Total Anomalous Pulmonary Venous Connection: An Analysis of Current Management Strategies in a Single Institution

Background Repair of total anomalous pulmonary venous connection (TAPVC) continues to be associated with significant mortality. We reviewed patients undergoing consecutive TAPVC repairs over a 10-year period at Childrenʹs Hospital Boston. The impact of current surgical and perioperative management strategies on short-term outcomes (postrepair pulmonary venous obstruction and mortality) is evaluated. Methods All patients with surgically corrected TAPVC from November 1989 to December 2000 were included. Charts were reviewed for patient demographics, operation variables, and postoperative course. Results There were 123 patients in the cohort, of which 72 (59%) were male. The median age and weight at operation were 10 days and 3.6 kg, respectively. Sixty-eight (55%) patients presented with pulmonary venous obstruction, and 65 (53%) underwent emergent TAPVC repair. Thirty-nine (32%) had single-ventricle anatomy, and 84 (68%) had two-ventricle anatomy. Thirty patients (24%) died. Kaplan-Meier survival at 1 month was 65% (95% confidence interval [CI], 55% to 75%) for single-ventricle patients versus 90% (95% CI, 90% to 100%) for two-ventricle patients; at 36 months it was 47% (95% CI, 35% to 59%) versus 87% (95% CI, 81% to 93%), respectively. By Cox multivariable regression analysis, a single ventricle (p< 0.001, hazard ratio, 4.8; 95% CI, 2.5 to 9.2) was an independent mortality risk factor. Prerepair pulmonary venous obstruction was a multivariate risk factor for death among single-ventricle patients. Postrepair pulmonary venous obstruction occurred in 11%. If year of operation is used as a predictor, two-ventricle patient survival has significantly improved (p< 0.05). Conclusions Despite current interventions, single-ventricle patients continue to have a worse prognosis than two-ventricle patients.