Abstract

Between August 1999 and December 2007, 72 consecutive patients with single ventricle physiology underwent a modified Fontan procedure after a bidirectional Glenn shunt using an extracardiac polytetrafluoroethylene conduit without fenestration. Nitric oxide gas inhalation was commenced just after cardiopulmonary bypass together with intravenous phosphodiesterase III inhibitor administration. After oral intake was started, pulmonary vascular dilators such as beraprost, sildenafil, bosentan were given orally according to amount of chest drainage and patient's condition. After discharge, oxygen therapy at home was continued for three months. No hospital death occurred after surgery. All patients were followed by our institute and follow-up period was 44.2+/-26 (36-106.8) months. One late death occurred during this follow-up period after re-operation. Cardiac catheterization after the Fontan completion showed transpulmonary gradient of 5.9+/-2.4 mmHg, systemic output of 3.4+/-2.1 l/min m2. Arterial oxygen saturation (SaO(2)) at the latest outpatient visit was 94.4+/-3.8%. According to our clinical experience with two-staged total cavopulmonary connection using an extracardiac conduit without fenestration, fenestration in the Fontan circuit is not necessary when performing the Fontan completion. Two-staged extracardiac total cavopulmonary connection without fenestration can be satisfactorily completed with the aid of pulmonary vasodilation therapy.

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