Scimitar syndrome: the curved Turkish sabre

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2014;17(1):56-61. doi: 10.1053/j.pcsu.2014.01.003.


Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies characterized by an anomalous pulmonary vein (scimitar vein) that drains into the inferior vena cava, a hypogenetic right lung, and dextroposition of the heart. It has been reported in 3% to 6% of patients with partial anomalous pulmonary venous connection. Patients are either diagnosed early with severe symptoms (infantile type) or late with minimal symptoms (childhood/adult type). In this chapter we reviewed the history, pathophysiology, presentation, diagnosis, and treatment methods of Scimitar syndrome in the current era.

Publication types

  • Review

MeSH terms

  • Adult
  • Cardiac Surgical Procedures / methods*
  • Diagnosis, Differential
  • Diagnostic Imaging
  • Humans
  • Infant, Newborn
  • Scimitar Syndrome / diagnosis
  • Scimitar Syndrome / physiopathology
  • Scimitar Syndrome / surgery*