Congenital cardiovascular malformations are the most common birth defect, occurring in almost 1% of live births. Although congenital heart lesions are common in aggregate, they are comprised of rare and diverse disorders, ranging from simple lesions (eg, atrial septal defect and patent ductus arteriosus) that can be corrected with surgery or catheterization to single ventricle that can be palliated by the Fontan procedure. Dramatic advances in medical and surgical therapy have reduced the mortality rate from virtually all forms of congenital heart disease, and the great majority of children with congenital heart disease now survive to adulthood. The 2006 Report of the National Heart, Lung, and Blood Institute Working Group on Research in Adult Congenital Heart Disease estimated that, by 2005, the number of adults with congenital heart disease had reached 1 million. For the first time, more adults than children with congenital heart disease are alive in the United States. The complexity of congenital heart disease in adults is at least as great as that in children and will increase further as a wave of patients with single ventricle, including those with hypoplastic left heart syndrome, reach young adulthood. Moreover, the dramatic increase in the population of adult survivors of congenital heart disease has been accompanied by increased recognition of long-term morbidities that will confront the adult cardiologist. Finally, although congenital heart disease usually presents in infancy and childhood, some lesions, such as atrial septal defect, may be first diagnosed in adulthood.
Articles in the series Congenital Heart Disease for the Adult Cardiologist will be published twice monthly over the next 6 months. The series will begin with basic lesions, including atrial septal defects, ventricular septal defects, and patent ductus arteriosus. Later topics will include more complex anomalies, including left ventricular outflow obstruction (eg, bicuspid aortic valve, coarctation of the aorta, and subaortic and supravalvar aortic stenosis); lesions affecting the right ventricular outflow tract (eg, valvular pulmonary stenosis, tetralogy of Fallot, double chamber right ventricle, and double outlet right ventricle); transposition of the great arteries (eg, dextro-transposition of the great arteries and levo-transposition of the great arteries; and Ebstein’s anomaly. An article on univentricular heart will review this most complex family of congenital cardiovascular malformations. An article on congenital coronary artery anomalies will focus on anomalous origin of a coronary artery from the opposite sinus, associated with sudden death in the young. Pulmonary vascular disease and arrhythmias in adult patients with congenital heart disease will each be topics of articles because of the importance of these sequelae. A final article will provide an overview of interventional catheterization in adult congenital heart disease, including the most commonly performed percutaneous procedures, such as valvuloplasty, angioplasty, and device closure.
Adult cardiologists will increasingly be responsible for the long-term care of individuals with congenital heart disease. We hope that this series will provide them with a framework for understanding abnormalities of cardiac morphology and their translation to altered physiology, clinical manifestations, and therapies.