The adult with congenital heart disease

Summary Internal medicine cardiovascular specialists and internists are going to be seeing increasing numbers of adult patients with congenital heart malformations, and most of them will have had one or more surgical or therapeutic catheter procedures. The nonsurgical cases will have either benign defects or important but inoperable anomalies, or the physician may encounter the rare case which, though amenable to surgery, has escaped operation until adult life. Among patients with postoperative congenital heart disease, a few are totally cured and require no special follow-up or any special precautions, e.g., those with certain cases of atrial septal defect of ostium secundum types, patent ductus arteriosus without pulmonary hypertension, and fully repaired total anomaly of pulmonary venous return. Others who are very much improved by definitive repair have residual defect for which surgical treatment was not attempted, e.g., bicuspid aortic valve in the patient who had surgical repair of coarctation or the patient with persistent cleft of a mitral valve leaflet for which no repair was attempted at the time of closure of the ostium primum type of atrial septal defect. Some patients have had incomplete and unsuccessful repair of a defect but yet are symptomatically improved compared with their preoperative status, e.g., those with tetralogy of Fallot with loss of right-to-left shunting by closure of the ventricular septal defect but persistent right ventricular outflow tract or pulmonary artery branch obstruction. Patients with persistently high intracardiac pressure from unrelieved obstruction are at higher risk for sudden death than they were preoperatively, especially those with a high grade of ventricular ectopy. Despite symptomatic improvement compared with the preoperative status, such cases must have reoperation, if feasible, as a means to prevent a sudden catastrophe. Patients with congenital malformations have symptoms that are distinctly different from those with acquired heart disease. Furthermore, their reactions to their problems and to their lives and work in relation to others are often colored by their restricted and overprotected childhoods, which were often filled with doctor visits, several admissions to the hospital, one or more operations, and usually several heart catheterizations. Otherwise, these patients are capable of the same level of achievement intellectually, and only a few have important defects of other systems that would interfere with their roles as independent and productive adults. The problem arises as to what group of physicians will care for these patients in the future. If the internal medicine cardiologists intend to do so, they should, as trainees in cardiovascular disease, seek a minimum of 2 to 4 months in a pediatric cardiology program in which they would have the opportunity to learn pediatric cardiology and congenital heart disease by first-hand observation and supervised participation. In the mentime, many adult patients with congenital malformations are still under the care of pediatric cardiologists. If this system is to continue in the future, pediatric cardiologists will need additional training in adult cardiovascular disease to fulfill their obligations to their adult patients.