A syndrome comprising a number of congenital abnormalities of the thorax. Main features are hypoplasia of the right lung (the most constant feature) with dextroposition of the heart, and a partial or complete abnormal pulmonary venous return of the right lung in the inferior vena cava or a hepatic vein: the scimitar vein. Congenital heart defects, especially atrial septal defect, occur in fewer than half of all cases.
The principal radiological sign is an arc-like shadow, resembling the blade of a Turkish sword or scimitar, produced by the anomalous vein which courses downward parallel to the right atrium to its connection with the inferior vena cava.
Aetiology unknown. Most cases are sporadic but some are transmitted as an autosomal dominant trait. Frequency about 1 per 2-3 million. More common in females.
In 1836, Raoul Chassinat and George Cooper, independently of each other, described single cases of rare congenital malformations that included venous drainage below the diaphragm. The first report that likened the classic curved draining vein to a scimitar or Turkish sword was Halasz et al in 1956. The term «scimitar syndrome», based on the typical roentgenological findings, was introduced in 1960 by the American paediatric cardiologist Catherine A. Neill et al. The term congenital pulmonary venolobar syndrome was introduced by Benjamin Felson (1913-1988) in 1973.
This is Halasz' own description of his work on the scimitar syndrome:
"The work was heavily based on the techniques developed by Liebow to prepare corrosion casts of the heart and lungs to accurately demonstrate the distribution of the vasculature. The whole concept of foregut anomalies was not well established at that point and, I Think, the major contribution was to pull together the embryologic and clinical data into a unified concept."
- Raoul Chassinat:
Observation d’anomalies anatomiques remarquables de l’appareil circulatoire, aved hépatocèle congénitale, n’ayant donné lieu pendant la vie à aucun symptôme particulier: suivie de réflexions.
Archives générales de médecine, Paris, 1836, II: 80-91.
- George Cooper:
Case of malformation of thoracic viscera: consisting of imperfect development of right lung and transposition of the heart.
London Medical Gazette, 1836, 18: 600-602.
- N. A. Halasz, K. H. Halloran, A. A. Liebow:
Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation, Dallas, Texas, 1956, 14: 826-46.
- C. A. Neill, C. H. Ferenoz, D. C. Sabiston, H. Sheldon, in:
The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage: “Scimitar syndrome.
Bulletin of the Johns Hopkins Hospital, 1960, 107: 1-21.
- B. Felson:
Chest roentgenology. Philadelphia, Pa: Saunders, 1973: 87-92.
- Michael E. Mulligan:
History of Scimitar syndrome. Letter to the editor. Radiology, 1999, 210: 288-190.