A familial variant of the Scimitar syndrome with a meandering pulmonary vein.

We report a rare variant of the Scimitar syndrome, in which the right lower pulmonary vein takes a meandering course before finally connecting in normal fashion to the left atrium. The pulmonary parenchymal segment drained by the tortuous vein is supplied by a systemic collateral artery, which was coiled via a catheter. We also closed the intracardiac defects by surgery. The elder brother of the patient had classical Scimitar syndrome.