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.

We performed a double switch operation for the patients with corrected congenital transposition of the great arteries concomitant with intra-cardiac abnormalities including dextrocardia, non-confluent pulmonary artery and Ebstein's malformation between April 2003 and August 2006. The mean age and weight at the time of surgery were 38 months (range 2-89 months) and 10.7kg (range 4.6-16.1kg), respectively. Before the double switch operation, one patient had received a right modified BT shunt as a neonate and another had received bilateral modified BT shunts at the age of one month and 2 months respectively, followed by a central pulmonary artery angioplasty with installation of a right ventricle to a pulmonary artery shunt at the age of 5 years. For definitive repair, the Senning+Rastelli procedure was performed in two patients and Senning+Jatene procedure was performed in one patient. Mitral valve-and tricuspid valve plasties were performed, the atrialized right ventricle was plicated in the patient with Ebstein's malformation during the double switch operation. A Senning procedure was performed in patients with apicocaval juxtapositions. We reconstructed the systemic venous chamber with a dog-ear-like structure made from suture line pouches at the site of upper and lower portions of the atrial free wall, and the pulmonary venous chamber was completed, without augmentation with additional material. The mean surgery, cardiopulmonary bypass-and aortic cross clamp times were 606, 318 and 151 min, respectively. Postoperative CT scans showed smooth systemic venous returns and no pulmonary vein obstruction. No arrhythmias of any kind were detected after the double switch operation. These results suggest the suture line pouch technique in the atrial switch operation is useful in the double switch operation.