Repair of total anomalous pulmonary venous drainage (TAPVD) is associated with significant mortality and post-repair pulmonary vein obstruction. Patients undergoing TAPVD repair (1982-1996, n=155) were retrospectively reviewed to identify high risk subsets and patterns of post-repair pulmonary vein obstruction.

Early mortality was 25%, and 10 year actuarial survival was 59%. The site of venous connection did not predict survival (p=0.44). Univentricular hearts, and associated complex lesions were predictors of poor survival by multivariable analysis (p<0.05).

The site of venous connection was associated with pulmonary vein obstruction (p<0.02). Thirteen patients (9.7%) developed late pulmonary vein obstruction, most frequently with the infracardiac (8/51 pts: 15.7%) and the mixed subtypes (2/13 pts: 15.4%) when compared to intracardiac (2/33 pts: 6.1%) and supracardiac subtypes (1/58 pts: 1.7%). Seventeen reoperations were performed on these 13 patients with a 54% long-term survival (median follow-up: 6 yrs). Mortality was associated with bilateral obstruction (6/9 pts, p<0.05 vs unilateral). A sutureless atrial reconstruction has been successful in 2 of 3 survivors with bilateral obstruction at > 1 year follow-up.

TAPVD in association with a single ventricle or other complex lesions confers a high mortality rate. Post-repair pulmonary vein obstruction in patients with bilateral disease may be amenable to a sutureless atrial reconstruction.