• Joseph A Dearani, Constantine Mavroudis, James Quintessenza, Barbara J Deal, Carl L Backer, Patrick Fitzgerald, Heidi M Connolly, and Jeffrey P Jacobs.
• Mayo Clinic, Rochester, Minnesota 55905, USA. dearani.joseph@mayo.edu
• Curr. Opin. Pediatr. 2009 Oct 1; 21 (5): 565-72.

Purpose Of ReviewThis article reviews three common abnormalities requiring surgical treatment in adults with congenital heart disease (CHD): Ebstein's anomaly, the failing Fontan, and pulmonary valve disease.Recent FindingsAs of 2000, more adults than children are alive with CHD. Each year, more of these adults with CHD undergo surgery. Three common operations performed on adults with CHD are surgery for Ebstein's anomaly, Fontan revision, and pulmonary valve replacement (PVR). Recent advances have been made in each of these three operations.SummarySurgery for Ebstein's anomaly can now be 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. Fontan conversion involves: conversion of the previously created atriopulmonary connection to a total cavopulmonary artery, extracardiac Fontan; 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 concomitant procedures to treat associated lesions. The need for PVR is increasing for many adults with CHD. In the past, residual defects such as chronic pulmonary regurgitation following repair of tetralogy of Fallot were considered benign. Recent evidence suggests that pulmonary regurgitation causes significant morbidity, producing right ventricular dilatation and dysfunction, exercise intolerance, arrhythmias, and sudden death. Multiple options exist for PVR including several recent developments such as PVR with a man-made polytetrafluoroethylene bicuspid valve and percutaneous PVR.

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