Clinical Review

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



Acquired Cardiovascular Disease

As the ACHD patient ages, acquired cardiovascular disease becomes a significant issue. Approximately 80% of adults with CHD have at least 1 cardiovascular risk factor,22 though overall there is a relative lack of specific data regarding the U.S. population. Surveillance of the Canadian CHD population older than 65 years shows a 47% prevalence of hypertension,23 with increased risk in certain conditions such as aortic coarctation and renal disease associated with CHD. Although studies on the increased risk of diabetes mellitus in the ACHD population have yielded conflicting results,22,24 there is evidence of abnormal glucose metabolism in ACHD patients, which is a predictor of cardiac morbidity and mortality.25,26 The incidence of hyperlipidemia in U.S. ACHD patients is estimated to be at least as high as that of the general population.1 These factors combine with abnormalities in the myocardial substrate, hemodynamic abnormalities, arrhythmias, and sequelae of surgical repairs to confer an increased risk of ischemic heart disease and cerebrovascular disease in the ACHD population.15,27 One large case-control cohort study showed that the risk for ischemic heart disease was 16.5 times higher in patients with CHD as compared with non-CHD patients, with the highest incidence being in those with conotruncal defects and severe non-conotruncal defects. Interestingly, hypertension and diabetes were less common among CHD patients with ischemic heart disease than among non-CHD patients with ischemic heart disease.28

Adults with CHD have an increased risk for cerebrovascular disease compared with the general population, and cerebrovascular disease appears to occur at a younger age.29 The risk of ischemic stroke in individuals with ACHD younger than 55 years is 9 to 12 times higher than that in the general population. As in the general population, the incidence of ischemic stroke in ACHD patients increases with age, and in those older than 55 years, the incidence remains 2 to 4 times higher than in the general population.30,31

Clearly, complications arising from therapeutic interventions in CHD patients contribute significantly to morbidity/mortality in adult life, which underscores the need for life-long follow up and prevention of lapses in care.

The Transition from Pediatric to Adult Care

The monitoring and evaluation of CHD patients throughout life requires close coordination between the patient’s primary care physician, cardiologist, ACHD specialist, and other specialists, as appropriate. The timing of routine follow-up appointments is largely dependent on the severity of the congenital heart lesion and clinical status of the individual patient. Routine surveillance often includes cardiac imaging, preconception/genetic counseling, Holter screenings for arrhythmia, laboratory testing, and titration of medication. Unfortunately, only 30% of adults with CHD receive the recommended cardiac care.32

Children with chronic conditions transitioning to adulthood frequently experience a drop off in coordinated services as they transition from pediatric to adult medicine. Adult institutions often have less multidisciplinary support staff in the form of social workers and case management.33 Furthermore, a recent systematic review of articles that outlined the transition process from pediatric to adult cardiology in the CHD population showed that a high proportion of patients were either lost to follow up or had long gaps in care after leaving pediatric cardiology, with the first lapse in care commonly occurring at approximately age 19 years.28,34 A 2004 study showed that only 48% of adolescents with CHD underwent successful transition.35 A multicenter study of 922 ACHD patients found a gap in care lasting longer than 3 years in 42%, with 8% having gaps exceeding 10 years.36 Another study showed that lapses exceeding 2 years occurred in 63% of patients, with a median duration of lapse of medical care of 10 years. The most common reasons for lapse in care were: being told that cardiac follow up was not required (33%); being discharged from a children’s hospital without appropriate follow up plans in place (23%); being aware of need for follow up but having no symptoms (19%); lack of insurance (18%); and ignoring follow up recommendations for fear of receiving bad news (7%).37 Moreover, living independently from one’s parents was independently associated with a lapse in care, and patients with moderate complexity defects were more likely to experience a lapse than those with high complexity defects.

In the absence of a structured transition program, there is often delayed or inadequate care, which can result in significant emotional and financial stress on families and increased stress on the health care system.38 Inadequate, incomplete, or nonexistent transition and transfer for care has been shown to lead to poor health outcomes. Patients who experienced a lapse in care were 3 times more likely to require urgent cardiac intervention and to have an adverse outcome.37 The urgent interventions required by these patients included pulmonary valve replacement, mitral and tricuspid valve repair/replacement, VSD closure, pulmonary artery stenting, Fontan revision, and pacemaker/defibrillator placement.37 Clearly, there is significant room for improvement in the transition process of patients with CHD.

Best Practices in Transitioning CHD Patients to Adulthood

The overarching goal of pediatric to adult care CHD transition programs is to empower the patient and their support system to assume ownership of the disease process in order to maximize quality of life, life expectancy, and productivity.39 This involves ensuring that the patient has a thorough understanding of their diagnosis, heart anatomy, prior cardiac interventions, limitations imposed upon them by their condition, and the frequency of their anticipated follow-up care. The components of a successful transition program include a systematic approach to addressing the medical, psychosocial, and educational/vocational needs of the adolescent as he or she moves from the family-centered pediatric to the patient-centered adult health care system.40 The visits during the transition period are also an opportunity to discuss reproductive issues and the need for planning pregnancies for women with CHD. The goal is to encourage autonomy and promote ownership of their medical condition to the best of their social-cognitive ability. Adolescents should be encouraged to speak alone with their doctor to foster independence and self-management in their disease process; this has been shown to be protective against failure in transition.32 They should be encouraged to start calling their doctors, requesting refills, and making appointments.

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