Multicenter Analysis of Early Childhood Outcomes Following Repair of Truncus Arteriosus

Background Literature describing morbidity and mortality following truncus arteriosus repair is predominated by single-center reports. We created and analyzed a multicenter dataset to identify risk factors for late mortality and right ventricle-to-pulmonary artery (RV-PA) conduit reintervention for this patient population. Methods We retrospectively collected data on children who underwent repair of truncus arteriosus without concomitant arch obstruction at 15 centers between 2009 and 2016. Cox regression survival analysis was conducted to determine risk factors for late mortality, defined as death occurring after hospital discharge and greater than 30 days after surgery. Probability of any RV-PA conduit reintervention was analyzed over time using Fine-Gray modelling. Results We reviewed 216 patients, with median follow-up of 2.9 years (range:0.1-8.8). Operative mortaility occurred in 15 patients (7%). Of the 201 survivors, there were 14 (7%) late deaths. DiGeorge syndrome (HR:5.4; 95%CI:1.6-17.8) and need for postoperative tracheostomy (HR:5.9; 95%CI:1.8-19.4) were identified as independent risk factors for late mortality. At least one RV-PA conduit catheterization or surgical reintervention was performed in 109 patients (median time to reintervention:23 months, range:0.3-93). Risk factors for reintervention included use of pulmonary or aortic homografts versus Contegra® bovine jugular vein conduits (HR:1.9; 95%CI:1.2,3.1) and smaller conduit size (HR per mm/m2:1.05; 95%CI:1.03,1.08). Conclusions In a multicenter dataset, DiGeorge syndrome and need for tracheostomy postoperatively were found to be independent risk factors for late mortality after repair of truncus arteriosus, while risk of conduit reintervention was independently influenced by both initial conduit type and size