Left heart obstruction at multiple sites (Q105073)

A congenital cardiovascular malformation in which more than one of the following lesions are present in the normally connected heart: (1) supravalvar or intravalvar mitral ring, (2) mitral valvar stenosis, (3) mitral subvalvar stenosis, including a parachute deformity of the mitral valve, (4) subaortic stenosis, (5) valvar aortic stenosis, (6) supravalvar aortic stenosis and (7) aortic coarctation. Additional information: Left heart obstruction at multiple sites, also known as Shone syndrome, consists of multilevel hypoplasia or obstruction of left heart structures. This entity is based on the original report from Shone (1963) that analysed 8 autopsied cases and described the tendency of multiple obstructive, or potentially obstructive, conditions to coexist. Only 2 of the 8 cases exhibited four conditions, with the other cases exhibiting only two or three of the anomalies.