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Shone's syndrome: Insights from three‐dimensional echocardiography
Authors:Martín F. Vivas  María T. Politi  Laura M. Riznyk  María F. Castro  Gustavo Avegliano  Ricardo Ronderos
Affiliation:Section of Echocardiography, Cardiac Imaging Department, Cardiovascular Institute of Buenos Aires, Buenos Aires, Argentina
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
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