BC43.6

ICD-11 MMS 2026-01

Arrhythmogenic ventricular cardiomyopathy

Description

Arrhythmogenic ventricular cardiomyopathy is a cardiomyopathy characterised by myocardial cell loss with partial or total replacement of right ventricular muscle by adipose and fibrous tissue, beginning subepicardially to become transmural in time, sparing the papillary muscles and trabeculae, and often associated with aneurysms particularly of the right ventricular outflow tract. There is progressive systolic impairment with ventricular dilation and marked propensity for ventricular arrhythmias of right, as well as left, ventricular origin. Classically a disease of the right ventricle, more recent evidence suggests left ventricular involvement to a varying extent in up to 75% of cases, as well as isolated left ventricular disease.

Index terms

  • Arrhythmogenic ventricular cardiomyopathy
  • arrhythmogenic ventricular dysplasia
  • Arrhythmogenic ventricular cardiomyopathy due to desmosomal mutation
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  • Arrhythmogenic ventricular cardiomyopathy with right ventricular dominance
  • ARVC - [Arrhythmogenic right ventricular cardiomyopathy]
  • ARVD - [Arrhythmogenic right ventricular dysplasia]
  • arrhythmogenic right ventricular cardiomyopathy
  • arrhythmogenic right ventricular dysplasia
  • Familial isolated arrhythmogenic right ventricular dysplasia
  • Naxos disease
  • Keratosis palmoplantaris with arrhythmogenic cardiomyopathy
  • Arrhythmogenic ventricular cardiomyopathy with left ventricular dominance
  • arrhythmogenic left ventricular cardiomyopathy
  • arrhythmogenic left ventricular dysplasia
  • Woolly hair – palmoplantar keratoderma – dilated cardiomyopathy
  • Carvajal syndrome
  • Carvajal-Huerta syndrome

Exclusions from above levels

  • BC42 – Myocarditis
  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 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

  • BC43.0 – Dilated cardiomyopathy
  • BC43.1 – Hypertrophic cardiomyopathy
  • BC43.2 – Restrictive cardiomyopathy
  • BC43.3 – Endocardial fibroelastosis
  • BC43.4 – Cardiomyopathy due to drugs or other external agents
  • BC43.5 – Stress-induced cardiomyopathy
  • BC43.7 – Diabetic cardiomyopathy
  • BC43.Y – Other specified cardiomyopathy
  • BC43.Z – Cardiomyopathy, unspecified

Foundation entity

Arrhythmogenic ventricular cardiomyopathy – http://id.who.int/icd/entity/702771512

Allowed postcoordination axes

Part of: BC43: Cardiomyopathy › 11: Diseases of the circulatory system