PM1004: Bilateral Ductus Arteriosus with Ipsilateral Branch Pulmonary Artery Insertion in a Patient with Malalignment Ventricular Septal Defect, Double Outlet Right Ventricle and Pulmonary Atresia

Introduction: Bilateral ductus arteriosus is a very rare congenital cardiac anomaly. It is usually associated with other intracardiac abnormalities like pulmonary atresia, ventricular septal defect (VSD), non-confluent branch pulmonary arteries, and the right aortic arch.

Case Description: A newborn with a prenatal diagnosis of complex congenital heart disease. Postnatal transthoracic echocardiogram showed malalignment VSD, double outlet right ventricle with dextro-malposed great arteries (right ventricular aorta) and pulmonary atresia with patent ductus arteriosus dependent pulmonary circulation. Advanced imaging with cardiac computed tomography (CT) was performed to further evaluate branch pulmonary artery and ductal anatomy, which showed bilateral ductus arteriosus with insertion to ipsilateral branch pulmonary arteries (Figure 1). He underwent complete surgical repair with Rastelli procedure (7 mm right ventricle to main pulmonary artery femoral vein valved conduit placement and baffling of the VSD to the aorta), and bilateral ducti ligation. Follow-up cardiac CT was performed due to concern for branch pulmonary stenosis which showed mildly hypoplastic right pulmonary artery. (Figure 2). Clinically patient is doing well at the time of this report.

Discussion: Bilateral patent ductus arteriosus is an uncommon cardiac anomaly hypothesized to be from abnormal embryonic resorption of the aortic arch. To the best of our knowledge, there were only two cases reported in the literature of bilateral ductus arteriosus in patients with double outlet right ventricle and pulmonary atresia.

Conclusion: We report such an extremely rare case of bilateral patent ductus arteriosus inserting to ipsilateral branch pulmonary arteries in a patient with double outlet right ventricle and pulmonary atresia with confluent branch pulmonary arteries.