Modified Fontan conversion: an alternative technique for grown-up patients

Method A 28-year-old woman (tricuspid atresia/Blalock-Taussing shunt) underwent atriopulmonary Fontan procedure at the age of 3. At 25-year follow-up, she developed a progressive worsening in functional class and palpitations, despite antiarrhythmic treatment. Imaging studies showed no stenosis in the atriopulmonary connection. She underwent Fontan conversion. The right atrium (RA) was extremely dilated and the right pulmonary artery (RPA) was noted to have a striking small diameter/ short length along with a dilated IVC. The RA was downsized, the atriopulmonary Fontan was disconnected and cryo-Cox-Maze IV procedure was carried-out. After cooling the patient to 28°C, ventricular fibrillation was induced (12 minutes) and carbon dioxide field flooding was employed to create an atrial septal defect. The RA was closed and the TCPC was performed (24-mm Goretex conduit): the proximal end-to-end anastomosis to the inferior-venae-cavae was done. The distal anastomosis (conduit-to-RPA) was desestimated due to the size mismatch. An end-to-end anastomosis between the main PA and the conduit was then done followed by the end-toside anastomosis of the superior-venae-cavae (SVC) to the conduit. The patient was weaned from cardiopulmonary bypass (307 minutes) uneventfully. Epicardial leads and dual-chamber pacemaker were implanted.