Total anomalous pulmonary venous connection: Factors associated with mortality and recurrent pulmonary venous obstruction

Surgical repair of total anomalous pulmonary venous connection (TAPVC) is associated with high rates of mortality and need for reintervention. The purpose of this study was to identify variables associated with surgical mortality and, in particular, to define predictors of recurrent pulmonary venous obstruction. All patients who underwent surgical repair for TAPVC from 2005 to 2010 at a single institution were included in our analysis. Hospital course, operative data, and outpatient records were reviewed. Fifty-one patients were available for review and all were included in the analysis. Anatomic TAPVC subtypes included supracardiac 26 (51%), intracardiac 10 (19.6%), infracardiac 9 (17.6%), and mixed 6 (11.8%). Pulmonary venous obstruction was present at initial operation in 13 (25.5%) patients. Median age at repair was 18 days and median weight was 3.6 kg. Single-ventricle physiology was present in 9 (17.6%), with a diagnosis of heterotaxy syndrome in 7 (13.7%). There were 5 (9.8%) operative and 2 late deaths. Recurrent pulmonary venous obstruction requiring reintervention was found in 8 (15.7%) patients with median time to reintervention of 220 days. Obstructed TAPVC was found to be associated with surgical mortality (p = 0.01). Cardiopulmonary bypass (p = 0.02) and aortic cross-clamp times (p = 0.03) were found to be associated with increased risk for reintervention. Intraoperative transesophageal echocardiography findings of a mean confluence gradient 2 mm Hg or greater was found to be markedly associated with recurrent pulmonary venous obstruction requiring reintervention (p ≤ 0.001). Mortality after repair of TAPVC is highest in patients presenting with obstruction at time of repair. Longer cardiopulmonary bypass and cross-clamp times are associated with recurrent pulmonary venous obstruction requiring reintervention. The strongest association with need for reintervention was in patients with intraoperative transesophageal echocardiography Doppler evidence of pulmonary venous obstruction.