Shone’s Complex

Shone’s complex, as it was originally described, consists of a constellation of four left heart obstructive lesions: supravalvular mitral ring, parachute mitral valve, subaortic stenosis, and coarctation of the aorta. Given the rarity of having all four components simultaneously present as a complete complex, the term is often used in a more generic manner to describe a cardiac defect in which two or more features of left heart obstruction are found. Depending upon which lesions are present and the degree to which they are obstructive, Shone’s complex can present with a wide spectrum of pathophysiology and clinical manifestations. Therefore, each patient should be individually evaluated, with diagnosis primarily made by echocardiography to guide medical and surgical management. Outcomes and prognoses are generally good in the short and long term, and continue to improve with time, although they are heavily dependent on the type of lesions involved.