Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment

Ann Cardiol Angeiol (Paris). 2020 Nov;69(5):317-322. doi: 10.1016/j.ancard.2020.09.025. Epub 2020 Oct 14.

Abstract

Scimitar syndrome is a variant of partial anomalous pulmonary venous return with an aberrant vein, the Scimitar vein, draining the right lung to the inferior vena cava instead of the left atrium, resulting in a left-to-right shunt. The classic frontal radiographic finding, designated as "the scimitar sign", is of a scimitar (a Turkish sword) shaped density along the right cardiac border. The diagnosis can be made by echocardiography, and cardiac catheterisation remains the gold standard to assess the left-to-right shunt. However, the place of multimodal cardiac imaging by computed tomography and magnetic resonance imaging is increasing. We report the case of a 26 year-old man presenting with chest pain during a brief panic attack, in whom scimitar syndrome was associated with a bicuspid aortic valve, a clinical association rarely reported in the literature. CT and MRI cardiac imaging was as accurate as echocardiography and hemodynamics, particularly for shunt quantification.

Keywords: Cardiac computed tomography; Cardiac magnetic resonance imaging; Cardiopathie congénitale; Congenital cardiac anomalies; Imagerie par résonance magnétique cardiaque; Scanner cardiaque; Scimitar syndrome; Syndrome de cimeterre; Transthoracic echocardiography; Échocardiographie transthoracique.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Bicuspid Aortic Valve Disease / complications
  • Bicuspid Aortic Valve Disease / diagnostic imaging*
  • Bicuspid Aortic Valve Disease / physiopathology*
  • Cardiac Imaging Techniques
  • Humans
  • Magnetic Resonance Imaging*
  • Male
  • Multimodal Imaging
  • Scimitar Syndrome / complications
  • Scimitar Syndrome / diagnostic imaging*
  • Scimitar Syndrome / physiopathology*
  • Tomography, X-Ray Computed*