Shone's syndrome: Insights from three-dimensional echocardiography

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.