Neonatal right ventricle to pulmonary connection as a palliative procedure for pulmonary atresia with ventricular septal defect or severe tetralogy of Fallot

Right ventricle to pulmonary artery connection (RVPA connection) without prosthetic material has been our ideal strategy to palliate pulmonary atresia with ventricular septal defect (VSD) or severe tetralogy of Fallot for the last decade. We speculate that RVPA connection ensures adequate postoperative haemodynamics for symptomatic neonates and promotes pulmonary artery rehabilitation. The present study was undertaken to assess the outcome of this strategy. Between 2000 and 2010, among 107 patients who benefited from an RVPA connection, 57 were neonates. Forty-eight of these underwent autologous tissue reconstruction, 5 using left atrial appendage. Median weight was 2.9 kg (range 1.8-4.4). Median Nakata index was 100 mm(2)/m(2) (range 17-185 mm(2)/m(2)); 12% had major aortopulmonary collaterals. All patients were reviewed retrospectively. End-points were death or complete repair; reintervention for restrictive pulmonary blood flow was considered as failure. At follow-up, we evaluated reintervention after complete repair, and quality of life. There were 2 early deaths (RV hypoplasia and RV failure) and 3 late sudden deaths (range 3-6 months). Pulmonary blood flow required to be increased in 8 patients: 4 underwent shunt after a median delay of 1 month; RVPA connection enlargement was needed in 3; 1 patient had percutaneous angioplasty. Finally, 47 patients (81%) had a complete repair, of which 70% were performed without prosthetic material at a median age of 7 months (range 2-53), with a median Nakata index of 221 mm(2)/m(2) (range 102-891). One patient died early and 1 was a failure with opening of the VSD after intracardiac repair. At last follow-up, 4 patients were still awaiting repair, with 1 late death and 5 who had required reintervention after intracardiac repair; there were 3 conduit replacements and 2 balloon dilatation patch enlargements. The neonatal RVPA connection approach (i) provides an acceptable survival rate with a satisfactory haemodynamic adaptation, (ii) facilitates rehabilitation of PAs and (iii) avoids the use of prosthetic graft at correction.