Scimitar syndrome | definition of scimitar syndrome by Medical dictionary
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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
a radiographic artifact caused by a congenital disorder in which the right lower pulmonary vein drains into the inferior vena cava. On a chest radiograph, the abnormal vessel configuration produces a scimitar-shaped shadow.
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.
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.
References in periodicals archive
Scimitar syndrome is a very rare pathology which occurs with a rate of 2/100 000 and which presents in the infancy, childhood or adulthood with a female/male ratio of 2/1.
A novel approach to the surgical correction of scimitar syndrome.
This case demonstrates how comprehensive, non-invasive imaging has replaced invasive techniques such as cardiac catheterisation and pulmonary angiography in the diagnosis of scimitar syndrome
Thorax computed tomography revealed a connection between the inferior vena cava (IVC) and the right inferior pulmonary vein subdiaphragmatically, which was commented as the Scimitar syndrome
21 Atrial Level Shunts Including Partial Anomalous Pulmonary Venous Connection and Scimitar Syndrome