BC44

ICD-11 MMS 2026-01

Noncompaction cardiomyopathy

Description

Noncompaction cardiomyopathy is a morphologic abnormality of the myocardium predominantly affecting the apex of the ventricle characterised by hypertrabeculation and deep inter-trabecular recesses, usually accompanied by an abnormally thin subepicardial layer of compacted myocardium, that is generally but not always associated with ventricular dysfunction. Additional information. Noncompaction cardiomyopathy classically involves the left ventricle but can also involve only the right ventricle or both. It can occur as an isolated finding or in association with a dilated, hypertrophic, or mixed cardiomyopathic phenotype. It has been described in association with complex congenital heart disease, coronary artery anomalies and as an isolated finding, with and without musculoskeletal and other system abnormalities.

Index terms

  • Noncompaction cardiomyopathy
  • Left ventricular noncompaction
  • Familial-genetic noncompaction cardiomyopathy
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  • Sarcomeric noncompaction cardiomyopathy
  • X-linked noncompaction cardiomyopathy
  • Noncompaction cardiomyopathy due to Barth syndrome
  • Noncompaction cardiomyopathy due to laminopathy
  • Noncompaction cardiomyopathy due to Z-line protein/Cypher mutation
  • Noncompaction cardiomyopathy due to Z-line protein/Cypher mutation (Zaspopathy)
  • Left ventricular noncompaction cardiomyopathy
  • Right ventricular noncompaction cardiomyopathy
  • Biventricular noncompaction cardiomyopathy
  • Noncompaction cardiomyopathy associated with congenital heart disease

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations and chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • BC40 – Acquired atrial abnormality
  • BC41 – Acquired ventricular abnormality
  • BC42 – Myocarditis
  • BC43 – Cardiomyopathy
  • BC45 – Cardiomegaly
  • BC46 – Intracardiac thrombosis
  • BC4Y – Other specified diseases of the myocardium or cardiac chambers
  • BC4Z – Diseases of the myocardium or cardiac chambers, unspecified

Foundation entity

Noncompaction cardiomyopathy – http://id.who.int/icd/entity/226977635

Allowed postcoordination axes

Part of: 11: Diseases of the circulatory system