii) Tricuspid valve atresia;
(iv) Double inlet left ventricle; and
(v) Some variations of double outlet right ventricle.
E. How do we evaluate arrhythmias?
1. What is an arrhythmia? * * * Although we use the term
``arrhythmia'' in the listings, the term ``dysrhythmia'' may also be
used in the medical evidence to describe this condition.
* * * * *
4. What will we consider when you have an implanted cardiac
defibrillator and you do
[[Page 38866]]
not have arrhythmias that meet the requirements of 104.05?
a. * * * 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. * * * 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.
* * *
* * * * *
F. How do we evaluate other cardiovascular disorders?
1. What is ischemic heart disease (IHD) and how will we evaluate
it in children? * * * If you have IHD, we will evaluate it under
4.04 in part A.
2. How will 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. * * *
3. What is cardiomyopathy and how will we evaluate it?
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 several types: dilated,
hypertensive, hypertrophic, and restrictive.
b. We will evaluate cardiomyopathy under 4.04 in part A, 104.02,
or 104.05, depending on its effects on you.
4. How will we evaluate valvular heart disease? We will 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. * * *
a. After your heart transplant, we will 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.
* * * * *
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 will we evaluate chronic rheumatic fever or rheumatic
heart disease? We will 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 will evaluate these
disorders under 104.02 or 104.05, respectively.
* * * * *
9. * * *
a. * * *
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 medically equals a
cardiovascular disorders listing, such as 4.11 in part A, or a
listing in 101.00. If no listing is met or medically equaled, we
will evaluate any functional limitations imposed by your lymphedema
when we consider whether you have an impairment(s) that functionally
equals the listings.
* * * * *
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 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. * * *
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
[[Page 38867]]
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. Mechanical circulatory support device (except an
extracorporeal membrane oxygenation (ECMO) while hospitalized, at
home, or both (see 104.00C4). Consider under a disability for 12
months 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).
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
104.00D5).
OR
D. For infants under 1 year of age at the time of filing, with
life-threatening congenital heart disease (see 104.00D3c) 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).
[FR Doc. 2022-12980 Filed 6-28-22; 8:45 am]
BILLING CODE 4191-02-P