Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy

Children with visceral heterotaxy often present with total anomalous pulmonary venous drainage (TAPVD) associated with univentricular congenital heart disease. We reviewed our experience with the primary surgical management of this lesion under these circumstances. Over a recent 10-year span, 38 patients within the first 3 days of life were admitted to our institution and underwent primary palliation. Twenty-one of them had TAPVD, 18 to a systemic vein. Twelve (67%) of these 18 were seen with obstruction of the anomalous connection and underwent emergency operation. In 7 patients, repair of TAPVD was combined with a systemic-pulmonary artery shunt because of additional obstruction of the pulmonary blood supply, with two deaths. One patient had primary shunting and then repair of TAPVD. Four patients underwent repair of TAPVD alone. Two of them then showed signs of insufficient pulmonary blood flow, received a shunt in a second procedure, and subsequently died. Early mortality in the group with obstructed TAPVD was thus 4 (33%) of 12 patients. Statistical analysis of all 38 patients (univariate analysis, chi 2 testing) showed that neither the presence of TAPVD (p = 0.7) nor TAPVD repair alone (p = 0.8) or with shunting (p = 0.8) was a definite risk factor for early death. The performance of a shunt during the first operation, however, was associated with lower early mortality (p = 0.03). Total anomalous pulmonary venous drainage is a common finding in newborns with visceral heterotaxy. Its presence and its subsequent early repair (requiring cardiopulmonary bypass) do not increase the mortality risk. The need of a concomitant shunt in obstructed TAPVD can initially be underestimated.