Surgical experiences of fenestrated total cavopulmonary connection in patients under four year of age

: The fenestrated Fontan operation is an interim steps for patients who considered to be at high risk for one step Fontan procedure. We performed total cavopulmonary connection with baffle fenestration in five patients under four year of age (33 month of mean age at operation). All patients previously underwent palliative surgery (Blalock shunt in 3, PA banding in 2). Three patients weighed less than 10 kg. Pulmonary vascular resistance index (PARI) were 2.03 +/- 0.18 U/BSA and exceeded 2 U/SQm in 3 patients. Nakata's indice were ranged from 282 to 644 (mean 394) mm2/BSA. Bidirectional cavopulmonary shunt was considered to be alternative pathway for three patients in staged Fontan strategy. Postoperative hemodynamics were relatively stable in all patients and weaned from mechanical ventilation within 5 days with various arterial oxygen saturation. There was no pleural effusion lasting longer than 1 week. One patient died of ileus caused from congenital malrotation. Follow up catheter examination at 1 year showed 83 to 96% of SaO2 and survived patients were doing well. Fenestrated TCPC can be applied safely in younger patients who are considered to need surgical treatments in staged Fontan strategy. Whether fenestration should be closed has to be wait further studies.