Clinical Review

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



From the Greenville Health System, Greenville, SC.


  • Objective: To review the management of patients with congenital heart disease (CHD) transitioning from pediatric to adult care.
  • Methods: Review of the literature.
  • Results: Persons with CHD require close monitoring and evaluation throughout life to address the physiologic consequences of acquired cardiopulmonary, gastrointestinal, and renal disease in the setting of underlying congenital heart lesions. During the transition from pediatric to adult cardiology, a high proportion of patients are lost to follow up or have long gaps in care after leaving pediatric cardiology, which can lead to poor outcomes. Care of the adult with CHD requires close coordination between the patient’s primary care physician), cardiologist, adult CHD specialist, and other specialists. The transition process for CHD patients begin at 12 years of age, with a goal of discussing future expectations of the child’s education, employment, and independent living. Successful transition programs use 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.
  • Conclusion: The transition from pediatric to adult care in ACHD patients is best provided through a comprehensive transition program that begins in early adolescence and enables patients to take charge of their disease process in adulthood, allowing them to maximize their quality of life and societal contributions.

Keywords: adult; congenital heart defects; complications; disease management; patient care team.

The population of adults with congenital heart disease (CHD) in developed countries has grown at an exponential rate in the past 4 decades. With advances in medical care and surgical interventions, the proportion of pediatric patients reaching adulthood has increased from 15% in the 1930s-60s to more than 95% for patients with mild to moderate complexity CHD. The rate of survival to adulthood for patients with severely complex CHD remains lower at around 56%.1

There are now more adult than pediatric patients with CHD in the United States. Because adult CHD (ACHD) patients have increased morbidity and mortality in their young adult years, it is imperative for all providers to understand and address the long-terms needs of this population. Unfortunately, adults with CHD do not always receive adequate health care, frequently because they are lost to follow-up, particularly during their adolescent years when they are expected to gain independence in their medical management. As will be discussed, CHD is a chronic illness fraught with numerous expected and unexpected complications that require close monitoring and re-interventions. Effectively anticipating and addressing these complications requires a standardized and comprehensive process of transition from the pediatric to the adult population to ensure maximal quality of life.


The actual prevalence of ACHD in the United States is unknown, as a national database of persons with CHD has not been established.2 In contrast, Europe and China have maintained databases that enable ongoing monitoring of the evolving CHD epidemiology in those regions.3,4 The best estimates of the U.S. incidence and prevalence of ACHD stem from extrapolations from Canadian data. According to this data, there were more than 1.2 million adults with ACHD in the United States in 2012, with an anticipated 5% annual increase.1,5 However, the limitations of such extrapolations must be noted, as the Canadian population does not perfectly mirror that of the United States. Canada has lower infant mortality and adult obesity rates, and the United States has larger African American and Hispanic populations.6 Also, the juxtaposition of universal access to health care in Canada and the socioeconomic class–dependent access in the United States causes variations in care and outcomes of ACHD between the 2 populations. These differing genetic and social backgrounds may change the incidence of CHD by affecting maternal-fetal health.7

The 32nd Bethesda Conference on “Care of the Adult with Congenital Heart Disease” in 2000 was tasked with characterizing the ACHD population in the United States. This project found a prevalence similar to that of the Canadian extrapolation and showed that among persons with ACHD in the United States, 45% have mild disease, 37% moderate disease, and 13% severe disease.8


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