• Daniel J DiBardino and Jeffrey P Jacobs.
• Division of Cardiothoracic Surgery, San Diego School of Medicine, University of California, La Jolla, California.; Rady Children׳s Heart Institute, San Diego, California.
• Semin. Thorac. Cardiovasc. Surg. 2014 Jan 1;26(2):132-44.

AbstractAs 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.Copyright © 2014. Published by Elsevier Inc.

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