Obstruction of the Anomalous Pulmonary Venous Connection in Scimitar Syndrome: Progression to Spontaneous Anomalous Pulmonary Vein Atresia during Infancy without Intervention

Abstract

Scimitar, or pulmonary venolobar, syndrome is a rare form of congenital heart disease that is characterized by total or partial anomalous pulmonary venous return from the right lung into the inferior vena cava (IVC) with frequently associated right pulmonary hypoplasia. The term “scimitar syndrome” was first proposed in 1960 by Neill et al.1 due to the frequently appreciated radiological shadow caused by the anomalous pulmonary vein on chest x-ray that resembles a Middle Eastern scimitar sword. Scimitar syndrome is also characterized by cardiac dextroposition (due to right lung hypoplasia), anomalous systemic arterial blood supply to the right lower lung, and bronchial abnormalities affecting the right lung.