The Specialty of Adult Congenital Heart Disease

Abstract

Over the last three decades, adults with congenital heart disease (ACHD) have become one of the fastest growing populations of adults with chronic heart disease. Despite significant progress in laying the foundations to meet the rising care needs for ACHD patients, limited access to care and suboptimal data are impediments to continued progress. The successes of pediatric cardiologists and surgeons have permitted individuals with conditions that previously would have been lethal in childhood to live and thrive well into adulthood. This fact, in combination with improvements in imaging technology permitting identification of clinically silent lesions, has led to rapid growth in the numbers of patients with ACHD in the general population (1, 2). This success, however, has created a unique challenge to the field of cardiology. Adult cardiologists are not required by the Accreditation Council for Graduate Medical Education (ACGME) to have specific training in caring for patients with congenital heart disease. As a result, most adult cardiologists lack adequate familiarity with congenital cardiac problems to provide optimal care. ACGME requirements for pediatric cardiology training, in contrast, focus on congenital heart disease and require fellows to have experience specifically in ACHD. Nevertheless, pediatric cardiologists have limited exposure to cardiologic issues common in adult patients, such as coronary vascular disease or the cardiovascular effects of cocaine abuse, for example. In recognition of the need for a uniquely trained cadre of individuals to care for this population, the ACGME has recently created a training pathway for ACHD, open to both pediatric and adult cardiologists, and ACHD is now recognized as a subspecialty of both pediatric and adult cardiology. In addition, the most recent guideline statement from the American College of Cardiology and American Heart Association on the management of patients with ACHD recommends that patients with all but the most simple of lesions be evaluated by ACHD specialists (3). The benefits of these recommendations were demonstrated in a recent population-based study from Quebec, Canada, in which referrals for ACHD specialty care resulted in decreased mortality rates (4). As rates of hospitalization and inpatient care costs among ACHD patients continue to grow and the ACHD population ages, the importance of access to ACHD-trained practitioners will increase (5–8). To meet the growing needs to serve the ACHD population, more ACHD-trained specialists are needed. In 2009, there were just over 2000 board-certified or -eligible pediatric cardiologists in the United States (9). In contrast, only approximately 200 examinees sat for the first ACHD board exam in October 2015. In the US and Canada, however, it is estimated there are now more adults than children living with congenital heart disease (2, 3). In addition to meeting the growing demand for clinical care, more ACHD specialists are needed to broaden the therapeutic options available to improve outcomes in this unique population. The few well-conducted, randomized, placebo-controlled trials in ACHD have failed to demonstrate the efficacy of modern neurohumoral modulation in improving outcomes in ACHD patients (10–16). Although these failures may indicate inefficacy of the tested medications, trends present in these studies suggest that patient heterogeneity and inadequate study power may have contributed to their failure. Continued progress demands cooperation between providers to optimize patient recruitment and to identify novel ACHD-specific outcomes with greater sensitivity to clinical changes in this population. It is essential that adult cardiologists make good on the promises implied by the successes of our pediatric colleagues, to improve quality and quantity of life for ACHD patients. To achieve this goal, the cardiology community must encourage fellowship training in ACHD, increase cooperation between providers, and increase awareness of the importance of disease-specific care. In this way we can continue to make a major difference in the lives of some of the greatest medical success stories of the last century.