Imaging description

An 18-month-old girl had persistent shortness of breath and progressive hypoxemia. She had a complicated prior history of liver transplantation at the age of 9 months for biliary atresia accompanying left isomerism with polysplenia. She had had absence of the intrahepatic inferior vena cava (IVC) with azygos continuation, as well as absence of the intrahepatic portal vein. Her current liver function was good; she had undergone prior closure of an atrial septal defect (ASD) but had no other evidence of congenital heart disease.

The frontal chest radiograph (Fig. 27.1a) demonstrated elevation of the right hemidiaphragm (known paralysis), external pacing wires (known heart block), and a nasogatric tube in place (difficulty feeding because of shortness of breath). Sternotomy wires and multiple right upper quadrant clips were present from the prior surgeries. There was no focal abnormality but non-specific prominence of the cardiac silhouette and pulmonary vessels (right side partly obscured by pacemaker), suggestive of shunt vascularity.