The incidence of moderate and severe forms of congenital heart disease (CHD) is 6 per 1000 live births. If bicuspid aortic valves (BAVs) are included, the incidence increases to 19 per 1000 live births.1 Without early medical or surgical treatment, the majority of patients with complex CHD would not survive to adulthood.2 Surgical and medical advances over the past 60 years have dramatically altered the once bleak prognosis of patients with CHD. In the current era, more than 85% of patients with CHD survive to reach adulthood, and most live productive and functional lives.3,4 Many patients have undergone surgical interventions that were once thought to be curative. However, with the exception of early surgical ligation of a patent ductus arteriosus (PDA), a surgical “cure” for complex CHD without operative sequelae or need for reoperation does not exist.5
Adults with operated and unoperated CHD require long-term follow-up. This long-term care should include cardiologists with experience in or who specialize in adult CHD (ACHD). Standardized diagnostic and treatment guidelines have emerged from the American College of Cardiology (ACC) and American Heart Association (AHA).6 Cardiologists trained and experienced in patients with complex CHD can minimize diagnostic and treatment errors with a strong knowledge base in pathophysiology, cardiovascular hemodynamics, and prognosis of congenital lesions.
These patients may also face a variety of psychosocial issues, including self-image problems; difficulties in acquiring and maintaining health and life insurance; exercise and activity limitations; and issues of sexuality, contraception, and reproduction. Patient referral to a regional team of experts in a recognized ACHD center is encouraged to facilitate specialized counseling and management.
Specialized ACHD centers have developed in North America and Europe, but there remains a need for more specialized centers even in these regions.7 Requirements for comprehensive ACHD care centers include dedicated ACHD clinics, ACHD cardiologists, congenital cardiac surgeons, nurse specialists, and consultants. Training and research are of pivotal importance. The development of management, research, and training guidelines over the past two decades is a major step forward for the field of ACHD.6,8,9,10,11 Another encouraging development is the establishment of the Adult Congenital Heart Association (ACHA) in the United States. This nonprofit patient-initiated association seeks to improve the quality of life and extend the lives of adults with CHD by leading legislative efforts, sponsoring patient and physician conferences, and identifying and certifying comprehensive ACHD centers in the United States. Multicenter and prospective clinical research in ACHD has increased in frequency and variety.12 A dedicated multicenter research network, the Association for Adult Research in Congenital Cardiology, was established in 2007 in North America to foster and implement multicenter research.13 A recent collaboration between the ACHA and the National Heart, Lung, and Blood Institute also strives to identify and promote key areas of research in ACHD.