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 Children's 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.