Structural Heart Disease in Adults

The incidence of congenital heart defects (CHD) is approximately 8 per 1,000 live births. With the advances in the diagnosis and management of CHD, a large number of these children survive into adulthood. The current estimate of adults with CHD is in the order of 1 million in the U.S. alone. Because of the high prevalence of coronary heart disease in adults, the major emphasis in training cardiologists is in the area of adult coronary heart disease. Similarly, most cardiologists’ experience is predominantly in adult coronary heart disease. Consequently, there is a relative lack of expertise in the arena of CHD in the adult cardiology community. Therefore, it is important that efforts be made to impart CHD knowledge in the continuing medical education of cardiologists. The symposium on CHD in this and the next issue of the Journal is an attempt to fulfill this requirement. The type of CHD seen in adults is different from that seen in childhood. The type of adult heart disease can best be understood by examining the incidence of the CHD at centers that care for adults with CHD. At one center, the outpatients seen were analyzed and the frequency, in descending order, was as follows: left-to-right shunts, left ventricular (LV) outflow tract obstruction, cyanotic heart defects, arrhythmias and pulmonary stenosis. In another institution, the defects were: left-to-right shunts, tetralogy of Fallot, single ventricle, LV outflow tract obstruction and transposition of the great arteries (TGA). The distribution of the inpatient population was slightly different: single-ventricle lesions including tricuspid atresia, secundum atrial septal defects (ASDs), Eisenmenger syndrome, pulmonary atresia and tetralogy of Fallot, in that order. The patients who required surgical intervention had yet a different distribution: Ebstein’s anomaly of the tricuspid valve, pulmonary atresia with ventricular septal defect (VSD), tetralogy of Fallot, coarctation of the aorta and LV outflow tract obstructive lesions. In another center, the surgical case distribution was: aortic valve replacement, ASD closure, change of RV outflow conduit, coarctation repair and systemic-to-pulmonary artery shunts. The type of surgery performed also changed over time. Catheter interventions included ASD/patent ductus arteriosus (PDA) closures, stents in the pulmonary artery, recoarctation ballooning or stenting, closure of superfluous vessels and percutaneous pulmonary valve replacement. In this issue of the Journal, several papers related to CHD are included. In the first paper, Robert Horvath and his associates from the University of Miami in Miami, Florida, discuss the role of transcatheter management of coarctation of the aorta. The techniques and results of balloon angioplasty and stent placement are discussed in detail. Balloon angioplasty of native and recurrent coarctation is an effective mode of treatment in infants and children. Stents may be more appropriate in adolescents and adults. Future directions in the management of coarctation include covered stents, open-ring stents and biodegradable stents. In the second paper, Marc Del Rosario et al from the University of Missouri in Columbia, Missouri, and Borgess Medical Center in Kalamazoo, Michigan, review the role of percutaneous interventions in adults with CHD. They describe transvenous closure of shunt lesions such as ASD, VSD, PDA, Fontan fenestrations, baffle leaks following prior atrial switch procedures for TGA and pulmonary arteriovenous malformations. They also describe transcatheter management of obstructive lesions such as coarctation of the aorta, pulmonary artery stenosis, baffle obstructions following prior atrial switch procedures for TGA, pulmonary valve stenosis and aortic stenosis. They conclude that advances in device technology and improved outcomes have resulted in the more frequent use of percutaneous interventions in adults with CHD. Two rare coronary artery anomalies were discussed in this issue. First, Sedat Turkoglu et al describe a rare congenital coronary artery anomaly with two left anterior descending coronary arteries (one arising from the left anterior descending coronary artery and the other from the right coronary artery) and an anomalous origin of the left circumflex coronary artery from the right coronary artery; the patient had severe obstruction of the left anterior descending artery arising from the common left coronary artery that was successfully relieved by percutaneous intervention. In the Clinical Images section, Matthew Purvis et al from the Medical Center of the Rockies in Fort Collins, Colorado, nicely illustrate an anomalous origin of the left main coronary artery from the ascending aorta by selective coronary angiography; this patient was studied because of chest discomfort and atrial fibrillation. The CT angiography images with three-dimensional reconstruction demonstrated this rare abnormality clearly. However, there does not appear to be any atherosclerotic disease. These two are very rare coronary artery anomalies and are of interest, and may not necessarily, by themselves, indicate disease. In the final paper in this issue, Paul Chiam and his colleagues from Lenox Hill Heart and Vascular Institute in New York, New York, describe a novel approach to address cardiac perforation during patent foramen ovale closure. While we want to avoid such complications, dealing with it when it happens as effectively as the authors have done is commendable. These reviews and reports, while not addressing all the issues related to CHD in adults, have begun the process of fulfilling the objective stated above.