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 septal defect and hypoplastic or discontinuous intrapericardial pulmonary arteries is still under discussion. Collateral arteries may be hardly 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 patients. Three had Blalock-Taussig shunts placed previously, and intrapericardial pulmonary arteries were absent in all patients but 1, in whom they were hypoplastic. Central pulmonary arteries were enlarged with pericardial patches or replaced with tube grafts; the number of unifocalized collateral arteries varied between two and eight. Results. One patient died of respiratory failure and sepsis (16.7%). Oxygen saturation increased from 76% (range, 65% to 88%) preoperatively to 96% (range 91% to 99%) postoperatively. Mean postoperative pulmonary artery pressure was 30 mm Hg (range, 28 to 34 mm Hg). One patient had to be reoperated 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 II. Conclusions. Transverse thoracosternotomy gives excellent access to the anatomical structures necessary to correct complex cases of pulmonary atresia with ventricular septal defect and may reduce the number of surgical procedures.