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Shone's syndrome: Insights from three‐dimensional echocardiography

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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… Click to show full 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: three dimensional; mitral ring; echocardiography; shone syndrome

Journal Title: Echocardiography
Year Published: 2018

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