Sinus venosus ASD with anomalous right superior pulmonary vein and pulmonary hypertension

This 23-years old lady presented with pulmonary hypertension. An echocardiogram suggested a shunt, but the site of the shunt could not be located. A cardiac CT was then performed.

It showed evidence of pulmonary hypertension in the form of dilatation of the pulmonary arteries (Fig. 1) and distension of the RA and RV (Fig. 2). A sinus venosus ASD was exquisitely visualized (Fig. 3) along with anomalous drainage of the right superior pulmonary vein into the SVC (Fig. 4).

The two entities always go together and it is the de-roofing of the sinus venosus by the anomalous drainage of the right superior pulmonary vein that leads to the sinus venosus ASD. This case was similar in almost all respects to the one published earlier.