Preferred Label : Left heart obstruction at multiple sites;
ICD-11 definition : A congenital cardiovascular malformation in which more than one of the following lesions
are present: (1) supravalvar or intravalvar mitral ring, (2) mitral subvalvar stenosis,
(3) a parachute deformity of the mitral valve, (4) subaortic stenosis, (5) valvar
aortic stenosis, and (6) aortic coarctation. Shone's syndrome consists of multilevel
hypoplasia or obstruction of the left-heart. The syndrome is based on the original
report from Shone that was based on analysis of 8 autopsied cases and described the
tendency of these four obstructive, or potentially obstructive, conditions to coexist.
Only 2 of the 8 cases exhibited all four conditions, with the other cases exhibiting
only two or three of the anomalies.;
ICD-11 synonym : Shone's anomaly; Shone syndrome; Shone's complex; Shone's disease;
Origin ID : 295410302;
Automatic exact mappings (from CISMeF team)
A congenital cardiovascular malformation in which more than one of the following lesions
are present: (1) supravalvar or intravalvar mitral ring, (2) mitral subvalvar stenosis,
(3) a parachute deformity of the mitral valve, (4) subaortic stenosis, (5) valvar
aortic stenosis, and (6) aortic coarctation. Shone's syndrome consists of multilevel
hypoplasia or obstruction of the left-heart. The syndrome is based on the original
report from Shone that was based on analysis of 8 autopsied cases and described the
tendency of these four obstructive, or potentially obstructive, conditions to coexist.
Only 2 of the 8 cases exhibited all four conditions, with the other cases exhibiting
only two or three of the anomalies.
