A central shunt to rehabilitate diminutive pulmonary arteries in patients with pulmonary atresia with ventricular septal defect

Objectives We evaluated our clinical experiences on rehabilitation of native pulmonary arteries (PAs) with a central shunt using an expanded polytetrafluoroethylene (ePTFE) tube graft in management of pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs) with diminutive PAs. Methods From March 1993 to June 2013, a total of 28 consecutive patients underwent surgery for pulmonary atresia with VSD and MAPCAs. We identified 15 patients who underwent a central shunt procedure using an ePTFE tube graft. Median age and weight at the initial operation were 1.91 months and 5.22 kg, respectively. Ligation of MAPCAs was performed in 3 patients concomitantly. The techniques used were a U-shaped central shunt in 13 patients, and a side-to-side aorto-ePTFE tube graft anastomosis in 2 patients. Results There was no surgical mortality, and no shunt occlusion. The mean follow-up duration was 70.7 ± 67.1 months. Complete repair was achieved in 13 patients (13 of 14 of the follow-up patients; 92.9%), and the remaining patient is waiting for complete repair. The median age at complete repair was 19.4 months. The mean PA index before the initial central shunt procedure was 22.7 ± 13.2 mm 2 /m 2 , which increased to 149.4 ± 86.6 mm 2 /m 2 at the final recordings before shunt division, and 185.9 ± 84.0 mm 2 /m 2 before complete repair. There were 2 late mortality cases. The overall survival rates were 92.9%, 82.5%, and 82.5%, at 1, 5, and 10 years, respectively. Conclusions A central shunt, using an ePTFE tube graft, is an effective initial palliative procedure for rehabilitation of the diminutive PAs in patients with pulmonary atresia with VSD and MAPCAs.