Congenital Heart Disease in Adults

Advances in diagnostic methods and in the surgical and medical care of infants and children with congenital heart disease have resulted in one of the most successful rehabilitation programs that medicine has witnessed.1'2 In the United States alone, there are presently between 500,000 and 600,000 adults with congenital heart disease. Each year, about 20,000 open operations for congenital heart disease (an approximate minimum) are performed in the United States (personal communication, 1990, Dr. Peter Einstein, Assistant Professor of Surgery and Pediatrics, University of Tennessee). Of an estimated 25,000 infants currently born with congenital malformations of the heart and circulation, over 85% will reach adulthood. The heavy investments in time and resources needed to accomplish these results should now be matched with a similar dedication to the adolescent and adult with congenital heart disease. Potential subspecialty professional resources (1989 figures) include 884 boardcertified pediatric cardiologists, too few to cope with the patient load of adults with congenital heart disease even if all 884 were committed to do so, and 12,119 board-certified medical cardiologists, only a small minority of whom have knowledge of, interest in, or responsibility for adults with congenital cardiac malformations.23 Congenital heart disease in adults is still a largely unrecognized subspecialty but is emerging as a discipline that requires special expertise. The adult congenital heart disease patient population includes those who have never undergone cardiac surgery, those who have undergone cardiac surgery and require no further operation, those who have had palliation with or without anticipation of reparative surgery, and those who are inoperable apart from organ transplantation. The following remarks deal broadly with what this new area of cardiovascular interest presently comprises: multidisciplinary facilities for comprehensive care, survival patterns (natural and postoperative), medical considerations, surgical considerations, and postoperative residua and sequelae.