As of 2000, more adults than children are alive with
congenital heart disease. Each year, more of these adults with
congenital heart disease undergo surgery. Adults with
congenital heart disease require lifelong surveillance, follow-up imaging, and clinical decision making by appropriately trained and familiar physicians and extenders. Three common challenges facing adults with
congenital heart disease are the neglected patient, weak programmatic infrastructure, and the planning and management of pregnancy in the adult with
congenital heart disease. Many challenges must be overcome in order to establish and maintain a comprehensive medical and surgical program for adults with
congenital heart disease. Three common and challenging groups of adults with
congenital heart disease who may require surgery are adults requiring tricuspid valve surgery for
Ebstein's malformation, those requiring reoperation for failing
Fontan circulation, and those requiring pulmonary valve replacement (PVR). Surgery for
Ebstein's anomaly is now offered to older patients at low risk and with good late outcome. The operation includes tricuspid valve repair or replacement and frequent concomitant procedures such as
atrial septal defect closure,
arrhythmia surgery (the
Maze procedure), and
coronary artery bypass grafting. Patients undergoing previous iterations of the
Fontan operation are especially prone to
arrhythmia and failing circulation. Fontan conversion with
arrhythmia surgery and pacemaker
therapy was developed by Constantine Mavroudis, Barbara Deal, and Carl Backer to treat these challenging patients, with the first such operation performed in 1994. Fontan conversion involves: (1) conversion of the previously created atriopulmonary connection to a total cavopulmonary connection (TCPC), extracardiac Fontan; (2)
arrhythmia surgery, typically with a modified biatrial
Maze procedure along with placement of an antitachycardia, dual-chamber pacemaker with
steroid-eluting epicardial leads in all patients; and (3) concomitant procedures to treat associated lesions. The need for pulmonary valve replacement is increasing for many adults with
congenital heart disease. In the past, chronic
pulmonary regurgitation following repair of
tetralogy of Fallot was considered benign. Current evidence demonstrates that chronic
pulmonary regurgitation causes significant morbidity by producing right ventricular dilatation and dysfunction, exercise intolerance, arrhythmias, and
sudden death. Multiple options exist for pulmonary valve replacement including several recent developments such as pulmonary valve replacement with a hand-sewn
polytetrafluoroethylene bicuspid valve and percutaneous pulmonary valve replacement. Reoperative cardiac surgery is common in adults with
congenital heart disease. Although a history of previous cardiac surgery does not independently confer a significant incremental risk of operative mortality, patients with the greatest number of previous surgeries appear to be a higher risk group. Multi-institutional data about adults with
congenital heart disease from The Society of Thoracic Surgeons Congenital Heart Surgery Database can be used to estimate prognosis and council patients and their families. The six manuscripts reviewed in this article have been selected to give a flavor of the state of the art in the domain of caring for adults with
congenital heart disease and to provide important information about the long term management of patients undergoing successful pediatric cardiac surgery.