Chapter 31. Congenital Heart Disease in Adults

General Considerations

Congenital cardiac anomalies are the most common birth defects in humans, affecting approximately 0.8 in 100 live births. While the incidence of congenital heart disease is expected to decline as a consequence of improved prenatal diagnosis, the number of patients surviving with congenital heart disease, both in the United States and worldwide, has increased significantly over the past three decades. Over 85% of infants born with cardiovascular anomalies now can expect to reach adulthood. Reduced mortality rates can be attributed to improved diagnostic abilities, enhanced surgical and nonsurgical therapies, and improvements in intensive care. For the first time, the number of adults with congenital heart disease exceeds the number of children with the disorders.

The increase in the number of adults with congenital heart disease requires that the physicians managing the care of these patients have improved knowledge of simple and complex anatomy and physiology. Although actual numbers are difficult to ascertain, it has been estimated that approximately 1 million adults in the United States alone currently have congenital heart disease and that the number of patients reaching adulthood with treated congenital heart disease will increase by approximately 9000 per year.

These patients fall into several broad categories: those surviving into adulthood without intervention and perhaps without clinical recognition, those surviving with curative surgical or nonsurgical intervention, and those surviving with palliative surgical or nonsurgical intervention. Nonsurgical interventions may include catheter-based valvuloplasty, stenting, coiling, or device occlusion. The patients who are today making the transition into the adult congenital heart disease population have hemodynamic and cardiac problems differing from those in previous eras. Surgical techniques have evolved, intervention occurs earlier and is often definitive rather than palliative, and a greater number of patients with complex single-ventricle physiology and various modifications of cavopulmonary anastomoses (Glenn shunt, Fontan procedure) will reach adulthood. Because of the unique clinical conditions and needs of these patients, the American College of Cardiology and the American Heart Association have recommended that all patients with moderate or complex congenital heart disease be evaluated at least once by a physician with specialized training and expertise in adult congenital heart disease. Furthermore, it is recommended that diagnostic and interventional cardiac catheterization and electrophysiologic and surgical procedures in these patients be performed in regional adult congenital heart disease centers.