Case Report:
Institutional review board approval and informed consent from the parents and child were obtained for this case report. An 11-year-old girl, weighing 27 kgs, presented with exertional dyspnea and profound central cyanosis with resting saturations of 70% on room air. Her 2D echocardiography-color Doppler evaluation revealed tricuspid atresia, pulmonary atresia, normally related great arteries with severe LPA stenosis supplied by a stenotic patent ductus arteriosus (PDA). Her computed tomography angiogram confirmed the echo findings with the pulmonary end of the ductus showing significant narrowing and a bifid appearance with each limb supplying the respective branch PAs (Figures 1A, 2). The LPA measured less than 1 mm, 6 mm and 14 mm at its origin, mid segment and hilum respectively while the RPA was uniformly 13mms though its ductal communication was only around 1mm. The main pulmonary artery (MPA) was normal sized and continued as right pulmonary artery (RPA).