TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE IN NEWBORNS WITH VISCERAL HETEROTAXY

Children with visceral heterotaxy often present with total anomalous p ulmonary venous drainage (TAPVD) associated with univentricular congen ital heart disease. We reviewed our experience with the primary surgic al management of this lesion under these circumstances. Over a recent 10-year span, 38 patients within the first 3 days of life were admitte d to our institution and underwent primary palliation. Twenty-one of t hem had TAPVD, 18 to a systemic vein. Twelve (67%) of these 18 were se en with obstruction of the anomalous connection and underwent emergenc y operation. In 7 patients, repair of TAPVD was combined with a system ic-pulmonary artery shunt because of additional obstruction of the pul monary blood supply, with two deaths. One patient had primary shunting and then repair of TAPVD. Four patients underwent repair of TAPVD alo ne. Two of them then showed signs of insufficient pulmonary blood now, received a shunt in a second procedure, and subsequently died. Early mortality in the group with obstructed TAPVD was thus 4 (33%) of 12 pa tients. Statistical analysis of all 38 patients (univariate analysis, chi(2) testing) showed that neither the presence of TAPVD (p = 0.7) no r TAPVD repair alone (p = 0.8) or with shunting (p = 0.8) was a defini te 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 findin g in newborns with visceral heterotaxy. Its presence and its subsequen t early repair (requiring cardiopulmonary bypass) do not increase the mortality risk. The need of a concomitant shunt in obstructed TAPVD ca n initially be underestimated.