Clinical Review

Adults with Congenital Heart Disease: The Critical Transition from Pediatric to Adult Care



Exercise Capacity and Restrictions

The ability to exercise is an important factor in the quality of life of ACHD patients, especially in the adolescent period when participation in school and recreational athletics oftentimes functions as a social institution. Exercise ability is influenced by both real limitations imposed by limited cardiopulmonary reserve as a result of underlying pathology and by misconceptions of and anxiety about their ability to safely participate in these activities. There is evidence of diminished aerobic activity in all groups with CHD. However, symptomatic restrictions account for only approximately 30% of all barriers to exercise,51 and some studies have shown that exercise training programs can improve functional capacity and some standards of quality of life in CHD patients, in addition to the general health benefits associated with obesity prevention.52

Recommendations regarding exercise capacity are often addressed at primary care visits, and should be reinforced by the patient’s cardiologist. In general, most patients with repaired or mild defects can engage in moderate- to high-intensity exercise; those with more complex defects, cyanosis, or arrhythmias should be evaluated by an ACHD specialist to determine an appropriate level of activity.27 The “exercise prescription” provided to the patient should include type of exercise tolerated as well as heart rate goals and limits. In patients with extremely limited exercise capacity, a cardiac rehabilitation program can be beneficial. The presence of significant pulmonary hypertension, cyanosis or aortic stenosis, symptomatic arrhythmias, or evidence of myocardial dysfunction usually restricts the degree of exercise; full recommendations by activity and lesion type can be found in the guidelines proposed by the 36th Bethesda Conference.53 The importance of serial and regular evaluations is emphasized in these guidelines due to changing hemodynamic status of the patient over time as their cardiac lesions evolve and new complications arise.

Social and Psychological Impact of Chronic Illness

Living with a chronic disease can have a psychological impact on the child and transitioning adolescent. Frequent hospitalizations, physician visits, medical tests, and management of medical emergencies take a toll on the patient’s self-image and self-esteem, particularly during their formative adolescent years. Adolescents with CHD often feel “different” from their peers due to their condition,54 causing them to withhold disclosures about their heart disease to others out of fear of its impact on personal and professional relationships. Recent studies have shown that children and adolescents with CHD are at risk of internalizing problems and exhibiting behavior problems;55 they are also more likely to have impaired quality of life secondary to their increased incidence of psychosocial difficulties.56 The social and physical debility often experienced by patients with ACHD leads to a higher incidence of depression and anxiety in this population.57 Studies have shown that ACHD patients are interested in psychological treatment and peer support of their mood and anxiety disorders.58

At least some degree of the mental health issues ACHD patients experience is thought to have a physiological basis and be related to early cyanosis and neonatal surgical bypass duration. Prolonged duration of deep hypothermic circulatory arrest (DHCA) during corrective surgery is associated with reduced social competence, and has been found to be an independent risk factor for anxiety, depression, aggressive behavior, and attention deficiencies.59 In other studies, DHCA has been associated with decreased intellectual ability and worse fine motor skills, memory, and visuospatial skills, among other neurodevelopmental outcomes.60-62 Psychiatric disorders have also been associated with genetic syndromes like DiGeorge syndrome.63 This impacts executive function, leading to missed appointments, delay in clinical visits, and medication noncompliance. Given the potential for worse outcomes and risk of transition failure, primary care providers should routinely evaluate CHD patients for mood disorders and neurocognitive delay.

Social Determinants of Health and Medical Legal Partnerships

Social determinants of health and workplace discrimination play a large role in determining the ability of individuals with CHD to achieve adequate health care and maintain gainful employment. Individuals with CHD often face significant challenges as they prepare to enter the workforce, including discrimination within the workplace and maintaining employment through medical emergencies. Studies have shown that while educational milestones are similar between patients with and without CHD, those with CHD are much less likely to be employed.64 Challenges facing adolescents as they enter the workforce include hiring discrimination, physical challenges imposed by functional limitations, and misunderstanding of disease process and actual functional capacity. Career counseling is therefore an integral part of the transitioning process and should be started in early adolescence to allow for full assessment of mental, physical, and social abilities.65

Medical-legal partnerships (MLPs) can be extremely beneficial to the CHD population adversely affected by social determinants of health and workplace discrimination. These partnerships integrate lawyers into health care to address legal problems that create and perpetuate poor health; on a broader scale, these partnerships can advance and support public policy changes that improve population health.66

The major social determinants of health addressed by MLPs are income supports/insurance, housing/utilities, employment/education, legal status, and personal/family stability (summarized in the mnemonic I-HELP).67 Some of the more specific areas in which MLPs may assist in the delivery of care to CHD patients include case management, translation services, health literacy, and legal aid/legal services. ACHD patients also often experience a significant loss of services, including physical, occupational, and speech therapy and nutrition services, as adult clinics may not be prepared to provide these services. While physicians can best address the individual patient’s health, members of the legal system can address the systemic ailments that propagate that patient’s recurrent hospitalizations and other use of medical resources. Members of the legal system are present onsite in health care settings and participate in clinical meetings, which allows a coordinated and comprehensive screening for social needs that may harm a patient’s health.

Next Article: