COMPLETE REPAIR OF PA-VSD WITH DIMINUTIVE OR DISCONTINUOUS PULMONARY-ARTERIES BY TRANSVERSE THORACOSTERNOTOMY

Background. Optimal treatment and the optimal sequence of surgical and interventional steps to correct pulmonary atresia with ventricular se ptal defect and hypoplastic or discontinuous intrapericardial pulmonar y arteries is still under discussion. Collateral arteries may be hardl y accessable through median sternotomy at total correction. Bilateral transsternal thoracotomy gives wide access to the heart, both pleural spaces and hilar structures. Methods. We used this incision for total correction of pulmonary atresia with ventricular septal defect in 6 pa tients. Three had Blalock-Taussig shunts placed previously, and intrap ericardial pulmonary arteries were absent in all patients but 1, in wh om they were hypoplastic. Central pulmonary arteries were enlarged wit h pericardial patches or replaced with tube grafts; the number of unif ocalized collateral arteries varied between two and eight. Results. On e patient died of respiratory failure and sepsis (16.7%). Oxygen satur ation increased from 76% (range, 65% to 88%) preoperatively to 96% (ra nge 91% to 99%) postoperatively. Mean postoperative pulmonary artery p ressure was 30 mm Hg (range, 28 to 34 mm Hg). One patient had to be re operated on through the same incision due to scarring and shrinkage of the peripheral anastomoses. Six months after operation 2 patients are in New York Heart Association functional class I and 2 are in class I I. Conclusions. Transverse thoracosternotomy gives excellent access to the anatomical structures necessary to correct complex cases of pulmo nary atresia with ventricular septal defect and may reduce the number of surgical procedures.