Pulmonary arteries rehabilitation and complete anatomical correction in pulmonary atresia with VSD, hypoplastic PA and MAPCAs.

Conventional treatment of pulmonary atresia with ventricular septal defect (VSD), hypoplastic pulmonary arteries (PA) and major aorto-pulmonary collat erals (MAPCAs) is controversial: from symptomatic and palliative treatment for some authors to surgery with unifocalisation of collaterals for others. These treatments never use native pulmonary arteries as only source of pul monary flow, but create. nea-pulmonary arteries,. Nine cases of pulmonary a tresia with VSD, hypoplastic PA and MAPCAs were treated by rehabilitation o f native PA through a staged approach: 1) surgical neonatal connection betw een right ventricule (RV) and hypoplastic PA, 2) evaluation and interventio nnal catheterism with angioplasty of PA stenosis and closure of collaterals , 3) complete surgical correction with reconstruction of right outflow trac k and PA and closure of VSD. After first surgical stage of RV-PA connection at the mean age of 4.8 month s (+/- 3.6 months), 8 patients were alive and underwent 22 cardiac catheter isms (mean of 2.7 per patient), with angioplasty of PA, and occlusion of MA PCAs in 6 and 2 patients respectively. Seven patients underwent complete an atomical correction at the mean age of 28.8 months (+/- 17.7 months) with o ne late death. The 6 remaining patients had encouraging hemodynamic status (RV pressure/LV pressure ratio at 0.6 +/- 0.26; mean left and right distal pulmonary pressure at 15.2 mmHg (+/- 9.1 mmHg)), and good functionnal statu s (3 in NYHA functionnal class 1, and 3 in class 2), for a mean follow-up o f 79.5 months (+/- 41.4 months). One patient had reoperation on right outfl ow track stenosis, 6 years after correction. This small series enhances the feasibility of a staged approach with rehabi litation of small PA, allowing complete surgical correction with the native PA with good hemodynamic and functional results in pulmonary atresia, with VSD, hypoplastic PA and MAPCAs.