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. With the exception of early surgical ligation of a patent ductus arteriosus (PDA), a surgical "cure" for 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. Standardized diagnostic and treatment guidelines have emerged from the American College of Cardiology (ACC) and American Heart Association (AHA).6 Diagnostic and treatment errors by cardiologists inexperienced in patients with complex CHD can be minimized by improving understanding of the pathophysiology, hemodynamics, and prognosis of various lesions. Patient referral to regional adult CHD experts is encouraged to facilitate specialized counseling and management. These patients 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.
Specialized adult CHD centers have developed in North America and in Europe, but there remains a need for more specialized centers even in these regions.7 Requirements include close collaboration among adult and pediatric cardiologists, cardiac surgeons, nurse specialists, and consultants. Training and research are of pivotal importance. The development of management, research, and training guidelines over the past decade is a major step forward for the field of adult CHD.8-12 Another encouraging development is the establishment of the Adult Congenital Heart Association (ACHA) in the United States. This patient-initiated association is a nonprofit organization that seeks to improve the quality of life and extend the lives of adults with CHD. Multicenter and prospective clinical research in adult CHD have increased in frequency and variety.13 A dedicated multicenter research network, the Association for Adult Research in Congenital Cardiology, was established in 2007 in North America. The goal of this network is to foster and implement multicenter research.14
Many adults with CHD have not had, or may never require, surgical intervention. The most common defects incidentally encountered in adulthood are small ventricular or atrial septal defect (ASD), mild pulmonary stenosis, BAVs, and mitral valve prolapse (Table 84–1). Less common, but more complex, conditions include nonrestrictive central shunts with right-to-left shunt reversal resulting in the cyanotic Eisenmenger syndrome. Most patients require long-term follow-up to monitor for ...