Pulmonary Atresia and Ventricular Septal Defect: A Therapeutic Challenge in Pediatric Cardiology and Cardiac Surgery

From 1986 to 1997, 41 newborn infants and 19 children (1–6 years) with pulmonary atresia and ventricular septal defect were treated at the Heart Center North Rhine-Westphalia. The infants' birth weights ranged from 1140 g to 4100 g (mean 2990 g). Eighteen of these 60 patients had significant additional cardiovascular malformations and they were excluded from this study. The results of surgical treatment in the remaining 42 patients are presented and discussed here. Twenty patients had a main pulmonary arterial trunk with confluent branch pulmonary arteries and nine had an atretic trunk with confluent pulmonary arteries. In nine patients without confluence, one branch pulmonary artery originated normally from the main pulmonary trunk and the other originated abnormally. In four patients the intrapericardial vessels were completely missing. The Nakata index measured by cineangiography ranged from 50 to 240 mm2/m2 (mean 150 mm2/m2). All newborns with a patent ductus arteriosus received prostaglandin intravenously. In eight of them oral prostaglandin was used to provide long-term stability before surgery. A total of 65 surgical aortopulmonary anastomoses were created, primary repair was carried out in six patients, and secondary corrective surgery was performed in 21 patients. After the first anastomosis, oxygen saturations increased by 5–15% (mean 8%), depending on the pulmonary vascular pathology. When a second shunt was created in the same patient, the increase in oxygen saturations ranged from none to 7%. After a mean interval of four years following the last operation, inspiratory vital capacity and intrathoracic gas volume were measured in 22 patients. Values of both studies decreased progressively as more thoracotomies were performed. All 42 patients were treated surgically and 13 died (31%). A primary or secondary repair was performed in 27 with 23 survivors (85%), and 15 had palliative procedures with six survivors (40%). Twenty of the 29 survivors (69%) are in a good clinical condition.