If you have feedback, let us know.

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 MMS LA88.Y
ICD-11 Code 295410302

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.

Synonyms/Abbreviations

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

Parent

Congenital left ventricular anomaly

Siblings

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.