Persistent primitive hepatic venous plexus with Scimitar syndrome: Description of a case and review of the literature

Abstract

Persistent primitive hepatic venous plexus is an anomaly of the systemic venous return characterised by postnatal persistence of the foetal intrahepatic venous drainage. Scimitar syndrome is a condition that consists of partial anomalous pulmonary venous return of the right pulmonary venous drainage into the systemic veins, associated with pulmonary artery hypoplasia with the underdeveloped right lung, pulmonary sequestration, and cardiac malposition. Both conditions are rare and together have been rarely described in the literature. We report the first case of this combination of lesions imaged by cardiac magnetic resonance imaging with a three-dimensional reconstruction and reviewed the literature to characterise this uncommon combination.