BC43.10

ICD-11 MMS 2026-01

Familial-genetic hypertrophic cardiomyopathy

Description

Familial isolated hypertrophic cardiomyopathy is the presence of non-syndromic hypertrophic cardiomyopathy in multiple members of a pedigree, or in the presence of a genetic mutation known to be significantly associated with hypertrophic cardiomyopathy.

Index terms

  • Familial-genetic hypertrophic cardiomyopathy
  • Familial hypertrophic cardiomyopathy
  • Hereditary ventricular hypertrophy
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  • Hypertrophic cardiomyopathy due to disorder of fatty acid metabolism
  • Fatty acid oxidation or ketogenesis disorder with hypertrophic cardiomyopathy
  • Hypertrophic cardiomyopathy due to carnitine palmitoyl transferase II deficiency
  • Hypertrophic cardiomyopathy due to Carnitine-acylcarnitine translocase deficiency
  • Hypertrophic cardiomyopathy due to carnitine deficiency
  • Hypertrophic cardiomyopathy due to glycogen storage disease
  • Glycogen storage disease with hypertrophic cardiomyopathy
  • Hypertrophic cardiomyopathy due to AMP-activated protein kinase deficiency
  • Hypertrophic cardiomyopathy due to PRKAG2 syndrome
  • Hypertrophic cardiomyopathy due to Pompe disease
  • Hypertrophic cardiomyopathy due to glycogen debranching enzyme deficiency
  • Hypertrophic cardiomyopathy due to LAMP2 deficiency disease
  • Hypertrophic cardiomyopathy due to lysosomal storage disease
  • Lysosomal disease with hypertrophic cardiomyopathy
  • Hypertrophic cardiomyopathy due to Anderson–Fabry disease
  • Hypertrophic cardiomyopathy due to Hurler disease
  • Hypertrophic cardiomyopathy due to Hurler disease (MPS I)
  • Hypertrophic cardiomyopathy due to mitochondrial disorder
  • Mitochondrial disease with hypertrophic cardiomyopathy
  • Hypertrophic cardiomyopathy due to MELAS
  • Hypertrophic cardiomyopathy due to MELAS - [Mitochondrial Encephalopathy, Lactic acidosis, and Stroke-like episodes]
  • Hypertrophic cardiomyopathy due to Friedreich's ataxia
  • Other specified familial diseases associated with hypertrophic cardiomyopathy
  • Hypertrophic cardiomyopathy due to primary amyloidosis
  • Sarcomeric hypertrophic cardiomyopathy
  • Syndromic hypertrophic cardiomyopathy
  • Hypertrophic cardiomyopathy due to craniofacialcutaneous syndrome
  • Hypertrophic cardiomyopathy due to Noonan syndrome
  • Hypertrophic cardiomyopathy due to Noonan syndrome with multiple lentigines
  • Hypertrophic cardiomyopathy due to LEOPARD syndrome
  • Hypertrophic cardiomyopathy due to Costello syndrome
  • Hypertrophic cardiomyopathy due to Beckwith–Wiedemann syndrome
  • Hypertrophic cardiomyopathy due to Swyer's syndrome
  • Hypertrophic cardiomyopathy due to Leprechaunism
  • Hypertrophic cardiomyopathy due to Donohue 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

  • BC43.11 – Non-obstructive hypertrophic cardiomyopathy
  • BC43.12 – Obstructive hypertrophic cardiomyopathy
  • BC43.1Y – Other specified hypertrophic cardiomyopathy
  • BC43.1Z – Hypertrophic cardiomyopathy, unspecified

Foundation entity

Familial-genetic hypertrophic cardiomyopathy – http://id.who.int/icd/entity/1408928442

Part of: BC43.1: Hypertrophic cardiomyopathy › BC43: Cardiomyopathy › 11: Diseases of the circulatory system