Management of late complications after classic Fontan procedure by conversion to total cavopulmonary connection

Classic Fontan procedures, such as atriopulmonary connection or atrioventri cular connection, are associated with right atrial dilatation and several l ate complications. Seven patients with univentricular heart who presented w ith exercise intolerance (n = 7), severely dilated right atrium (n = 7) wit h pulmonary vein compression (n = 3), atrial arrhythmias resistant to medic al treatment (n = 4), cyanosis (n = 4), diffuse effusions and oedema (n = 1 ), and protein-losing enteropathy (n = 1), underwent conversion to total ca vopulmonary connection 5.8-14.4 years after a previous atriopulmonary conne ction (n = 6) or atrioventricular connection (n = 1). A 14-year-old boy who , preoperatively, was in ventricular failure and a very poor state died ear ly after conversion because of low cardiac output. All survivors had either marked or partial clinical improvement with regression of cardiomegaly, ab sence of pulmonary vein compression or cyanosis, and recovery of sinus rhyt hm. Conversion to total cavopulmonary connection appears to be effective in the treatment of late complications after classic Fontan procedures. It sh ould be considered early in symptomatic patients, before significant ventri cular dysfunction and clinical deterioration ensue. (C) 1999 Published by E lsevier Science Ltd on behalf of The International Society for Cardiosvacul ar Surgery. All rights reserved.