Overview

The Social Security Administration (SSA) asked the Institute of Medicine (IOM) to conduct a 1-year study and make recommendations for improving the cardiovascular disability criteria in the SSA Listing of Impairments. The Listings, as they are known, contain criteria for determining incapacity to work due to the effects of more than 120 adult and 90 childhood diseases and conditions that cause disability. SSA uses the Listings as a screening tool early in the disability determination process to identify applicants (referred to as claimants in this report) for benefits who are so severely impaired that further information about their vocational capacity or other factors, such as age, educational attainment, and work history, is not needed to rule favorably on their claims for disability benefits. This screening device shortens the decision process, which gives claimants a more rapid decision and saves SSA substantial administrative costs.

There are different criteria that are not based on work for children under age 18 in the Supplemental Security Income program. However, as for adults, the Listings for children are meant to identify the most severely impaired claimants, so that SSA does not have to go through the entire disability evaluation process.

Given the role of the Listings in expediting decisions and reducing administrative workload, it is important that they conform to advances in treatment and diagnostic methods as well as to the changing nature of employment in the United States. The Listings should also be consistent, correct, and as easy to use as possible. Accordingly, SSA periodically revises them. To assist in the process of revising the cardiovascular system listings, SSA asked the IOM to form an expert committee to “review the medical



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Overview The Social Security Administration (SSA) asked the Institute of Medicine (IOM) to conduct a 1-year study and make recommendations for improving the cardiovascular disability criteria in the SSA Listing of Impairments. The Listings, as they are known, contain criteria for determining incapacity to work due to the effects of more than 120 adult and 90 childhood diseases and conditions that cause disability. SSA uses the Listings as a screening tool early in the disability determination process to identify applicants (referred to as claimants in this report) for benefits who are so severely impaired that further information about their vocational capacity or other factors, such as age, educational attainment, and work history, is not needed to rule favor- ably on their claims for disability benefits. This screening device shortens the decision process, which gives claimants a more rapid decision and saves SSA substantial administrative costs. There are different criteria that are not based on work for children under age 18 in the Supplemental Security Income program. However, as for adults, the Listings for children are meant to identify the most severely impaired claimants, so that SSA does not have to go through the entire dis- ability evaluation process. Given the role of the Listings in expediting decisions and reducing administrative workload, it is important that they conform to advances in treatment and diagnostic methods as well as to the changing nature of employment in the United States. The Listings should also be consistent, correct, and as easy to use as possible. Accordingly, SSA periodically revises them. To assist in the process of revising the cardiovascular system listings, SSA asked the IOM to form an expert committee to “review the medical 

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 CaRdIoVaSCUlaR dISabIlIty literature to determine the latest standards of care, the latest technology for the understanding of disease processes, and the latest science demonstrat- ing the effect of cardiovascular disorders on patients’ health and functional capacity” and “to make concrete recommendations that are designed to im- prove the utility of the cardiovascular listings for evaluating cardiovascular disability claims by improving the sensitivity and specificity of the listing criteria to identify individuals who meet SSA’s definition of disability” (see Box 1-1 in Chapter 1 for relevant excerpts from the contractual statement of work). The IOM formed a committee of cardiovascular experts (see Appendix A for their biographies). The committee met four times in plenary session. Much of the work was performed by groups of committee members as- signed to address specific cardiovascular conditions. Committee members heard presentations and received position statements from clinical experts and advocacy groups and considered their comments. They reviewed the relevant scientific literature, based their recommendations on existing evi- dence, and drew on the clinical practice guidelines of the American College of Cardiology (ACC) and American Heart Association (AHA). The body of this report contains the committee’s consensus conclusions and recommen- dations concerning the cardiovascular system listings (Chapters 5 through 14). A description of the literature search methodology and an analysis of the ACC/AHA guidelines are found in Appendixes B and C, respectively. SOCIAL SECURITY DISABILITY The Social Security Act defines disability as “the inability to engage in any substantial gainful activity by reason of any medically determinable physical or mental impairment(s) 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.” Under the Supplemental Security Income (SSI) program, a child is “considered disabled if he or she has a medically deter- minable physical or mental impairment or combination of impairments that causes marked and severe functional limitations, and that can be expected to cause death or that has lasted or can be expected to last for a continuous period of not less than 12 months.” The SSA disability standard is not based on a diagnosis or on de- gree of anatomical impairment alone. Rather, it focuses on the functional limitations imposed by one or more impairments and how they affect an individual’s capacity to work given the limitations caused by the medical impairment or impairments and his or her age, education, and work history, or in the case of children, how the impairment or impairments limit age- appropriate activities at home, at school, and in the community. Deciding if someone meets the SSA disability standard by considering these factors

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 oVERVIEW is time and resource intensive. The Listings are a means for SSA to quickly identify claims that would be approved by the full evaluation process with- out having to go through the full process.1 THE LISTINGS AND THE DISABILITY DECISION PROCESS Initially, SSA uses a five-step decision process to determine whether adults are disabled (Figure O-1).2 The steps are followed in a set order, and the analysis stops at any step at which a decision about disability is made. The Listings are applied at the third step. Step 1 The SSA field office determines whether the claimant is engaging in substantial gainful activity.3 If yes, the claimant is found not disabled. If no, the claim is forwarded to the disability determination services (DDS) for evaluation. Step 2 In most states, a DDS team comprising a lay “disability examiner” and a “medical consultant” or “psychological consultant” determines if there is evidence of a severe, medically determinable impairment or combination of impairments.4 If no, the claimant is found not disabled. If a severe impair- ment or impairments are indicated, the analysis proceeds to Step 3. 1 SSA has three additional ways to expedite decisions: (1) flagging TERI (TERminal Illness) cases for expedited processing; (2) using a predictive model to identify QDD (Quick Disability Determination) cases that are highly likely to be allowed and processing them within 20 days; and (3) using CAL (Compassionate ALlowances) to approve cases with certain diagnoses—ei- ther terminal (e.g., gallbladder cancer) or permanently disabling (e.g., mixed dementia). 2 If denied benefits, a claimant may appeal. SSA has several levels of appeal, and the claimant also has the right to appeal in federal court (described in Chapter 2). Although the Listings are used at each level of appeal, for simplicity, this report focuses on the initial determinations made by state agencies called disability determination services (DDSs) that, by law, make the initial determinations for SSA. 3 Substantial gainful activity is generally defined as earning more than a certain monthly amount, which is $1,640 for statutorily blind individuals and $1,000 for nonblind individu- als in 2010. 4 In some DDSs, disability examiners are permitted to make certain disability determinations alone under a pilot program that SSA is conducting.

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 CaRdIoVaSCUlaR dISabIlIty Step 1. Is the individual working and Yes engaging in substantial gainful activity ? Not No disabled Step 2. Does the individual have any No impairment or combination of impairment s that significantly limits his or her physical or mental ability to do basic work activities? Not disabled Yes Step 3. Does the individual have an Yes impairment (s) that meets or medically equals the severity of an impairment in the Listing of Impairments? Disabled No Step 4. Considering the individual’s residual functional capacity (RFC) and Yes the physical and mental demands of the work he or she did in the past, is the Not individual able to perform past relevant work? disabled No Step 5. Considering the individual’s RFC, Yes No age, education, and past work experience, is he or she able to do any other work? Disabled Not disabled FIGURE O-1 Five-step sequential evaluation process for adults. SOURCES: 20 CFR §§ 404.1520 and 416.920. Figure 2-1 and O-1.eps Step 3 The DDS team compares the information in the case file with the List- ings. If the impairment or set of impairments meets all of the requirements of a specific listing, or equals one in severity, the claimant is generally found disabled.5 If not, the analysis proceeds to Step 4. 5 The law also includes a “duration requirement.” The impairment must be expected to result in death or the disability must have lasted or be expected to last for a continuous period of at least 12 consecutive months.

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 oVERVIEW Step 4 The DDS team assesses the claimant’s residual functional capacity (RFC)6 and compares it with the physical and mental demands of work he or she did in the past 15 years. If the claimant is judged able to do past relevant work, he or she is found not disabled. Otherwise, the analysis proceeds to Step 5. Step 5 The DDS team decides whether the claimant is able to do any other work, given his or her RFC, age, education, and past work experience. If the claimant can do other work, he or she is found not disabled. If not, and if the impairment or impairments meet the duration requirement, he or she is found disabled. SSI Children’s Claims Claims of children under SSI go through the first three steps of the deci- sion process. Because the law provides a stricter standard of disability for SSI children based on listing-level severity, there are no steps for children beyond Step 3. However, if a child’s impairment or impairments do not meet or medically equal a listing, the child may still be found disabled at Step 3 based on a finding that the impairment or impairments “function- ally equal the listings.” Unlike the policies of meeting and medically equal- ing listings, functional equivalence does not refer to any specific listings. Rather, the finding is based on limitations of age-appropriate abilities in several functional domains, such as task completion and social interaction (see Chapter 2 for further explanation). This policy is unique to children under the SSI rules. CARDIOVASCULAR DISABILITY TRENDS Currently, there are eight adult cardiovascular system listings and five child-specific listings (Table O-1).7 (If a child has a condition not included in the child-specific cardiovascular listings, e.g., an aortic aneurysm, he or 6 In general, RFC is what the individual can still do in a work setting despite the limitations from all of his or her medical impairments, including any impairments that are not severe. 7 Unless otherwise indicated, the Listings referred to in this summary are contained in the SSA document, disability Evaluation Under Social Security, issued in September 2008 (SSA, 2008a,b). This document contains the current cardiovascular listings and is also available on- line for adults at http://www.ssa.gov/disability/professionals/bluebook/AdultListings.htm and for children at http://www.ssa.gov/disability/professionals/bluebook/ChildhoodListings.htm.

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0 0 CaRdIoVaSCUlaR dISabIlIty TABLE O-1 Current Adult and Child Cardiovascular Listings Adult Child 4.02 Chronic heart failure 104.02 Chronic heart failure 4.04 Ischemic heart disease 4.05 Recurrent arrhythmias 104.05 Recurrent arrhythmias 4.06 Congenital heart disease 104.06 Congenital heart disease 4.09 Heart transplant 104.09 Heart transplant 4.10 Aneurysm of aorta or major branches 4.11 Chronic venous insufficiency 4.12 Peripheral arterial disease 104.13 Rheumatic heart disease SOURCES: SSA, 2008a (for adults), 2008b (for children). she is evaluated using the adult Listings criteria.) The cardiovascular listings were last updated in 2006. In calendar year 2009, SSA allowed approximately 64,000 adult claims for cardiovascular disability. Of these, 15,000 (24 percent) were allowed based on impairments that met or medically equaled listings. Chronic heart failure (CHF) accounted for 42 percent of the listings-based allowances in 2009, followed by chronic venous insufficiency (21 percent) and ischemic heart disease (17 percent). In 2009, SSA also allowed approximately 3,500 child claims for cardio- vascular disability; most (82 percent) were for meeting or medically equal- ing a listing and the remainder (18 percent) were for functionally equaling a listing. Most of the child allowances (93 percent) were for a congenital heart condition or conditions. APPROACHES TO REVISING THE CARDIOVASCULAR LISTINGS Before summarizing the major conclusions and recommendations re- garding each of the cardiovascular listings, it is helpful to review issues that apply generally to all listings and that explain some commonalities in the recommendations. These include the criteria for evaluating the specific list- ings, the trade-offs between sensitivity and specificity inherent in designing a screening tool like the Listings, the limited predictive capacity of most clinical factors, and the safety of exercise tests. The general approach taken by the committee was to use the follow- ing algorithm to arrive at listing-level criteria that are the most objective and the easiest to administer, while maintaining a reasonable degree of accuracy.

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 oVERVIEW For each type of cardiovascular impairment: 1. Determine if there is a diagnosis that by itself indicates either (A) a terminal illness of short duration or (B) a condition so severely incapacitating that it is nearly always work disabling. If one exists, make it a sufficient requirement for a listing-level allowance (also consider recommending it as a compassionate allowance if it is not already designated as one). 2. If the diagnosis is not sufficient by itself to be a listing-level cri- terion, determine if there is an accepted measure of impairment severity shown to have a high degree of association with incapac- ity to work or with very serious functional limitations that would seem to preclude work. If there is, make that degree of impairment severity a sufficient requirement (given the diagnosis) for a listing- level allowance. 3. If the diagnosis and/or severity measure or measures are insufficient to accurately differentiate disabled from nondisabled claimants, re- quire evidence that the claimant is seriously functionally limited. Limitations of the Listings as a Screening Tool The Listings are used to determine if a claimant is able to engage in any gainful activity, that is, perform any work for pay or profit. In fact, the work capacity of a population of claimants with a given impairment will vary on a continuum from no work to high work capacity. Along this continuum, the line between having no work capacity and some work capacity can be difficult to draw. This uncertainty results in classification errors, that is, false negatives (claimants denied who should be allowed) and false positives (claimants allowed who should have been denied). As with other diagnostic screening tools, tightening the criteria reduces false posi- tives (thus increasing specificity), but it also increases the likelihood of false negatives (thus reducing sensitivity). Loosening the criteria will reduce false negatives (thus allowing a larger percentage of true positives to be allowed quickly), but it will also increase the number of false positives. SSA wants to keep the false-positive rate low, while maintaining the sensitivity of the Listings. The greater the sensitivity of a listing, the greater the percentage of Step 3 allowances that is made for those truly unable to work. Therefore, in formulating recommendations to improve the Listings, the committee focused on the trade-off between sensitivity and specificity and sought ways to increase sensitivity without unduly reducing specificity. The only way to increase both sensitivity and specificity is to find or develop a better discriminator between true positives and true negatives, which is a research goal discussed below.

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  CaRdIoVaSCUlaR dISabIlIty Limited Predictive Ability of Clinical Factors Objective clinical factors are limited in their ability to predict the func- tional capacity of an individual with an impairment or impairments. These limitations make it challenging to balance the sensitivity and specificity of the Listings criteria. For example, few individuals with a left ventricular ejection fraction (LVEF) of 30 percent or less are able to engage in usual daily activities, including working in an office, but some could. The latter would be false positives using the 30 percent criterion. Meanwhile, some individuals with an ejection fraction greater than 30 percent are totally incapacitated in the same circumstances. They would be the false negatives using this criterion. This imprecision of clinical measures of impairment in determining disability led the committee to recommend that evidence of functional limitations be required in addition to clinical criteria to meet most of the cardiovascular listings. Maximizing the Use of Exercise Testing The introductory section of the cardiovascular system listings highlights the extensive use of exercise tests to determine functional capacity in pa- tients with ischemic heart disease, CHF, and peripheral artery disease. The text also indicates that SSA might purchase an exercise test if needed to determine whether a claimant’s impairment or impairments meet or equal a cardiovascular listing; however, the current Listings are based on an overly strict assessment of the risk associated with the performance of these tests. In fact, an extensive body of research shows that exercise testing is safe in most cases and much safer than is assumed in the current Listings. The committee encourages SSA to revise the cardiovascular listings to conform to the current understanding of the safety of exercise tests. Chap- ters 4, 5, 7, and 8 provide further detail on the relevant medical literature on the safety of exercise testing. SUMMARY OF CONCLUSIONS AND RECOMMENDATIONS Most of this report consists of detailed chapters on specific cardio- vascular conditions that are already in the Listings or should be added. The chapters are summarized below, each followed by the committee’s full recommendations.

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 oVERVIEW Heart Failure, Cardiomyopathy, and Right Heart Failure Heart failure (HF) occurs when the heart is unable to supply sufficient oxygenated blood to body tissues to meet systemic aerobic requirements or when the heart can only do so with elevated pressures that eventually damage it. The primary manifestations of HF are fatigue, dyspnea, and pe- ripheral fluid retention, which alone or in combination may limit a patient’s ability to perform activities and potentially lead to disability. Most patients do not detect any functional limitation until their ejec- tion fraction is less than 40 percent. A few patients can live virtually normal lives with an ejection fraction as low as 20 percent, but most with disease this advanced have major limitation of activity. The most direct assessment of ability to work is the ability to perform physical exertion. A patient’s ability to perform activities of daily living, work, and vigorous exertion requires the integration of cardiovascular, pul- monary, and circulatory systems. Since the current cardiovascular listings were issued in 2006, exercise testing has been consistently demonstrated and widely accepted to be practical and safe for most patients with chronic HF. The committee also recommends revising the chronic HF listing to include separate evaluation criteria for three related conditions: chronic HF, hyper- trophic cardiomyopathy, and right HF. The current listing requires both an objective cardiac abnormality and a functional limitation, a construct that should be retained. In addition, the committee recommends adding a listing that can be met by having a left ventricular ejection fraction of 20 percent or less without having to demonstrate a functional limitation. RECOMMENDATION 5-1. Retain the current framework of listing 4.02 chronic heart failure, requiring both (A) an objective cardiac abnormality, and (B) a functional limitation. This framework would apply to each of the following: systolic heart failure, diastolic heart failure, hypertrophic cardiomyopathy, and right heart failure. Systolic Heart Failure The criteria for systolic heart failure should include: A. An objective cardiac abnormality demonstrated by a left ven- tricular ejection fraction of 30 percent or less (or an ejection fraction of a systemic ventricle in congenital heart disease with- out an anatomic, systemic left ventricle), or a left ventricular diameter of 7 cm or greater, AND

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4 4 CaRdIoVaSCUlaR dISabIlIty B. A functional abnormality demonstrated by one of three criteria: 1. Exercise testing (see Recommendation 5-2); or 2. Cardiologist-assessed excessive risk of exercise testing and cardiac limit to activities of daily living; or 3. Three hospitalization-equivalent events in the past 12 months. Diastolic Heart Failure The criteria for diastolic heart failure should include: A. An objective cardiac abnormality demonstrated by moderate or se- vere diastolic dysfunction, usually indicated by echocardiography, AND B. A functional abnormality can be demonstrated by one of three criteria: 1. Exercise testing (see Recommendation 5-2); or 2. Cardiologist-assessed excessive risk of exercise testing and cardiac limit to activities of daily living; or 3. Three hospitalization-equivalent events in the past 12 months. Hypertrophic Cardiomyopathy The criteria for hypertrophic cardiomyopathy should include: A. An objective cardiac abnormality demonstrated by a left ven- tricular or septal wall thickness greater than 15 mm in the ab- sence of another known cause for left ventricular hypertrophy (e.g., hypertension, aortic valve disease), AND B. A functional abnormality demonstrated by one of three criteria: 1. Exercise testing (see Recommendation 5-2); or 2. Cardiologist-assessed excessive risk of exercise testing and cardiac limit to activities of daily living; or 3. Three hospitalization-equivalent events in the past 12 months.

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 oVERVIEW Right Heart Failure The criteria for right heart failure should require both an objective cardiac diagnosis implicated as a cause of right heart failure and clini- cal evidence of functional limitation manifest as severe systemic venous congestion: A. An objective cardiac abnormality demonstrated by one of the following: 1. Congenital heart disease; or 2. Pulmonary hypertension; or 3. Right ventricular enlargement or dysfunction. AND B. Functional abnormalities meeting criteria for right heart failure: 1. Systemic venous congestion despite chronic diuretic ther- apy, assessed twice with at least 3 months in between, causing either • Peripheral edema to the knee or above; or • Severe ascites documented by abdominal imaging study. RECOMMENDATION 5-2. Revise the exercise criteria to reflect the current acceptability of exercise testing as safe in heart failure and the objective measurements that can now be performed during exercise test- ing. Limitation is defined as a standard treadmill test (or bicycle test) performed at a workload equivalent to one of the following criteria: • Less than 15 ml/kg/min peak VO2/(oxygen consumption) on cardiopulmonary exercise test; or • Less than 5 metabolic equivalents of task if using standard treadmill test without gas exchange. RECOMMENDATION 5-3. Add an additional listing route in which the objective cardiac abnormality of a left ventricular ejection fraction of 20 percent or less, documented twice with at least 3 months inter- vening, is sufficiently severe that demonstration of functional limitation is not needed to meet the listing.

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4 4 CaRdIoVaSCUlaR dISabIlIty RECOMMENDATION 10-4. Children from 6 months after definitive cardiac surgery until their 12th birthday and children from birth on- ward for whom surgery is not indicated, with congenital heart disease documented by appropriate medically acceptable imaging or cardiac catheterization, with one of the following criteria should be considered disabled: A. Cyanotic heart disease, with persistent, chronic hypoxemia as manifested by: 1. Hematocrit of 55 percent or greater; or 2. Arterial O2 saturation of less than 90 percent in room air, or resting arterial PO2 of 60 Torricelli or less; or 3. Hypercyanotic spells, syncope, characteristic squatting, or other incapacitating symptoms directly related to docu- mented cyanotic heart disease; or 4. Exercise intolerance with increased hypoxemia on exertion measured by pulse oximetry. OR B. Secondary pulmonary vascular obstructive disease with pulmo- nary arterial systolic pressure elevated to at least 70 percent of the systemic arterial systolic pressure determined by echocar- diography or right heart catheterization; OR C. Symptomatic acyanotic heart disease interfering seriously with the ability to independently initiate, sustain, or complete activities; OR D. Chronic heart failure manifested by: 1. Persistent tachycardia at rest (see Table I8); or 8 See Table 1, Section 104.02, Chronic Heart Failure. For tachycardia at rest, apical heart rate: under age 1, 150 beats per minute; ages 1 through 3, 130 beats per minute; ages 4 through 9, 120 beats per minute; ages 10 through 15, 110 beats per minute; and over age 15, 100 beats per minute (SSA, 2008b).

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 oVERVIEW 2. Persistent tachypnea at rest (see Table II9); or 3. Markedly decreased exercise tolerance; or 4. Growth disturbance with: a. An involuntary weight loss or failure to gain weight at an appropriate rate for age, resulting in a fall of 15 percentiles from an established growth curve (on the cur- rent Centers for Disease Control and Prevention [CDC] growth chart), which is currently present (see 104.00A3f) and has persisted for 2 months or longer; or b. An involuntary weight loss or failure to gain weight at an appropriate rate for age, resulting in a fall to below the third percentile from an established growth curve (on the current CDC growth chart), which is currently present (see 104.00A3f) and has persisted for 2 months or longer. RECOMMENDATION 10-5. Children age 12 and older should be considered disabled if they have congenital heart disease documented by appropriate medically acceptable imaging or cardiac catheterization, with one of the following criteria: A. Cyanosis at rest, and: 1. Hematocrit of 55 percent or greater; or 2. Arterial O2 saturation of less than 90 percent in room air, or resting arterial PO2 of 60 Torricelli (Torr) or less. OR B. Intermittent right-to-left shunting resulting in cyanosis on ex- ertion (e.g., Eisenmenger’s physiology) as determined by pulse oximetry and with arterial PO2 of 60 Torr or less or pulse oximetry 85 percent or less at a workload equivalent to 5 metabolic equivalents of task (METs) or less; OR 9 See Table II, Tachypnea at Rest, Section 104.02, Chronic Heart Failure. For tachypnea at rest, respiratory rate, under age 1, over 40 breaths per minute; ages 1 through 5, over 35 breaths per minute; ages 6 through 9, over 30 breaths per minute; and over age 9, over 25 breaths per minute (SSA, 2008b).

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  CaRdIoVaSCUlaR dISabIlIty C. Secondary pulmonary vascular obstructive disease with pulmo- nary arterial systolic pressure elevated to at least 70 percent of the systemic arterial systolic pressure determined by echocar- diography or cardiac catheterization; OR D. Single ventricle, including hypoplastic left heart syndrome, double inlet left ventricle, and Fontan operation for single ventricle; OR E. Chronic heart failure manifested by: 1. Exercise capacity with maximal oxygen consumption less than 15 ml/kg/min or work load less than 5 METs; or 2. Three hospitalizations or emergency room visits with use of intravenous medications for heart failure management in 1 year; or 3. Evidence of right heart failure manifested by: a. Symptoms of dyspnea, edema, or exercise intolerance; and b. Jugular venous distension, hepatomegaly, ascites, and/or dependent edema on three clinic visits in 1 year. RECOMMENDATION 10-6. Adults with a medically confirmed diag- nosis of congenital heart disease should be considered disabled if they also demonstrate one of the following: A. Cyanosis at rest, and: 1. Hematocrit of 55 percent or greater; or 2. Arterial O2 saturation of less than 90 percent in room air, or resting arterial PO2 of 60 Torricelli (Torr) or less; OR B. Intermittent right-to-left shunting resulting in cyanosis on ex- ertion (e.g., Eisenmenger’s physiology) as determined by pulse oximetry and with arterial PO2 of 60 Torr or less or pulse oximetry 85 percent or less at a workload equivalent to 5 metabolic equivalents of task (METs) or less;

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 oVERVIEW OR C. Secondary pulmonary vascular obstructive disease with pulmo- nary arterial systolic pressure elevated to at least 70 percent of the systemic arterial systolic pressure determined by echocar- diography or right heart catheterization; OR D. Single ventricle including hypoplastic left heart syndrome, double inlet left ventricle, and Fontan operation for single ventricle; OR E. Diagnosis of congenital heart disease and chronic heart failure manifested by: 1. Exercise capacity with maximal oxygen consumption less than 15 ml/kg/min or work load less than 5 METs; or 2. Three hospitalizations or emergency room visits with in- travenous medication administration for heart failure man- agement in 1 year; or 3. Evidence of right heart failure manifested by: a. Symptoms of dyspnea, edema, or exercise intolerance; and b. Jugular venous distension, hepatomegaly, ascites, and dependent edema on three clinic visits in 1 year. Pulmonary Hypertension Pulmonary hypertension (PH) is present when the pulmonary artery pressures are elevated above normal. The term applies particularly to dis- eases that affect the small pulmonary arteries and markedly increase their resistance to blood flow. PH may be of unknown cause (idiopathic PH) or secondary to conditions such as connective tissue disease, sarcoidosis, an autoimmune disorder, congenital heart disease, and other inherited condi- tions. PH may also develop secondary to lung disease or hypoxemia, such as chronic obstructive pulmonary disease, interstitial lung disease, and obstructive sleep apnea. Pulmonary embolism and left-sided heart disease are common causes of PH. PH is associated with high mortality and marked functional limitations.

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  CaRdIoVaSCUlaR dISabIlIty The condition is often quite advanced at the time of diagnosis, largely be- cause PH symptoms are nonspecific and the diagnosis is difficult to make. Patients with PH show marked limitations on standard functional status measures and show evidence of reduced functional capacity on standard HF measures. PH patients with pulmonary artery hypertension are advised to avoid heavy physical exertion or isometric exercise, which may lead to loss of consciousness. Patients with PH may be unable to work either because of functional limitations or their use of continuous intravenous treatment. There is no current cardiovascular listing for PH. There is a listing for one type of PH, cor pulmonale, in the respiratory system listings. The committee recommends that SSA establish a new listing for PH in the car- diovascular system listings. RECOMMENDATION 11-1. The Social Security Administration should establish a new listing in the cardiovascular system for pul- monary hypertension. The new listing should allow claimants with pulmonary hypertension documented by right heart catheterization to meet the listing if (A) there is evidence of severe pulmonary hyperten- sion OR (B) there is evidence of moderate pulmonary hypertension AND of marked functional limitations. A. Evidence of severe pulmonary hypertension, which is associ- ated with severe functional limitation, includes any of the following: • Mean pulmonary artery pressure of 40 mm Hg or greater; or • Pulmonary vascular resistance of 6 Wood units (mm Hg per liter per minute) or greater; or • Continuous parenteral therapy with prostacyclin analogs. OR B. Evidence of moderate pulmonary hypertension, which imposes severe functional limitation on many but not all individuals, includes any of the following: • Recurrent syncope secondary to pulmonary hypertension; or • Right heart failure (same criteria as for heart failure list- ing); or • Mean pulmonary artery pressure between 25 and 39 mm Hg; or

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 oVERVIEW • Pulmonary vascular resistance above 3 and below 6 Wood units. AND Evidence of marked functional disability provided by either of the following: • An exercise capacity of less than 5 metabolic equivalents of task; or • Three or more hospital admissions within a consecutive 12-month period to treat right heart failure or pulmonary hypertension. Valvular Heart Disease Valvular heart disease (VHD) is characterized by damage to or a con- genital defect in one or more heart valves. Damaged or defective valves can cause two types of problems: either they fail to open properly (a condition called stenosis), impeding blood flow, or they leak (a condition called re- gurgitation), permitting backward blood flow. Valve conditions may be congenital, arise from inflammation, or occur due to complications from infections. Mild or moderate valve disease is usually asymptomatic at first but, due to the progressive and degenerative nature of the disease, may eventually become severe and may lead to heart failure and death if left untreated. Symptoms of VHD in decreasing fre- quency include shortness of breath or dyspnea, chest pain and palpitations, syncope, or near syncope. The committee recommends establishing a listing for severe symptom- atic aortic stenosis, which is usually fatal within 5 years unless the valve is replaced and could cause sudden death from the exertion of manual labor. Determining disability using measurements of functional criteria is not advised for individuals with severe symptomatic aortic stenosis, be- cause there is great risk to the patient in performing exercise tests. Severe symptomatic mitral stenosis, aortic regurgitation, or mitral regurgitation may also warrant disability at the listing level, but these patients must demonstrate functional limitation in addition to an objective diagnosis of severity. RECOMMENDATION 12-1. Provide a listing-level pathway to dis- ability for symptomatic claimants with objective evidence via echocar- diogram or other appropriate medically acceptable imaging of severe aortic stenosis, characterized by mean gradient greater than 40 mm Hg,

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0 0 CaRdIoVaSCUlaR dISabIlIty jet velocity greater than 4.0 m/sec, valve area less than 1.0 cm2, and valve area index less than 0.6 cm2/m2. RECOMMENDATION 12-2. Provide a listing-level pathway to dis- ability for symptomatic claimants with objective evidence via echocar- diogram or other appropriate medically acceptable imaging of severe mitral stenosis, aortic regurgitation, or mitral regurgitation and dem- onstrated functional limitation. Objective evidence is measured by one of the following: • Severe mitral stenosis characterized by mean gradient greater than 10 mm Hg, pulmonary artery systolic pressure greater than 50 mm Hg, and valve area less than 1.0 cm2; or • Severe aortic regurgitation characterized by regurgitant volume greater than or equal to 60 ml/beat, and regurgitant orifice area greater than or equal to 50 cm2, and increased left ven- tricular size; or • Severe mitral regurgitation characterized by regurgitant volume greater than or equal to 60 ml/beat or regurgitant fraction greater than or equal to 50 percent, and regurgitant orifice area greater than or equal to 0.40 cm2, and enlarged left atrial size and enlarged left ventricular size. Functional limitation(s) from severe mitral stenosis, aortic regurgita- tion, or mitral regurgitation would be demonstrated by one of the following: • Three hospitalizations with heart failure in 12 months; or • Inability to achieve 5 metabolic equivalents of task on an ex- ercise test; or • Objective evidence of right heart failure. Arrhythmias The current listing for arrhythmia is met if the claimant has recurrent arrhythmias that cause syncope or near syncope that is not reversible and does not respond to prescribed treatment. The link between arrhythmia and syncope or near syncope must be documented on an electrocardiogram. The committee recommends that the listing define listing-level arrhyth- mias as tachycardia or bradycardia, because these are the arrhythmias that are potentially incapacitating and should be documented in the medical record. Furthermore, because episodes of near syncope due to arrhyth- mia are difficult to document and because there can be other severe and

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 oVERVIEW disabling symptoms of arrhythmia, the committee recommends that the listing be met if the symptoms of arrhythmia seriously limit the ability of the claimant to independently initiate, sustain, or complete activities of daily living. RECOMMENDATION 13-1. Revise listing 4.05 to define arrhythmias as recurrent episodes of tachycardia or bradycardia documented by electrocardiography or other appropriate medically acceptable testing; that cause cardiac syncope, near syncope, or other debilitating symp- toms; are not due to a reversible cause; do not respond to prescribed treatment; and very seriously limit the ability to independently initiate, sustain, or complete activities of daily living or instrumental activities of daily living. Aneurysm or Dissection of the Aorta and Peripheral Arteries Aneurysms can impinge on adjacent body structures as they enlarge, causing symptoms such as breathlessness, wheezing, cough, tracheal devia- tion, or pain. Aortic dissections can impede the flow of blood to an adjacent branch peripheral artery and cause ischemia in affected organs or other tissues. According to the ACC/AHA guidelines for thoracic aortic disease: “For patients with a current thoracic aortic aneurysm or dissection, or previously repaired aortic dissection, employment and lifestyle restrictions are reasonable, including the avoidance of strenuous lifting, pushing, or straining,” although aerobic (but not isometric) exercise is considered to be reasonably safe. The current listing for aneurysm is met if a clamant has an aneurysm from any cause with dissection that cannot be controlled by treatment. SSA considers the dissection uncontrolled if there is persistent chest pain due to progression of the dissection; an increase in the size of the aneurysm; or compression of one or more branches of the aorta supplying the heart, kidneys, brain, or other organs. The committee recommends that the listing distinguish between aneu- rysm and dissection because one may occur without the other. In place of the requirement for dissection that is not controlled by treatment, the list- ing should require that the aneurysm or dissection cause chronic debilitat- ing symptoms as a result of its effects on the function of the heart, brain, peripheral nerves, or limbs despite treatment. In patients with a genetic predisposition to aneurysm formation or aortic dissection, such as Marfan syndrome, the listing should accept that standard medical management may prescribe substantial restrictions in physical activity in adults and children with asymptomatic aortic aneurysms or dissections, which may affect em- ployment possibilities.

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  CaRdIoVaSCUlaR dISabIlIty RECOMMENDATION 14-1. Revise listing (4.10) to require the pres- ence of chronic disabling symptoms due to the aneurysm or dissection. Disabling symptoms may be the result of the functional impairment to the heart, brain, peripheral nerves, or limbs due to the aneurysm or dissection. Claimants should be evaluated under the appropriate related cardiovascular listings or listings for other body systems if necessary. RECOMMENDATION 14-2. Revise the introductory text to the car- diovascular system to account for the following changes: • Include the term dissection in the primary description of the condition (i.e., aneurysm or dissection); • Develop the definitions for aneurysm and dissection to include: An aneurysm is a bulge in the aorta or a peripheral artery. A dissection of the aorta or its branches occurs when the inner lining of the artery is “torn” and begins to separate from the rest of the arterial wall. An aneurysm or dissection may com- promise organ function and produce symptoms by the com- pression of other structures in the tissue or body compartment or induce ischemia by compromising the flow of blood to the heart, kidneys, brain, or other organs; • Revise the effects of aneurysm or dissection to include: An aneurysm or dissection can cause heart failure, renal (kidney) failure, or neurological complications. If an aneurysm or dis- section is present, there must be one or more of these associ- ated symptomatic conditions; the condition(s) are evaluated using the appropriate associated listings; and • Revise the diagnostic criteria of Marfan syndrome to include: There is no specific laboratory test to diagnose Marfan syn- drome, although the mutation in the gene that causes it has been defined. The diagnosis is generally made by medical his- tory, including family history and physical examination includ- ing an evaluation of the musculoskeletal features, a slit-lamp eye examination, and a heart test(s), such as an echocardio- gram. In some cases, a genetic analysis may be useful, but such analyses may not provide any additional helpful information. Include a description of Loeys-Dietz syndrome as another ex- ample of a genetic disorder with increased risk of aortic aneu- rysm and/or dissection affecting both children and adults.

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 oVERVIEW COMORBIDITIES Many individuals with a severe cardiovascular impairment, such as heart failure, coronary artery disease, and peripheral vascular disease, have comorbid conditions that further reduce their capacity to work. For exam- ple, major depressive disorders often affect individuals with cardiovascular conditions, with depression present in up to 20 percent of patients with cardiac conditions. Depression has a significant, negative impact on the overall functional capacity of affected individuals and increases morbidity and mortality. Other prevalent conditions that combine with cardiovascular disease to increase functional limitations include diabetes mellitus, chronic obstructive pulmonary disease and other respiratory diseases, and obesity. The committee has no recommendations for changing SSA’s current policies for dealing with multiple impairments, none of which meets a cardiovascular listing by itself, but which might combine in such a way that the claimant equals the Listings. How to evaluate the effect of comor- bidities in applying the cardiovascular listings is based on judgment of the adjudicators that cannot be reduced to a formula. The committee intends the information in this chapter to underline the effect of comorbidities and to better inform those decisions. FUTURE DIRECTIONS FOR IMPROVING THE LISTINGS In the course of its work, the committee encountered a number of knowledge gaps in evaluating the effectiveness of the cardiovascular list- ings, such as the relationship of anatomical severity measures and func- tional limitation and the effect of comorbidities. Additional research would reduce information gaps and improve listing quality. To better inform the next revisions of any body system within the Listings, the committee encourages SSA to support a full and balanced program of in-house and external research in four areas: policy implications, programmatic issues, correlation of impairments with functional limitations, and the underlying prevalence of impairments and disability in the population. RECOMMENDATION 16-1. SSA should plan and sponsor a balanced program of research to improve the reliability, validity, and utility of the Listings in four areas: policy implications, programmatic issues, correlation of impairments and impairment severity with functional limitations related to work capacity, and the underlying prevalence of and trends in impairments in the population. This program would also enable SSA to enhance the other steps of the disability determination process.

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4 4 CaRdIoVaSCUlaR dISabIlIty REFERENCES SSA (Social Security Administration). 2008a. listing of impairments—adult listings (Part a). disability evaluation under Social Security (blue book). http://www.socialsecurity. gov/disability/professionals/bluebook/AdultListings.htm (accessed July 22, 2010). SSA. 2008b. listing of impairments—Childhood listings (Part b). disability evaluation under Social Security (blue book). http://www.socialsecurity.gov/disability/professionals/blue book/ChildhoodListings.htm (accessed July 22, 2010).