Absent pulmonary valve (APV) syndrome is a rare anomaly that is usually associated with tetralogy of Fallot (TOF) and causes severe respiratory distress by compression of the trachea. Mortality following surgical repair in these patients is increased, especially in infants presenting with severe respiratory compromise. Preoperative ventilator dependency and age at operation are risk factors for mortality after surgical repair. Between 1995 and 1999, 5 patients underwent surgical treatment for TOF with APV. The mean age at operation was 9 months (range: 1 to 29 months), and the mean weight at operation was 5.2kg (3.6-9.1kg). Among these patients, 3 patients presented with severe respiratory distress caused by dilatation of aneurysmal pulmonary artery (pulmonary artery index>2,000) and 2 of these patients were dependent on a respirator prior to surgical treatment. Reduction of dilated pulmonary artery, including anterior wall resection and posterior placation, was performed in all patients. In the patients with severe preoperative respiratory compromise, the right ventricular outflow tract was reconstructed with an extracardiac conduit with autologous pericardial valve leaflets to avoid pulmonary valve regurgitation after the operation and a transannular patch with a PTFE valve was used in 2 patients without respiratory compromise. One patient died suddenly 7 months after surgical intervention. Three of the surviving patients underwent a second right ventricular outflow tract reconstruction because of progressive right ventricular outflow tract stenosis. In two of these patients the right ventricular outflow tract was reconstructed with an extracardiac conduit with autologous pericardial valve leaflets at first operation. All surviving patients are well without any physical limitations and have been placed in NYHA class I. Despite the need for reoperation for right ventricular outflow tract stenosis, aggressive surgical treatment for TOF with APV has produced a satisfactory mid-term outcome.

The use of transapical aortic cannulation for arterial inflow during surgical repair of type A acute aortic dissection was evaluated. Thirty-six patients who underwent repair of type A aortic dissection were divided into 2 groups: those who underwent repair with transapical aortic cannulation (group A; 19 patients) and those who underwent repair with axillary and/or femoral artery cannulation (group C; 17 patients). Preoperative condition, estimated blood loss, transfusion requirements, and duration of the tube drainage and postoperative hospital stay did not differ in the 2 groups. Cannulations were successful in all patients, and none of the attempted inflow sites required moving to alternate sites in either group. The time to initiation of extracorporeal circulation (74.2±16.2min versus 88.8±12.5min, p=0.005) and the extracorporeal circulation time (175.2±55.5min versus 216.6±58.1min, p=0.036) was shorter in group A than in group C. However, the total operation time did not differ between the groups (309.3±112.5min in group A versus 363.4±130.9min in group C, p=0.198). All patients survived the operation, and there were no complications directly related to transapical aortic cannulation. Postoperative stroke tended to be lower in group A than in group C (5.3% versus 29.4%; p=0.081). There was 1 operative death in group A (5.3%) and 4 operative deaths in group C (23.5%) (p=0.167). These data demonstrate that the use of transapical aortic cannulation yielded more favorable results than other cannulation techniques for induction of extracorporeal circulation and for minimization of extracorporeal circulation time and postoperative morbidity. We conclude that transapical aortic cannulation represents a safe, effective and less invasive means of providing arterial inflow during cardiopulmonary bypass for patients undergoing surgical correction of type A aortic dissection.