TN 38 (10-26)

DI 34005.104 Cardiovascular Disorders

104.00 Cardiovascular Disorders (Effective date: 10/30/2026)

A. How do we define cardiovascular disorders and cardiovascular terms?

1. What do we mean by a cardiovascular disorder?

a. We mean any disorder that affects the proper functioning of the heart or the circulatory system (that is, arteries, veins, capillaries, and the lymphatic drainage). The disorder can be congenital or acquired.

b. Cardiovascular disorders result from one or more of four consequences of heart disease:

(i) Chronic heart failure (chronic HF) or ventricular dysfunction.

(ii) Discomfort or pain due to myocardial ischemia, with or without necrosis of the heart muscle.

(iii) Syncope, or near syncope, due to inadequate cerebral perfusion from any cardiac cause, such as obstruction of flow or disturbance in rhythm or conduction resulting in inadequate cardiac output.

(iv) Hypoxemia (reduced oxygen concentration in the blood) due to right-to-left shunt, or pulmonary vascular disease.

c. Disorders of the veins or arteries (for example, obstruction, rupture, or aneurysm) may cause impairments of the lower extremities (peripheral vascular disease), the central nervous system, the eyes, the kidneys, and other organs. We will evaluate peripheral vascular disease under 4.11 or 4.12 in part A, and impairments of another body system(s) under the listings for that body system(s).

2. What do we consider in evaluating cardiovascular disorders? The listings in this section describe cardiovascular disorders based on the medical and other evidence, including response to a regimen of prescribed treatment and functional limitations.

3. What do the following terms or phrases mean in these listings?

a. Medical consultant is a person defined in § 416.1016(a) of this chapter. This term does not include medical sources who provide consultative examinations for us. We use the abbreviation “MC” throughout this section to designate a medical consultant.

b. Persistent means that the longitudinal clinical record shows that, with few exceptions, the required finding(s) has been present, or is expected to be present, for a continuous period of at least 12 months, such that a pattern of continuing severity is established. By “exceptions,” we mean brief periods when the required finding(s) is greatly reduced or gone. These periods are so brief or inconsequential, the required finding(s) remains a factor in the person's condition.

c. Recurrent means that the longitudinal clinical record shows that, within a consecutive 12-month period, the finding(s) occurs at least three times, with intervening periods of improvement of sufficient duration that it is clear that separate events are involved. By “improvement of sufficient duration,” we mean the finding is greatly reduced or not present for long enough that the required finding(s) is no longer a factor in the person's condition.

d. Appropriate medically acceptable imaging means that the technique used is the proper one to evaluate and diagnose the impairment and is commonly recognized as accurate for assessing the cited finding.

e. A consecutive 12-month period means a period of 12 consecutive months, all or part of which must occur within the period we are considering in connection with an application or continuing disability review.

B. What documentation do we need to evaluate cardiovascular disorders?

1. What basic documentation do we need? We need sufficiently detailed reports of history, physical examinations, laboratory studies, and any prescribed treatment and response to allow us to assess the severity and duration of your cardiovascular disorder. A longitudinal clinical record covering a period of not less than 3 months of observations and treatment is usually necessary, unless we can make a determination or decision based on the current evidence we already have.

2. Why is a longitudinal clinical record important? We will usually need a longitudinal clinical record to assess the severity and expected duration of your impairment(s). If you have a listing-level impairment, you probably will have received medically prescribed treatment. Whenever there is evidence of such treatment, your longitudinal clinical record should include a description of the ongoing management and evaluation provided by your medical source(s). It should also include your response to this medical management, as well as information about the nature and severity of your impairment. The record will provide us with information on your functional status over an extended period of time and show whether your ability to function is improving, worsening, or unchanging.

3. What if you have not received ongoing medical treatment?

a. You may not have received ongoing treatment or have an ongoing relationship with the medical community despite the existence of a severe impairment(s). In this situation, we will base our evaluation on the current evidence we have. If you do not receive treatment, you cannot show an impairment that meets the criteria of these listings. However, we may find you disabled because you have another impairment(s) that, in combination with your cardiovascular disorder, medically equals a listing or functionally equals the listings.

b. Unless we can decide your claim favorably on the basis of the current evidence we already have, a longitudinal record is still important. In instances when there is no or insufficient longitudinal evidence, we may purchase a consultative examination(s) to help us establish the existence, severity, and duration of your impairment.

4. When will we wait before we ask for more evidence?

a. We will wait when we have information showing that your impairment is not yet stable and the expected change in your impairment might affect our determination or decision. In these situations, we need to wait to properly evaluate the severity and duration of your impairment during a stable period. Examples of when we might wait are:

(i) If you have had a recent acute event; for example, acute heart failure.

(ii) If you have recently had a corrective cardiac procedure; for example, open-heart surgery.

(iii) If you have started new drug therapy and your response to this treatment has not yet been established; for example, beta-blocker therapy for dilated cardiomyopathy.

b. In these situations, we will obtain more evidence 3 months following the event before we evaluate your impairment. However, we will not wait if we have enough information to make a determination or decision based on all of the relevant evidence in your case.

5. Will we purchase any studies? In appropriate situations, we may purchase studies necessary to substantiate the existence of a medically determinable impairment or to document the severity of your impairment, generally after we have evaluated the evidence we already have. We will not purchase studies involving exercise testing if there is significant risk involved or if there is another medical reason not to perform the test. We will follow sections 4.00C6, 4.00C7, 4.00C8 in part A, and 104.00B7, when we decide whether to purchase exercise testing. We will make a reasonable effort to obtain any additional studies from a qualified medical source in an office or center experienced in pediatric cardiac assessment. (See § 416.919g of this chapter.)

6. What studies will we not purchase? We will not purchase any studies involving cardiac catheterization, such as coronary angiography, arteriograms, or electrophysiological studies. However, if the results of a catheterization are part of the existing evidence we have, we will consider them together with the other relevant evidence. See 4.00C15a in part A.

7. Will we use exercise tolerance tests (ETTs) for evaluating children with cardiovascular disorders?

a. ETTs, though increasingly used, are still less frequently indicated in children than in adults, and can rarely be performed successfully by children under 6 years of age. An ETT may be of value in the assessment of some arrhythmias, and may be considered in 104.05B2. ETTs may also be used in the assessment of the severity of chronic heart failure and in the assessment of recovery of function following cardiac surgery or other treatment.

b. We will purchase an ETT only if we cannot make a determination or decision based on the evidence we have and an MC, preferably one with experience in the care of children with cardiovascular disorders, has determined that an ETT is needed to evaluate your impairment. We will not purchase an ETT if you are less than 6 years of age. If we do purchase an ETT for a child age 12 or younger, it must be performed by a qualified medical source in a specialty center for pediatric cardiology or other facility qualified to perform exercise tests of children.

c. For full details on ETT requirements and usage, see 4.00C3 in part A.

C. How do we evaluate chronic heart failure?

1. What is chronic heart failure (chronic HF)?

a. Heart failure is the inability of the heart to pump enough oxygenated blood to body tissues. This syndrome is characterized by symptoms and signs of pulmonary or systemic congestion (fluid retention) or limited cardiac output. Certain laboratory findings of cardiac functional and structural abnormality support the diagnosis of chronic HF. Heart failure can range from low ejection fraction due to muscle dysfunction to preserved ejection fraction with impaired relaxation of the left ventricle. We consider heart failure to be chronic when the condition persists or recurs over time despite treatment.

b. Chronic HF is considered in these listings as a single category whether due to atherosclerosis (narrowing of the arteries), cardiomyopathy, hypertension, congenital, or other heart disease. If the chronic HF is the result of primary pulmonary hypertension secondary to disease of the lung, we will evaluate your impairment under the listings in 3.00 (for example, 3.09) or 4.00 in part A, as appropriate.

2. What evidence of chronic HF do we need?

a. Cardiomegaly or ventricular dysfunction must be present and demonstrated by appropriate medically acceptable imaging, such as cardiac magnetic resonance imaging (MRI), chest x-ray, echocardiography (M-Mode, 2-dimensional, and Doppler), radionuclide studies, or cardiac catheterization. Other findings on appropriate medically acceptable imaging may include increased ventricular volume, increased pulmonary vascular markings, pleural effusion, and pulmonary edema.

b. Your medical history and physical examination should describe characteristic symptoms and signs of pulmonary or systemic congestion (fluid retention) or of limited cardiac output associated with the abnormal findings on appropriate medically acceptable imaging. When an acute episode of heart failure is triggered by a remediable factor, such as an arrhythmia, dietary sodium overload, or high altitude, cardiac function may be restored and a chronic impairment may not be present.

(i) Symptoms of congestion or of limited cardiac output include easy fatigue, weakness, shortness of breath (dyspnea), cough, feeding intolerance, gastrointestinal distress, or chest discomfort at rest or with activity. Children with chronic HF may also experience shortness of breath on lying flat (orthopnea) or episodes of shortness of breath that wake them from sleep (paroxysmal nocturnal dyspnea). They may also experience cardiac arrhythmias resulting in palpitations, lightheadedness, or fainting. Fatigue or exercise intolerance in an infant may result in prolonged feeding time or tube feeding, often associated with excessive respiratory effort and sweating.

(ii) Other manifestations of chronic HF may include repeated lower respiratory tract infections, wheezing, or growth failure (failure to thrive).

(iii) Signs of congestion may include hepatomegaly, ascites, increased jugular venous distention or pressure, rales, peripheral edema, rapid shallow breathing (tachypnea), or rapid weight gain. However, these signs need not be found on all examinations because congestion may be controlled by prescribed treatment or may not be present at the time of evaluation.

3. How do we evaluate growth failure due to chronic HF?

a. To evaluate growth failure due to chronic HF, we require documentation of the clinical findings of chronic HF described in 104.00C2 and the growth measurements in 104.02C within the same consecutive 12-month period. The dates of clinical findings may be different from the dates of growth measurements.

b. Under 104.02C, we use the appropriate table(s) under 105.08B in the digestive system to determine whether your growth is less than the third percentile.

(i) If you have not attained age 2, we use the weight-for-length table corresponding to your sex (Table I or Table II).

(ii) If you have attained age 2 but have not attained age 18, we use the body mass index (BMI)-for-age table corresponding to your sex (Table III or Table IV).

(iii) BMI is the ratio of your weight to the square of your height. We calculate BMI using the formulas in the digestive disorders body system (105.00).

4. How do we evaluate chronic HF treated with a mechanical circulatory support device? We use 104.02D to evaluate chronic HF treated with an implanted mechanical circulatory support device (MCSD), such as a left ventricle assistive device (LVAD) or a right ventricle assistive device (RVAD). Implanted MCSDs are intended for long-term circulatory support in helping the heart pump blood. For the purposes of 104.02D, an MCSD does not include extracorporeal membrane oxygenation (ECMO) or devices using Impella technology. Although these are forms of mechanical circulatory support, we do not include them in 104.02D because they are intended only for short-term circulatory support (maximum 30 days), used in a setting of imminent or actual cardiac arrest.

D. How do we evaluate congenital heart disease?

1. What is congenital heart disease? Congenital heart disease is any abnormality of the heart or the major blood vessels that is present at birth. Congenital heart disease includes abnormal structure of the individual heart chambers, valves, and blood vessels, and abnormal relative relationship of the chambers to each other that alters the normal pattern of blood flow. Surgery or an interventional catheterization procedure is the usual treatment, and with improving surgical techniques and medical management, more children with congenital heart disease are surviving into adulthood. Examples of congenital heart disease include:

a. Abnormalities of cardiac septation, including atrial or ventricular septal defect or atrioventricular canal;

b. Abnormalities resulting in cyanotic heart disease, including tetralogy of Fallot, transposition of the great arteries, truncus arteriosus, total anomalous pulmonary venous return, or Epstein malformation;

c. Valvular defects with obstructions or regurgitation to ventricular inflow or outflow, including pulmonary or aortic stenosis, pulmonary atresia, or coarctation of the aorta; and

d. Major abnormalities of ventricular development, including hypoplastic left heart syndrome or tricuspid atresia with hypoplastic right ventricle.

2. How do we evaluate conditions associated with congenital heart disease?

a. We will evaluate congenital heart disease that results in chronic HF with evidence of ventricular dysfunction or in recurrent arrhythmias under 104.02 or 104.05, respectively. Otherwise, we will evaluate your impairment under 104.06.

b. We need pulse oximetry measurements documented by medical sources using methods consistent with the prevailing state of medical knowledge and clinical practice to evaluate chronic hypoxemia in congenital heart disease under 104.06A3. These pulse oximetry measurements also must be consistent with the other evidence in the case record.

c. For 104.06D, life-threatening congenital heart disease does not include single ventricle; we evaluate single ventricle physiology separately under 104.06C. When we evaluate life-threatening congenital heart disease under 104.06D, we consider whether it responds to surgical treatment and, therefore, may not meet the 12-month duration requirement. Examples of impairments that in most instances will require life-saving surgery or a combination of surgery and other major interventional procedures (for example, multiple “balloon” catheter procedures) before age 1 include, but are not limited to, the following:

(i) Critical aortic stenosis with neonatal heart failure;

(ii) Critical coarctation of the aorta, with associated anomalies;

(iii) Complete atrioventricular canal defects;

(iv) Transposition of the great arteries;

(v) Tetralogy of Fallot; and

(vi) Multiple ventricular septal defects.

3. What is Eisenmenger syndrome? Eisenmenger syndrome refers to any surgically untreated congenital heart defect with intracardiac communication that over time leads to pulmonary hypertension, reversal of blood flow, and hypoxemia.

a. Lesions in Eisenmenger syndrome, such as large septal defects, are characterized by elevated pulmonary pressures or a high pulmonary flow rate. In response, the pulmonary blood vessels pathologically change, leading eventually to pulmonary hypertension. Development of Eisenmenger syndrome represents a point at which pulmonary hypertension is irreversible and the cardiac lesion is likely inoperable.

b. Examples of congenital heart disease that if untreated may cause pulmonary vascular disease leading to Eisenmenger syndrome include atrial septal defect (ASD), ventricular septal defect (VSD), and large patent ductus arteriosus (PDA). We evaluate Eisenmenger syndrome under 104.06A or 104.06B.

4. What is single ventricle? The term “single ventricle” (also known as “single ventricle physiology” or “functional single ventricle”) describes a diverse group of congenital cardiac anomalies sharing the common feature that only one of the two heart ventricles is adequately developed. At birth, one ventricle must functionally do the work of two, pumping blood for both the body (systemic) and the lungs (pulmonary). Because of this feature, the ultimate plan for cardiac reconstruction is similar for most of these anomalies. People with single ventricle will generally undergo staged reconstructive “Fontan procedures,” ultimately resulting in a “Fontan circulation.” Fontan circulation describes the hemodynamic state in which virtually all systemic venous return-blood passively flows directly into the pulmonary arteries via surgical or catheter-placed shunts, without (the blood) passing through a ventricle. The Fontan circulation results in difficulties augmenting, and sometimes maintaining, cardiac output. Some of the anomalies described as single ventricle include the following:

(a) Hypoplastic left heart syndrome;

(b) Hypoplastic right ventricle;

(c) Tricuspid valve atresia;

(d) Pulmonary atresia with intact ventricular septum;

(e) Double inlet left ventricle; and

(f) Some variations of double outlet right ventricle.

E. How do we evaluate arrhythmias?

1. What is an arrhythmia? An arrhythmia is a change in the regular beat of the heart. Your heart may seem to skip a beat or beat irregularly, very quickly (tachycardia), or very slowly (bradycardia). Although we use the term “arrhythmia” in the listings, the term “dysrhythmia” may also be used in the medical evidence to describe this condition.

2. What are the different types of arrhythmias?

a. There are many types of arrhythmias. Arrhythmias are identified by where they occur in the heart (atria or ventricles) and by what happens to the heart's rhythm when they occur.

b. Arrhythmias arising in the cardiac atria (upper chambers of the heart) are called atrial or supraventricular arrhythmias. Ventricular arrhythmias begin in the ventricles (lower chambers). In general, ventricular arrhythmias caused by heart disease are the most serious.

3. How do we evaluate arrhythmias using 104.05?

a. We will use 104.05 when you have arrhythmias that are not fully controlled by medication, an implanted pacemaker, or an implanted cardiac defibrillator, and you have recurrent episodes of syncope or near syncope. If your arrhythmias are controlled, we will evaluate your underlying heart disease using the appropriate listing. For other considerations when we evaluate arrhythmias in the presence of an implanted cardiac defibrillator, see 104.00E4.

b. We consider near syncope to be a period of altered consciousness, since syncope is a loss of consciousness or a faint. It is not merely a feeling of light-headedness, momentary weakness, or dizziness.

c. For purposes of 104.05, there must be a documented association between the syncope or near syncope and the recurrent arrhythmia. The recurrent arrhythmia, not some other cardiac or non-cardiac disorder, must be established as the cause of the associated symptom. This documentation of the association between the symptoms and the arrhythmia may come from the usual diagnostic methods, including Holter monitoring (also called ambulatory electrocardiography) and tilt-table testing with a concurrent ECG. Although an arrhythmia may be a coincidental finding on an ETT, we will not purchase an ETT to document the presence of a cardiac arrhythmia.

4. What do we consider when you have an implanted cardiac defibrillator and you do not have arrhythmias that meet the requirements of 104.05?

a. Implanted cardiac defibrillators are used to prevent sudden cardiac death in children who have had, or are at high risk for, cardiac arrest from life-threatening ventricular arrhythmias. The largest group of children at risk for sudden cardiac death consists of children with cardiomyopathy (ischemic or non-ischemic) and reduced ventricular function. However, life-threatening ventricular arrhythmias can also occur in children with little or no ventricular dysfunction. The shock from the implanted cardiac defibrillator rescues a child from what may have been cardiac arrest. However, as a consequence of the shock(s), similar to the effects of treatments for other cardiovascular disease, a child may experience psychological distress, which we may evaluate under the listings in 112.00.

b. Most implantable cardiac defibrillators have rhythm-correcting and pacemaker capabilities. In some children, these functions may result in the termination of ventricular arrhythmias without an otherwise painful shock. (The shock is like being kicked in the chest.) Implanted cardiac defibrillators may deliver inappropriate shocks, often repeatedly, in response to benign arrhythmias or electrical malfunction. Also, exposure to strong electrical or magnetic fields, such as from magnetic resonance imaging, can trigger or reprogram an implanted cardiac defibrillator, resulting in inappropriate shocks. We must consider the frequency of, and the reason(s) for, the shocks when evaluating the severity and duration of your impairment.

c. In general, the exercise limitations imposed on children with an implanted cardiac defibrillator are those dictated by the underlying heart impairment. However, the exercise limitations may be greater when the implanted cardiac defibrillator delivers an inappropriate shock in response to the increase in heart rate with exercise, or when there is exercise-induced ventricular arrhythmia.

F. How do we evaluate other cardiovascular disorders?

1. What is ischemic heart disease (IHD) and how do we evaluate it in children? IHD results when one or more of your coronary arteries is narrowed or obstructed or, in rare situations, constricted due to vasospasm, interfering with the normal flow of blood to your heart muscle (ischemia). The obstruction may be the result of an embolus, a thrombus, or plaque. When heart muscle tissue dies as a result of the reduced blood supply, it is called a myocardial infarction (heart attack). Ischemia is rare in children, but when it occurs, its effects on children are the same as on adults. If you have IHD, we evaluate it under 4.04 in part A.

2. How do we evaluate hypertension? Hypertension (high blood pressure) generally causes disability in children through its effects on other body systems, such as the brain, kidneys, or eyes, and we will evaluate these impairments by reference to the specific body system(s) that is affected. We also consider any limitations imposed by your hypertension when we consider whether you have an impairment that functionally equals the listings.

3. What is cardiomyopathy and how do we evaluate it?

a. Cardiomyopathy is a disease of the heart muscle. The heart loses its ability to pump blood (heart failure), and in some instances, heart rhythm is disturbed, leading to irregular heartbeats (arrhythmias). Usually, the exact cause of the muscle damage is never found (idiopathic cardiomyopathy).

b. There are various types of cardiomyopathy, which fall into two major categories: ischemic and nonischemic cardiomyopathy. Ischemic cardiomyopathy typically refers to heart muscle damage that results from coronary artery disease, including heart attacks. Nonischemic cardiomyopathy includes, but is not limited to several types: dilated, hypertrophic, restrictive, and arrhythmogenic.

c. We evaluate cardiomyopathy under 4.04 in part A, 104.02, or 104.05, depending on its effects on you.

4. How do we evaluate valvular heart disease? We evaluate aortic valvular disease under 4.07 in part A. We may also evaluate aortic valvular disease, as well as other forms of valvular disease, under 4.04 in part A, 104.02, 104.05, 104.06, or a listing in 111.00, depending on its effects on you.

5. What do we consider when we evaluate heart transplant recipients?

a. After your heart transplant, we consider you disabled under 104.09 for 1 year following the surgery because there is a greater likelihood of rejection of the organ and infection during the first year. If you develop cardiac allograft vasculopathy after your transplant, we will evaluate this impairment under 104.16.

b. However, heart transplant patients generally meet our definition of disability before they undergo transplantation. We will determine the onset of your disability based on the facts in your case.

c. We will not assume that you became disabled when your name was placed on a transplant waiting list. This is because you may be placed on a waiting list soon after diagnosis of the cardiac disorder that may eventually require a transplant. Physicians recognize that candidates for transplantation often have to wait months or even years before a suitable donor heart is found, so they place their patients on the list as soon as permitted.

d. When we do a continuing disability review to determine whether you are still disabled, we will evaluate your residual impairment(s), as shown by the evidence in your case record, including any side effects of medication. We will consider all evidence indicative of cardiac dysfunction in deciding whether medical improvement (as defined in § 416.994a of this chapter) has occurred.

6. How do we evaluate chronic rheumatic fever or rheumatic heart disease? We evaluate rheumatic fever or rheumatic heart disease under the listing appropriate to its effects on you, which may include heart failure or recurrent arrhythmias. If you have evidence of chronic heart failure or recurrent arrhythmias associated with rheumatic heart disease, we evaluate these disorders under 104.02 or 104.05, respectively.

7. What is hyperlipidemia and how do we evaluate it? Hyperlipidemia is the general term for an elevation of any or all of the lipids (fats or cholesterol) in the blood; for example, hypertriglyceridemia, hypercholesterolemia, and hyperlipoproteinemia. These disorders of lipoprotein metabolism and transport can cause defects throughout the body. The effects most likely to interfere with function are those produced by atherosclerosis (narrowing of the arteries) and coronary artery disease. We evaluate your lipoprotein disorder by considering its effects on you.

8. How do we evaluate Kawasaki disease? We evaluate Kawasaki disease under the listing appropriate to its effects on you, which may include major coronary artery aneurysm or heart failure. A major coronary artery aneurysm may cause ischemia or arrhythmia, which we evaluate under 4.04 in part A or 104.05. We will evaluate chronic heart failure under 104.02.

9. What is lymphedema and how do we evaluate it?

a. Lymphedema is edema of the extremities due to a disorder of the lymphatic circulation; at its worst, it is called elephantiasis. Primary lymphedema is caused by abnormal development of lymph vessels and may be present at birth (congenital lymphedema), but more often develops during the teens (lymphedema praecox). Secondary lymphedema is due to obstruction or destruction of normal lymphatic channels due to tumor, surgery, repeated infections, or parasitic infection such as filariasis. Lymphedema most commonly affects one extremity. Symptoms of lymphedema include but are not limited to swelling in an extremity, changes in skin, and pain or sensory changes in the affected area. The symptoms may limit movement in affected joints.

b. Lymphedema does not meet the requirements of 4.11 in part A, although it may medically equal the listing. We evaluate lymphedema by considering whether the underlying cause meets or medically equals any listing or whether the lymphedema medically equals a cardiovascular disorders listing, such as 4.11 in part A, or a listing in 101.00 or 114.00. If no listing is met or medically equaled, we evaluate any functional limitations imposed by your lymphedema when we consider whether you have an impairment(s) that functionally equals the listings.

10. What is Marfan syndrome and how do we evaluate it?

a. Marfan syndrome is a genetic connective tissue disorder that affects multiple body systems, including the skeleton, eyes, heart, blood vessels, nervous system, skin, and lungs. There is no specific laboratory test to diagnose Marfan syndrome. The diagnosis is generally made by medical history (including family history), physical examination (including an evaluation of the ratio of arm/leg size to trunk size), a slit lamp eye examination, and a heart test(s) (such as an echocardiogram). In some cases, a genetic analysis may be useful, but such analyses may not provide any additional helpful information.

b. The effects of Marfan syndrome can range from mild to severe. In most cases, the disorder progresses as you age. Most people with Marfan syndrome have abnormalities associated with the heart and blood vessels. Your heart's mitral valve may leak, causing a heart murmur. Small leaks may not cause symptoms, but larger ones may cause shortness of breath, fatigue, and palpitations. Another effect is that the wall of the aorta may be weakened and stretch (aortic dilation). This aortic dilation may tear, dissect, or rupture, causing serious heart problems or sometimes sudden death. We evaluate the cardiovascular manifestations of your Marfan syndrome under the appropriate criteria, such as 4.10 in part A, or, if necessary, consider the functional limitations imposed by your impairment.

c. Other genetic connective tissue disorders, such as Loeys-Dietz syndrome or Ehlers-Danlos syndrome, may have abnormalities associated with the heart and blood vessels. We evaluate the cardiovascular manifestations of your genetic connective tissue disorder under the appropriate criteria, such as 4.10 in part A, or, if necessary, consider the functional limitations imposed by your impairment.

11. What is cardiac allograft vasculopathy and how do we evaluate it? Cardiac allograft vasculopathy (CAV) may affect a person who has received a heart transplant and involves thickening in the walls of the coronary arteries that may progress quickly into serious vascular stenosis and heart dysfunction. Stenosis in CAV is caused by a pathological process different from classic atherosclerosis and treatment often is only palliative. We evaluate CAV under 104.16.

G. How do we evaluate other issues that affect the cardiovascular system?

1. How do we consider the effects of obesity when we evaluate your cardiovascular disorder? Obesity is a medically determinable impairment that may be associated with cardiovascular disorders. The additional body mass may make it harder for the chest and lungs to expand or may cause the heart to work harder to pump blood to carry oxygen to the body. The combined effects of obesity with a cardiovascular disorder can be greater than the effects of each of the impairments considered separately. We consider the additional and cumulative effects of obesity when we determine whether you have a severe cardiovascular disorder, a listing-level cardiovascular disorder, a combination of impairments that medically equals the severity of a listed impairment, and when we determine whether your impairment(s) functionally equals the listings.

2. How do we relate treatment to functional status? In general, conclusions about the severity of a cardiovascular disorder cannot be made on the basis of the type of treatment rendered or anticipated. The amount of function restored and the time required for improvement after treatment (medical, surgical, or a prescribed program of progressive physical activity) vary with the nature and extent of the disorder, the type of treatment, and other factors. Depending upon the timing of this treatment in relation to the alleged onset date of disability, we may need to defer evaluation of the impairment for a period of up to 3 months from the date treatment began to permit consideration of treatment effects, unless we can make a determination or decision using the evidence we have. See 104.00B4.

3. How do we consider hospitalizations? The hospitalizations in 104.02E and 104.06E do not all have to be for the same exacerbation or complication of your cardiovascular disorder(s). They may be for three different exacerbations or complications resulting from your cardiovascular disorder. The hospitalizations must be at least 30 days apart, and each one must last at least 48 hours, including hours in a hospital emergency department immediately before the hospitalization.

H. How do we evaluate cardiovascular disorders that do not meet one of these listings?

1. These listings are only examples of common cardiovascular disorders that we consider severe enough to result in marked and severe functional limitations. If your impairment(s) does not meet the criteria of any of these listings, we must also consider whether you have an impairment(s) that satisfies the criteria of a listing in another body system.

2. If you have a severe medically determinable impairment(s) that does not meet a listing, we will determine whether your impairment(s) medically equals a listing. See § 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 § 416.926a of this chapter. We will use the rules in § 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

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

OR

C. Growth failure as required in 1 or 2:

1. For children from birth to attainment of age 2, three weight-for-length measurements that are:

a. Within a consecutive 12-month period; and

b. At least 60 days apart; and

c. Less than the third percentile on the appropriate weight-for-length table under 105.08B1; or

2. For children age 2 to attainment of age 18, three BMI-for-age measurements that are:

a. Within a consecutive 12-month period; and

b. At least 60 days apart; and

c. Less than the third percentile on the appropriate BMI-for-age table under 105.08B2.

OR

D. An implanted mechanical circulatory support device (except an extracorporeal membrane oxygenation (ECMO) while hospitalized, at home, or both (see 104.00C4). Consider under a disability for 1 year from the date of implantation; after that, evaluate any residual impairment(s) under the criteria for the affected body system.

OR

E. Exacerbations or complications of chronic heart failure (see 104.00C1b) requiring three hospitalizations within a consecutive 12-month period and at least 30 days apart. Each hospitalization must last at least 48 hours, including hours in a hospital emergency department immediately before the hospitalization (see 104.00G3).

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. S_aO₂ (arterial oxygen saturation) less than or equal to 89 percent; or

b. PO₂ or P_aO₂ (partial pressure of oxygen) less than or equal to 60 mmHg; or

3. S_pO₂ (percentage of oxygen saturation of blood hemoglobin) measured by pulse oximetry either at rest, or after activity, while breathing room air, less than or equal to 87 percent on three evaluations at least 30 days apart within a consecutive 12-month period (see 104.00A3e).

OR

B. Pulmonary hypertension documented by cardiac catheterization while medically stable, as described in 1, 2, or 3:

1. Pulmonary arterial systolic pressure elevated to at least 70 percent of the systemic arterial systolic pressure; or

2. Pulmonary arterial systolic pressure equal to or greater than 70 mmHg; or

3. Mean pulmonary artery pressure equal to or greater than 40 mmHg.

OR

C. Single ventricle (for example, hypoplastic left or right ventricle) that has or will require Fontan procedures (see 104.00D4).

OR

D. For infants under 1 year of age at the time of filing, with life-threatening congenital heart disease (see 104.00D2c) that will require or already has required surgical treatment in the first year of life, and the impairment is expected to be disabling (because of residual impairment following surgery, or the recovery time required, or both) until the attainment of at least 1 year of age, consider under a disability until the attainment of at least age 1; after that, evaluate impairment severity with the appropriate listing.

OR

E. Exacerbations or complications of congenital heart disease (see 104.00D) requiring three hospitalizations within a consecutive 12-month period (see 104.00A3e) and at least 30 days apart. Each hospitalization must last at least 48 hours, including hours in a hospital emergency department immediately before the hospitalization (see 104.00G3).

104.07-104.08 [Reserved]

104.09 Heart transplantation (see 104.00F5). Consider under a disability for 1 year from the date of the transplant; after that, evaluate the residual impairment(s).

104.10–104.15 [Reserved]

104.16 Cardiac allograft vasculopathy (see 104.00F11), documented by appropriate medically acceptable imaging (for example, intravascular ultrasonography or coronary angiography).