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

Abstract

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.