Median sternotomy single stage complete unifocalization for pulmonary atresia, major aorto-pulmonary collateral arteries and VSD-early experience

Objective: It is a prospective study to assess the results of median sterno tomy, single stage complete unifocalization and repair for ventricular sept al defect (VSD), pulmonary atresia and major aorto pulmonary collateral art eries (MAPCAs). Methods: From June 97 to August 98, 20 patients were treate d with single stage complete unifocalization and repair. Their ages ranged from 6 months to 11 years. Through median sternotomy, all MAPCAs were disse cted and looped. On cardiopulmonary bypass, MAPCAs were anastomosed to nati ve pulmonary arteries (PAs) or to MAPCAs. VSD was closed if possible and RV to PA continuity was established with a homograft conduit. If complete rep air was not suitable, central shunt was done from ascending aorta to recons tructed PA with a polytetrafluroethylene graft. The patients were divided i nto three groups according to the arborization pattern in the lungs. Group 1 had well formed native PAs with MAPCAs, group 2 had hypoplastic PAs with MAPCAs and group 3 had only MAPCAs. Results: Twenty patients had 21 procedu res. All MAPCAs were unifocalized with tissue-to-tissue anastomosis for fut ure growth, except one in whom polytetra fluroethylene tube graft was used to attain the confluence. In group 1, all seven patients had complete unifo calization and repair. In group 2, four patients had RV to PA conduit and t wo patients had central shunt. In group 3, three patients had complete repa ir, three patients had RV to PA conduit and one patient had central shunt. There were three deaths, two in group 2 and one in group 3. The first patie nt died due to a wrong decision to close the VSD, the second patient died d ue to missed large MAPCA in preoperative angio and the third patient was a 7-year-old boy who died with irreversible pulmonary vascular changes due to unprotected MAPCAs. Conclusions: To conclude, complete repair/RV-PA condui t/central shunt should be done according to the size of the total pulmonary vasculature in patients with group 1, 2 and 3 with protected PAs/MAPCAs an d in hypoplastic or absent PAs with unprotected MAPCAs (less than 1 year) a nd protected MAPCAs. We are yet to determine the surgical procedure to be p erformed in hypoplastic/absent PAs with unprotected MAPCAs more than 1 year . It is very essential to delineate all the MAPCAs up to the level of the d iaphragm preoperatively. (C) 1999 Elsevier Science B.V. All rights reserved .