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).