Case Report:
Informed consent was obtained from the child and her parents for the
purpose of publication of her data. An 11-year-old girl presented to our
clinic with exertional dyspnea, severe central cyanosis and digital
clubbing with resting saturations of 70% while breathing room air. Her
2D echocardiography-color Doppler evaluation revealed tricuspid atresia,
pulmonary atresia, normally related great arteries, absence of
confluence with severe LPA stenosis at its origin. Her computer
tomography angiogram showed the MPA to be continuous with the RPA
without any connection to LPA with only a chord of tissue between both
ends measuring about 10 mm. The LPA was supplied by a patent ductus
arteriosus. The hilar LPA measured 14mms. (Figure 1A, 2A).