Palliation of Truncus Arteriosus Associated With Complete Atrioventricular Canal--Results of Single Ventricle Palliation.

ABSTRACT

BACKGROUND: Truncus arteriosus associated with complete atrioventricular canal defect is rare and continues to be a surgical challenge with high morbidity and mortality. In the absence of extension of the ventricular septal defect to the outlet septum, biventricular repair is precluded, and single ventricle palliation remains the only option. We present our experience with five patients with truncus arteriosus and complete atrioventricular canal defect who underwent single ventricular palliation. METHODS: Five patients with truncus arteriosus and complete atrioventricular canal defect managed along the single ventricle palliation pathway were retrospectively reviewed. Demographic, echocardiographic, cardiac catheterization, and perioperative data were analyzed. RESULTS: All patients underwent neonatal palliation tailored to their anatomy (excision of pulmonary arteries from the common trunk and systemic to pulmonary artery shunt in two patients, excision of pulmonary artery and right ventricle to pulmonary artery conduit in two patients, and bilateral branch pulmonary artery bands in one patient). There were two early deaths after neonatal palliation. At a median follow-up of 210 days (interquartile range 1,210 days), all three survivors have undergone second-stage palliation. Of these patients, one is interstage II to III, and two patients have completed their Fontan procedure and are doing well. CONCLUSION: Truncus arteriosus with complete atrioventricular canal defect is a rare combination of defects. Single ventricle palliation pathway with a tailored neonatal approach may be employed for patients with uncommitted ventricular septal defects.