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IPCCC ICD-11 Congenital Heart Atlas

You are here: Left heart obstruction at multiple sites Congenital left ventricular anomaly Congenital anomaly of a ventricle or the ventricular septum Structural developmental anomaly of heart or great vessels

IPCCC Term Left heart obstruction at multiple sites
IPCCC Code 01.01.33
ICD-11 Code 295410302


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 syndrome; Shone's syndrome; Shones anomaly; Shones complex; Shones disease

STS-EACTS-derived IPCCC term

Shones syndrome

EPCC-derived IPCCC term

Left heart obstruction at multiple sites (including Shone syndrome)

ICD-11 MMS code or crossmap

LA88.Y*: This Foundation term is not in ICD-11 MMS. If coding in MMS use term “Other specified congenital anomaly of a ventricle or the ventricular septum” with code LA88.Y

ICD-10 MMS code or crossmap

Q20.9**: If coding using ICD-10, crossmap to “Congenital malformation of cardiac chambers and connections, unspecified” with code Q20.9


Congenital left ventricular anomaly


Coding Notes

Shones 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.