Inferior sinus venosus defect and anomalous hepatic venous return to the coronary sinus leading to an Eisenmenger syndrome.

Inferior sinus venosus defect associated with left hepatic vein drainage to the coronary sinus is an extremely rare condition. We report the case of a 41-year-old man suffering from pulmonary arterial hypertension related to this unusual CHD. Planning of heart-lung transplantation in this case required accurate anatomical description.