Total anomalous pulmonary venous connection: Results of surgical repair of 100 patients at a single institution

ObjectiveSurgical repair of total anomalous pulmonary venous connection is associated with significant mortality and morbidity, especially in patients with single-ventricle physiology. This study analyzes total anomalous pulmonary venous connection surgical repair results at one institution to identify trends and indicators of positive outcome.MethodsOur cardiac surgery database identified 100 patients undergoing surgical repair of total anomalous pulmonary venous connection (1990–2008): supracardiac (52), cardiac (15), infracardiac (23), and mixed (10). The median age at repair was 14.6 days (range, 0–4 years), and the median weight was 3.5 kg (range, 1.3–15 kg). Patients were divided into 2 groups: biventricular (n = 83) or single-ventricle (n = 17) physiology. All but 1 of the patients with single-ventricle physiology had heterotaxy syndrome (94%), and 13 of 17 patients had supracardiac anatomy.ResultsThere were 12 operative deaths (4 in the biventricular group [5%] and 8 in the single-ventricle group [47%], P