scimitar syndrome


A rare vascular anomaly which is more common in females that may present in early childhood, in which some or all of the pulmonary veins drain into the inferior vena cava. The scimitar syndrome is often accompanied by hypoplasia of the right lung (RL), dextrocardia, and systemic arterial supply to the RL from descending aorta
Surgical anatomy RL and right pulmonary artery hypoplasia, dextrocardia, anomalous origin of pulmonary arteries from the aorta to RL, anomalous venous drainage of RL into the inferior vena cava and anomalous right diaphragm
Management Pneumonectomy or correction of cardiovascular ‘plumbing’ defects.

scimitar syndrome

A rare congenital malformation of the heart and lungs, marked by dextroposition of the heart, a malformed right lung, abnormal connections between the right pulmonary veins and the inferior vena cava, and abnormal pulmonary arterial connections to the right lung.