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10 Congenital Heart Disease this chapter includes a recommendation that evaluation for dis- ability due to congenital heart disease be divided into four age groupings consistent with the changed timing of surgery for these defects and the developmental capacities of these age groups; criteria for evaluating functional impairment for each age group; a recommendation that one form of congenital heart disease of great severity meet the listing without additional evaluation of functional limitation; and a recommendation that all persons with congenital heart disease being evaluated for disability have infor- mation included in their applications about comorbidities, specifi- cally any learning disability, cognitive impairment, depression, and anxiety. DESCRIPTION Congenital heart disease is an umbrella term that covers all heart de- fects present at birth, including dozens of defects that may occur singly or in combination. The abnormal structure of the cardiac chambers, valves, or great vessels in patients with congenital heart disease alters the normal pat- tern of blood flow. Additionally, individuals with congenital heart disease may develop cardiac complications such as arrhythmias, heart failure, and valve insufficiency, even after surgical correction of the structural abnor- malities. In the 1980s, survival to 1 year of age of all individuals born with congenital heart defects was 81 percent (Ferencz et al., 1993), but by 2003 this had improved to 92 percent (Tennant et al., 2010). 

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  CaRdIoVaSCUlaR dISabIlIty Surgery is the mainstay of treatment for congenital heart disease, and the expected results can be classified as follows: • Curative: Patients with these conditions rarely have long-term se- quelae after surgical correction in childhood. These conditions are patent ductus arteriosus, secundum atrial defect, and uncompli- cated ventricular septal defect. • Reparative: Patients with these defects are improved after correc- tive surgery, yet have lifelong sequelae, and some proportion will have significant late impairment. These defects are aortic stenosis, atrioventricular canal, coarctation of the aorta, partial anomalous pulmonary venous return, pulmonary stenosis, tetralogy of Fallot, total anomalous pulmonary venous return, d-transposition of the great arteries, and l-transposition (also called congenital corrected transposition of the great arteries). • Palliative: Surgery in these patients (if done) does not fully correct the underlying defect, so they are likely to have significant lifelong impairment of function. These defects are Eisenmenger syndrome, hypoplastic left heart syndrome, malaligned atrioventricular canal with single ventricle repair, single ventricle, tricuspid atresia, and unrepaired cyanotic heart disease. EPIDEMIOLOGY Eight out of 1,000 infants are born with congenital heart disease. Of these, approximately 25 percent require immediate surgical or catheter- based intervention (Ferencz et al., 1993). Even with treatment, the lifespan of individuals with congenital heart disease is limited compared with their peers; 89.5 percent of individuals with congenital heart disease are alive at age 20, but for some diagnoses (e.g., truncus arteriosus and single ventricle), the survival is much poorer (Tennant et al., 2010). Survival in individuals with congenital heart disease who reach adulthood is reduced (Verheugt et al., 2010). Death is commonly due to heart failure or sudden death, and risk is increased by arrhythmia, endocarditis, myocardial infarction, and pulmonary hypertension. In 2000, 1 million adults in the United States were living with congeni- tal heart disease. An estimated half of them had relatively simple residual disease, one quarter had moderately complex residual disease, and one quarter had severe residual disease (Warnes et al., 2001). However, func- tional disability is not limited to those with “severe” disease, as even an individual with “simple” disease may be disabled due to a complication.

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 CongEnItal hEaRt dISEaSE DIAGNOSTIC CRITERIA AND METHODS The diagnosis of congenital heart disease is made by physical exami- nation, echocardiography, magnetic resonance imaging, cardiac computed tomography, cardiac catheterization, and open-heart surgery. The ICD-9 diagnosis codes indicating congenital heart disease fall primarily between 745.0 and 747.9. Distinguishing among the various congenital heart disease defects can be difficult, and accurate diagnosis can require special expertise and training. Children with congenital heart disease are best evaluated, when possible, in pediatric cardiology centers and by echocardiographic centers certified in congenital heart disease. Adults with congenital heart disease are best evaluated by specialized centers devoted to the evaluation and treatment of such adults. These centers are recommended for their ongoing care (Warnes et al., 2008). CONSIDERATION OF NONCARDIAC CONGENITAL ANOMALIES The evaluation of disability status in individuals with congenital heart disease is further complicated by the presence of concomitant noncardiac congenital abnormalities in about 20 percent of cases (e.g., diGeorge syn- drome and Down syndrome, among others). Individuals with congenital heart disease also have a higher prevalence of neuropsychiatric disorders, in- cluding learning disabilities, cognitive impairment, depression, and anxiety. TREATMENT Recent Advances Surgical intervention for congenital heart disease began in the mid- 1960s and was first applied to infants in the mid-1970s. Advances in surgical techniques have continued since that time. These include the de- velopment of staged palliation for hypoplastic left heart syndrome. Cath- eter-based treatment is also evolving rapidly, permitting the placement of valves and closure of atrial and ventricular defects percutaneously in some patients. Drug treatments for the complications of congenital heart disease have also advanced, including the development of medications for pulmo- nary hypertension (Eisenmenger syndrome) and better drug treatments for heart failure and arrhythmias. Side Effects of Treatments With improvement in infant surgery and palliation of more complex lesions, the number of survivors of congenital heart disease has increased

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  CaRdIoVaSCUlaR dISabIlIty and the population of children and adults with congenital heart disease in the United States has grown. Residual defects after surgical repair may contribute to functional im- pairment (Perloff and Warnes, 2001: adapted from information presented in Table 2). They can be classified as follows: • Valvular: Mitral or tricuspid insufficiency, pulmonary insufficiency, mitral stenosis • Ventricular o Ventricular inversion, atrial baffling of blood flow o Hypertrophy o Abnormal systolic or diastolic function • Vascular: Abnormal venous connections • Prosthetic: Synthetic conduits, artificial valves Even when surgical treatment corrects the abnormal anatomy, patients may still have late complications and sequelae. The incidence of atrial ar- rhythmias increases with age: up to 40 percent of adults with congenital heart disease may have atrial arrhythmias by age 50 (Bouchardy et al., 2009). Pulmonary vascular resistance may be increased as a result of long- standing left-to-right shunting. Trends in Morbidity and Mortality Survival improved for infants to 90 percent by the 1990s, but long- term survival remains diminished compared with the general population. With extended lifespan after surgery, more late sequelae of congenital heart disease are evident among adults. DISABILITY Congenital Heart Disease and Work/Effort In one European database, 59 percent of adults with complex con- genital heart disease had paid employment, compared with 76 percent of patients with mild, complex congenital heart disease. The employment rate among adults with complex disease was significantly less than the general population (Kamphuis et al., 2002). Learning disabilities, depression, and syndromic comorbidities may also impair the ability of individuals with congenital heart disease to hold gainful employment. Other conditions that may affect overall functional status among congenital heart patients include developmental abnormalities, mental re- tardation, growth retardation, somatic abnormalities (auditory, dental,

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 CongEnItal hEaRt dISEaSE facial, musculoskeletal), central nervous system abnormalities, seizures, visual abnormalities, and medical disorders (e.g., renal insufficiency or liver cirrhosis). Congenital Heart Disease and Functional Limitation Children Prior to cardiac surgery many children are unable to feed normally, requiring frequent prolonged or enteral feedings. Children may lack normal muscle strength and, consequently, may have delayed development. They may be unable to attend day care because of weakness or intolerance to respiratory infections. Infants may require home monitoring of heart rate, respiratory rate, daily weight, and pulse oximetry prior to surgery, com- promising their ability to function (Kugler et al., 2009). Some infants have additional noncardiac congenital abnormalities that contribute to medical morbidity or developmental delay. Following cardiac surgery, children may have variable degrees of disability. At school age, 11 to 17 percent of children who have had an operation for their congenital heart disease have significant limitations in adaptive behavior, socialization, communication skills, and daily liv- ing skills (Majnemer et al., 2008). Even after arterial switch operation for transposition of the great arteries, one of the surgical procedures thought to be most successful, one quarter of children were functionally impaired 15 years later. Psychosocial deficits are common (Williams et al., 2003). Patients who have had only palliative surgery typically have severe lifelong functional limitations. Individuals born with single ventricles, for instance, have exercise capacity of only 50 to 60 percent of what is normal for their ages (Reybrouck and Mertens, 2005), and older individuals are more functionally impaired than younger individuals. Exercise capacity is also decreased in many other complex congenital heart diseases and de- clines at repeat testing (Manlhiot et al., 2009; Samman et al., 2008; Weipert et al., 1997). Persons with congenital heart disease may be limited in school or work ability by intrinsic or extrinsic factors. Intrinsic factors may include arrhythmia, chronic heart failure, depression, learning disability, and pul- monary hypertension. Extrinsic factors may include intolerance of lifting weight, intolerance of heat or humidity, lack of stamina, and decreased aerobic capacity. Many patients with congenital heart disease have restric- tive lung disease in addition to chronic heart disease (Lubica, 1996). Neuropsychiatric symptoms (fears, depression, or anxiety) have been iden- tified in 40 to 50 percent of children and adults with complex congenital heart

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0 0 CaRdIoVaSCUlaR dISabIlIty disease (Bromberg et al., 2003; Cohen et al., 2007; Gupta et al., 1998; Karsdorp et al., 2007; Kovacs et al., 2009; Loup et al., 2009; McCrindle et al., 2006; Norozi et al., 2006.; Spijkerboer et al., 2007; Toren and Horesh, 2007). Functional limitation in older children and adults with congenital heart disease may be assessed objectively by exercise testing with measurement of maximal oxygen consumption. Effort can be measured by the respiratory exchange ratio. Although measured exercise capacity correlates with per- ceived quality of life, children and adults with congenital heart disease tend to report greater exercise capacity than would be judged by peak exercise performance (Hager and Hess, 2005). age-Related Issues in Evaluation The functional consequences of congenital heart disease change as chil- dren grow into adolescence and adulthood. In addition, surgery alters the natural history of disease in both predictable and unpredictable ways. Surgery is the mainstay of treatment for congenital heart disease, but the timing and extent of surgery varies according to the specific defect. Most congenital heart defects are now repaired in infancy, certainly by age 1. Single ventricle defects, however, are repaired with staged surgery, using a sequence of several procedures over the first 2 to 3 years of life. Other defects that can be corrected in a single procedure are occasionally delayed until the child reaches a defined size or age. After the completion of planned corrective surgery, a patient with con- genital heart disease may be restored to full functional capacity (e.g., after “curative” procedures) or have residual function limitations. These are best assessed after about 6 months of recovery from the last planned operation. Growth and development continues through childhood and may alter functional capabilities. The functional status of patients should be rela- tively stable by age 12, at which time children can cooperate sufficiently to undergo exercise testing. Reevaluation of patients with congenital heart disease at this age can establish their longer-term capacities and facilitate educational and vocational planning. Functional limitations may develop at any age, however, due to the development of arrhythmia, heart failure, endocarditis, or pulmonary hypertension, among other problems. See Figures 10-1 through 10-4 for documentation of congenital heart defects likely to require surgery, from diagnosis of significant heart disease in infancy or childhood through age 12 to adult. CURRENT LISTINGS The current listings for children and adults are found in Boxes 10-1 (children) and 10-2 (adults).

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 CongEnItal hEaRt dISEaSE Diagnosis of Significant Heart Disease in Infancy or Childhood YES Surger y Planned YES NO Disabled until 6 months af ter planned Go to Group B (Figure 10 -2) surger y (or surgeries) 6 months af ter surger y (or surgeries) Go to Group A (Figure 10 -2) FIGURE 10-1 Documentation of congenital heart defect likely to require surgery, diagnosis of significant heart disease in infancy or childhood. Figure 10-1--LANDSCAPE BLACK WHITE.eps Group A : Birth ( From 6 Months After Hear t Surgery) to Age 12 OR Group B : Birth to Age 12, No Surgery Indicated Cyanotic Pulmonary Hear t Failure Single Acyanotic Hypertension Ventricle Hear t Persistent Symptoms and Disease + /- Fontan tachycardia very serious Pulmonary Palliation OR interference with Hematocrit > 55% ar tery systolic Persistent ability to OR pressure tachypnea independently Ar terial ox ygen 70 % systemic OR initiate, sustain, saturation < 9 0 % ar terial pressure Markedly or complete In room air or PO2 decreased age-appropriate < 6 0 mm Hg exercise activities OR tolerance Hypercyanotic OR spells, squatting Grow th OR disturbance Pulse oximetr y < 85% on exer tion FIGURE 10-2 Documentation of congenital heart defect likely to require surgery, disabled by Groups A and B, birth to age10-2.eps Figure 12.

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  CaRdIoVaSCUlaR dISabIlIty Group C: Ages 12 to18 Cyanotic Pulmonar y Hear t Failure Single Ventricle Hear t Hypertension VO2 ma ximum ≤ 15 Disease +/- Fontan ml /kg/ min or 5 Pulmonar y Palliation metabolic equivalents Hematocrit > 55% ar tery systolic of task on exercise OR pressure tolerance test Ar terial ox ygen 70 % systemic OR saturation < 90 % ar terial pressure Three hospital visits In room air or PO2 for intravenous < 6 0 mm Hg treatment in < 1 year OR OR Pulse oximetry Right hear t failure < 85% on exertion Symptoms: Dyspnea, edema, fatigue AND Signs: Jugular venous distension, ascites, edema on 3 clinic visits in 1 year FIGURE 10-3 Documentation of congenital heart defect, disabled by Group C, ages 12 to 18. CONCLUSIONS AND RECOMMENDATIONS All Individuals RECOMMENDATION 10-1. Learning disabilities, cognitive impair- ment, and associated noncardiac congenital anomalies are frequent comorbidities for individuals with congenital heart disease. Disability evaluators should be trained to understand the effects of these comor- bidities to better evaluate if a combination of impairments, no one of which meets a listing, equals a listing. RECOMMENDATION 10-2. Assessment of disability in children should account for the natural history of congenital heart disease and patterns of development by dividing children into three age/treatment groups:

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 CongEnItal hEaRt dISEaSE Adults : Ages 18 and Over Cyanotic Pulmonar y Hear t Failure Single Ventricle Hear t Hypertension + /- Fontan VO 2 ma ximum ≤ 15 Disease Palliation ml /kg/ min or 5 Pulmonar y Hematocrit > 55% metabolic equivalents ar tery systolic OR of task on exercise pressure Ar terial ox saturation tolerance test 70 % systemic < 90 % OR ar terial pressure In room air or PO2 Three hospital visits < 6 0 mm Hg for intravenous OR treatment in < 1 year Pulse oximetry < 85% on exer tion OR Right hear t failure Symptoms: Dyspnea, edema, fatigue AND Signs: Jugular venous distension, ascites, edema on 3 clinic visits in 1 year FIGURE 10-4 Documentation of congenital heart defect disabled as adults, ages 18 and over. Figure 10-4.eps Group A: Infants prior to and for 6 months after definitive cardiac surgery; Group B: Children from 6 months after definitive surgery until age 12 and children from birth onward for whom surgery is not indicated; and Group C: Children age 12 and older. RECOMMENDATION 10-3. Infants with a medically confirmed diag- nosis of cardiac malformation requiring open-heart surgery should be considered disabled until 6 months after definitive corrective surgery. A diagnosis of significant cardiac disease and documentation of a surgical plan or surgical event should be sufficient evidence of disability. RECOMMENDATION 10-4. Children from 6 months after defini- tive cardiac surgery until their 12th birthday and children from birth

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4 4 CaRdIoVaSCUlaR dISabIlIty BOX 10-1 Current Congenital Heart Disease Listing for Children 104.06 Congenital heart disease,  documented  by  appropriate  medically  ac- ceptable  imaging  (see  104.00A3d)  or  cardiac  catheterization,  with  one  of  the  following: A. Cyanotic heart disease, with persistent, chronic hypoxemia as manifested by: 1.    ematocrit of 55 percent or greater on two evaluations 3 months or more apart  H within a consecutive 12-month period (see 104.00A3e); or 2.    rterial O2 saturation of less than 90 percent in room air, or resting arterial PO2  A of 60 Torr or less; or 3.    ypercyanotic spells, syncope, characteristic squatting, or other incapacitating  H symptoms directly related to documented cyanotic heart disease; or 4.  Exercise intolerance with increased hypoxemia on exertion.  OR B.    econdary  pulmonary  vascular  obstructive  disease  with  pulmonary  arterial  S systolic pressure elevated to at least 70 percent of the systemic arterial systolic  pressure. OR C.    ymptomatic acyanotic heart disease, with ventricular dysfunction interfering  S very  seriously  with  the  ability  to  independently  initiate,  sustain,  or  complete  activities. OR D.  For infants under 12 months of age at the time of filing, with life-threatening    congenital heart impairment that will require or already has required surgical  treatment  in  the  first  year  of  life,  and  the  impairment  is  expected  to  be  dis- abling (because of residual impairment following surgery, or the recovery time  required, or both) until the attainment of at least 1 year of age, consider the  infant to be under disability until the attainment of at least age 1; thereafter,  evaluate impairment severity with reference to the appropriate listing. SOURCE: SSA, 2008b.

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 CongEnItal hEaRt dISEaSE BOX 10-2 Current Congenital Heart Disease Listing for Adults 4.06 Symptomatic congenital heart disease (cyanotic  or  acyanotic),  docu- mented  by  appropriate  medically  acceptable  imaging  (see  4.00A3d)  or  cardiac  catheterization, with one of the following:  A.  Cyanosis at rest, and:  1.  Hematocrit of 55 percent or greater; or  2.    rterial O2 saturation of less than 90 percent in room air, or resting arterial PO2  A of 60 Torr or less. OR B.  Intermittent  right-to-left  shunting  resulting  in  cyanosis  on  exertion  (e.g.,    E   isenmenger’s physiology) and with arterial PO2 of 60 Torr or less at a work- load equivalent to 5 METs or less. OR C. Secondary  pulmonary  vascular  obstructive  disease  with  pulmonary  arterial  systolic pressure elevated to at least 70 percent of the systemic arterial sys- tolic pressure. SOURCE: SSA, 2008a. onward 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.

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  CaRdIoVaSCUlaR dISabIlIty 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: Persistent tachycardia at rest (see Table I1); or 1. Persistent tachypnea at rest (see Table II2); or 2. 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. 1 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). 2 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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 CongEnItal hEaRt dISEaSE 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 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:

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  CaRdIoVaSCUlaR dISabIlIty 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; 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

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