BC43.00

ICD-11 MMS 2026-01

Familial-genetic dilated cardiomyopathy

Description

Familial-genetic dilated cardiomyopathy is the presence of dilated cardiomyopathy that is present in multiple members of a pedigree, or in the presence of a genetic mutation known to be significantly associated with dilated cardiomyopathy. Additional information: Candidate cytoskeletal and Z disk–encoding genes, most of whom are hypothesized to lead to abnormalities in force transmission, include δ-sarcoglycan, β-sarcoglycan, desmin, lamin A/C, metavinculin, muscle LIM protein, titin, α-actinin-2, nebulette, myopalladin, and ZASP (Z band alternatively spliced PDZ domain protein)

Index terms

  • Familial-genetic dilated cardiomyopathy
  • familial congestive cardiomyopathy
  • familial dilated cardiomyopathy
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  • primary familial dilated cardiomyopathy
  • Familial isolated dilated cardiomyopathy
  • Dilated cardiomyopathy due to fatty acid oxidation disorder
  • Fatty acid oxidation or ketogenesis disorder with dilated cardiomyopathy
  • Dilated cardiomyopathy due to primary carnitine deficiency
  • Dilated-hypokinetic cardiomyopathy due to primary carnitine deficiency
  • Dilated cardiomyopathy due to malonyl-CoA decarboxylase deficiency
  • Dilated-hypokinetic cardiomyopathy due to malonyl-CoA decarboxylase deficiency
  • dilated cardiomyopathy due to malonic aciduria
  • Dilated cardiomyopathy due to mitochondrial myopathy
  • Mitochondrial dilated cardiomyopathy
  • mitochondrial disease with dilated cardiomyopathy
  • Histiocytoid cardiomyopathy
  • focal lipid cardiomyopathy
  • infantile xanthomatous cardiomyopathy
  • oncocytic cardiomyopathy
  • Dilated cardiomyopathy due to Cytochrome C oxidase deficiency
  • Dilated cardiomyopathy due to Barth syndrome
  • Dilated cardiomyopathy due to Kearns–Sayre syndrome
  • Dilated cardiomyopathy due to MELAS
  • Dilated cardiomyopathy due to MELAS - [Mitochondrial Encephalopathy, Lactic acidosis and Stroke-like episodes]
  • Dilated cardiomyopathy due to MERRF
  • Dilated cardiomyopathy due to MERRF - [myoclonic epilepsy with ragged-red fibers]
  • Dilated cardiomyopathy due to neuromuscular disease
  • Neuromuscular disease with dilated cardiomyopathy
  • Dilated cardiomyopathy due to dystrophinopathy
  • Dilated cardiomyopathy due to Duchenne muscular dystrophy
  • Dilated cardiomyopathy due to Becker muscular dystrophy
  • Dilated cardiomyopathy due to laminopathy
  • Dilated cardiomyopathy due to lamin A/C mutation
  • Dilated cardiomyopathy due to laminopathy [LMNA]
  • Familial dilated cardiomyopathy with conduction defect due to LMNA mutation
  • Dilated cardiomyopathy due to Emery-Dreifus muscular dystrophy
  • Dilated cardiomyopathy due to limb-girdle muscular dystrophy
  • Dilated cardiomyopathy due to congenital muscular dystrophy
  • Dilated cardiomyopathy due to spinal muscular atrophy
  • Dilated cardiomyopathy due to SMA - [spinal muscular atrophy]
  • Dilated cardiomyopathy due to polymyositis
  • Sarcomeric dilated cardiomyopathy
  • X-linked infantile dilated cardiomyopathy
  • Dilated cardiomyopathy due to lysosomal storage disorder
  • dilated-hypokinetic cardiomyopathy due to lysosomal storage disorder
  • Dilated cardiomyopathy due to mucopolysaccharidoses
  • dilated-hypokinetic cardiomyopathy due to mucopolysaccharidoses
  • Dilated cardiomyopathy due to sphingolipidoses
  • dilated-hypokinetic cardiomyopathy due to sphingolipidoses
  • Dilated cardiomyopathy due to Gaucher disease

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

  • BC43.01 – Nonfamilial dilated cardiomyopathy
  • BC43.0Z – Dilated cardiomyopathy, unspecified

Foundation entity

Familial-genetic dilated cardiomyopathy – http://id.who.int/icd/entity/423719003

Part of: BC43.0: Dilated cardiomyopathy › BC43: Cardiomyopathy › 11: Diseases of the circulatory system