Obligatory Glenn shunt in fenestrated Fontan

Five high-risk patients undergoing the Fontan operation required large fenestration (1 cm) because of high central venous pressure and low cardiac output. Because of major arterial desaturation, obligatory Glenn shunts were performed. Three patients had pulmonary atresia, 1 had tricuspid atresia 1-B, and the fifth had single ventricle with subaortic stenosis. The age ranged from 16 to 40 months (mean age, 25 +/- 9 months) and weight from 7.9 to 14.6 kg (mean weight, 11 +/- 2 kg). One patient had single and 3 had bilateral subclavian pulmonary artery shunts. The fifth patient had pulmonary artery banding and coarctation repair followed by an aortopulmonary window and central shunt. The first 2 patients repeatedly had to go back on cardiopulmonary bypass for a larger fenestration and subsequently had an obligatory Glenn shunt because of arterial desaturation. The last 3 patients had planned obligatory Glenn shunt and large fenestration. The first patient died on the second postoperative day of a combination of prolonged operation, repeated cardiopulmonary bypass, and periods of hemodynamic instability. Three patients had closure of the adjustable fenestration under local anesthesia at 4, 5, and 8 weeks postoperatively. The last patient is awaiting closure. We believe that in certain high-risk patients, a large fenestration combined with an obligatory Glenn shunt should be considered to minimize repeated cardiopulmonary bypass and urgent tightening or closure of fenestration in the immediate postoperative period.