Shone's syndrome: Insights from three-dimensional echocardiography

Echocardiography. 2018 Mar;35(3):417-419. doi: 10.1111/echo.13787. Epub 2017 Dec 22.

Abstract

Shone's syndrome is a rare congenital anomaly defined as the presence of at least two of the following heart obstructions: a mitral supravalvular ring, a "parachute" mitral valve stenosis, subaortic stenosis, and aortic coarctation. A 58-year-old man presented with a mitral ring and a "parachute" mitral valve on two-dimensional transthoracic echocardiography, raising suspicion of Shone's syndrome. Three-dimensional transesophageal echocardiography revealed a subannular mitral ring inserted directly on the mitral leaflets, thus acting as a "valvar ring." This distinction can have therapeutic implications as a "valvar" mitral ring could require valve repair or replacement, instead of simple resection.

Keywords: congenital heart defects; mitral valve obstruction; three-dimensional echocardiography.

Publication types

  • Case Reports

MeSH terms

  • Diagnosis, Differential
  • Echocardiography, Three-Dimensional / methods*
  • Echocardiography, Transesophageal / methods*
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / diagnostic imaging*
  • Humans
  • Male
  • Middle Aged
  • Mitral Valve / abnormalities*
  • Mitral Valve / diagnostic imaging*
  • Mitral Valve Stenosis / complications
  • Mitral Valve Stenosis / diagnostic imaging
  • Syndrome
  • Tachycardia, Supraventricular / complications
  • Tachycardia, Supraventricular / diagnostic imaging