The outcome of children born with pulmonary atresia and intact ventricular septum in Sweden from 1980 to 1999

Objective-To study the outcome of all children born with pulmonary atresia and intact ventricular septum in Sweden between 1980 and 1999. Design-Retrospective study of medical records with review of the initial, preoperative angiocardiographic and echocardiographic examinations. Results-A total of 84 children were born with pulmonary atresia and intact ventricular septum, giving an incidence of 4.2 per 100 000 live births. In all, 77 were operated on with a 1-year survival rate of 75%. Thirty-six children had ventriculocoronary communications, with a 1-year survival rate of 50%. At the end of the study period, 52 children were alive, 32 with biventricular repair, and 19 with univentricular repair. Follow-up time was 14 days-20 years (median, 6 years). Statistical analysis of incremental risk factors for death showed statistical significance for low birth weight, male sex, muscular pulmonary atresia, and having a systemic-to-pulmonary shunt as the sole initial intervention. Conclusion-Complete national data of all patients born with pulmonary atresia and intact ventricular septum during 1980-1999 in Sweden revealed a total mortality in accordance with previous reports for results for surgery. Further improvements demand a thorough preoperative investigation of the cardiac anatomy, particularly of the ventriculocoronary communications, to enable right ventricular decompression whenever possible.