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

Abstract

Surgical 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. Our 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. There were 12 operative deaths (4 in the biventricular group [5%] and 8 in the single-ventricle group [47%], P < .01) and 9 late deaths (6 in the biventricular group [7%] and 3 in the single-ventricle group [18%], P < .05). Death by total anomalous pulmonary venous connection type was supracardiac (12/52; 23.1%), cardiac (1/15; 6.7%), infracardiac (3/23; 13.0%), and mixed (5/10; 50%). Pulmonary venous obstruction was present in 22 patients in the biventricular group (27%) and in 7 patients in the single-ventricle group (41%; P = .25). Mortality was 9 of 29 (31%) in those with pulmonary venous obstruction and 12 of 71 (17%) in those with nonpulmonary venous obstruction (P = .23). Deep hypothermic circulatory arrest was used in 38 patients (27 in the biventricular group, 32.5%; 11 in the single-ventricle group, 64.7%). Mean deep hypothermic circulatory arrest time was 31.4 +/- 10.7 minutes (P = not significant between groups). Median postoperative length of stay was 11 days (range, 0-281 days). Nineteen patients required reoperation for pulmonary venous stenosis (14 in the biventricular group and 5 in the single-ventricle group. P = .045); the median time to reoperation was 104 days (range, 4-753 days). Patients with total anomalous pulmonary venous connection with biventricular anatomy have good outcomes. Patients with single-ventricle anatomy have higher mortality and increased risk for pulmonary vein stenosis requiring reoperation. Mortality is highest in patients with mixed-type total anomalous pulmonary venous connection.