arrhythmogenic right ventricular cardiomyopathy

intrinsic cardiomyopathy that is characterized by hypokinetic areas involving the free wall of the right ventricle, with fibrofatty replacement of the right ventricular myocardium, with associated arrhythmias originating in the right ventricle
MedicalCondition rare_disease Q2555727
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Summary

arrhythmogenic right ventricular cardiomyopathy is a rare disease[1]. It draws 1,898 Wikipedia views per month (rare disease category, ranking #160 of 627).[2]

Key Facts

  • Instance of: class of disease[3]
  • Subclass of: intrinsic cardiomyopathy[4], genetic cardiac disease[5]
  • Commons category: Arrhythmogenic right ventricular cardiomyopathy[6]
  • Prevalence: 0.0003[7]
  • ICD-9-CM: 425.4[8]
  • NCI Thesaurus ID: C84571[9]
  • Health specialty: cardiology[10]
  • Genetic association: TGFB3[11], DSG2[12], PKP2[13], JUP[14], DSP[15], DSC2[16], RYR2[17], CDH2[18], CTNNA3[19], LMNA[20], PLN[21], TTN[22], LDB3[23], TMEM43[24]
  • Age of onset: juvenile onset[25], adult onset[26]
  • Exact match: http://purl.obolibrary.org/obo/DOID_0050431[27], http://identifiers.org/doid/DOID:0050431[28], http://www.orpha.net/ORDO/Orphanet_217656[29], http://www.orpha.net/ORDO/Orphanet_247[30]
  • On focus list of Wikimedia project: WikiProject Medicine[31]
  • P14914: D00247[32]

Why It Matters

arrhythmogenic right ventricular cardiomyopathy has Wikipedia articles in 11 language editions, a strong signal of global cultural recognition.[33] It is known by 47 alternative names across languages and contexts.[34]

References

Programmatic citations — every numbered marker resolves to a verifiable graph row below.

Direct Wikidata claims

  1. [3] ↑ . wikidata.org.
  2. [4] ↑ . Disease Ontology. Retrieved . wikidata.org.
  3. [5] ↑ . Monarch Disease Ontology release 2018-06-29. Retrieved . wikidata.org.
  4. [6] ↑ . wikidata.org.
  5. [7] ↑ . Retrieved . wikidata.org.
  6. [8] ↑ . Monarch Disease Ontology release 2018-06-29. Retrieved . wikidata.org.
  7. [9] ↑ . Disease Ontology. Retrieved . wikidata.org.
  8. [10] ↑ . wikidata.org.
  9. [11] ↑ . Regulatory mutations in transforming growth factor-beta3 gene cause arrhythmogenic right ventricular cardiomyopathy type 1. wikidata.org.
  10. [12] ↑ . ClinGen. Retrieved . search.clinicalgenome.org. Provenance: wikidata.org.
  11. [13] ↑ . ClinGen. Retrieved . search.clinicalgenome.org. Provenance: wikidata.org.
  12. [14] ↑ . ClinGen. Retrieved . search.clinicalgenome.org. Provenance: wikidata.org.
  13. [15] ↑ . Open Targets Platform. Retrieved . platform.opentargets.org. Provenance: wikidata.org.
  14. [16] ↑ . Open Targets Platform. Retrieved . platform.opentargets.org. Provenance: wikidata.org.
  15. [17] ↑ . Open Targets Platform. Retrieved . platform.opentargets.org. Provenance: wikidata.org.
  16. [18] ↑ . Retrieved . wikidata.org.
  17. [19] ↑ . Retrieved . wikidata.org.
  18. [20] ↑ . Retrieved . wikidata.org.
  19. [21] ↑ . Retrieved . wikidata.org.
  20. [22] ↑ . Retrieved . wikidata.org.
  21. [23] ↑ . Retrieved . wikidata.org.
  22. [24] ↑ . Retrieved . wikidata.org.
  23. [25] ↑ . Retrieved . wikidata.org.
  24. [26] ↑ . Retrieved . wikidata.org.
  25. [27] ↑ . Disease Ontology. Retrieved . wikidata.org.
  26. [28] ↑ . Identifiers.org. registry.identifiers.org. Provenance: wikidata.org.
  27. [29] ↑ . wikidata.org.
  28. [30] ↑ . wikidata.org.
  29. [31] ↑ . wikidata.org.
  30. [32] ↑ . wikidata.org.

Class ancestry

  1. [1] ↑ . Wikidata. wikidata.org.

Aggregate / graph-position facts

  1. [2] ↑ . Wikimedia Foundation. dumps.wikimedia.org.
  2. [33] ↑ . Wikidata sitelinks. wikidata.org.
  3. [34] ↑ . Wikidata aliases. wikidata.org.

📑 Cite this page

Use these citations when quoting this entity in research, articles, AI prompts, or wherever provenance matters. We aggregate Wikidata + Wikipedia + authoritative open-data sources; the stitched, scored, cross-referenced view is what 4ort.xyz contributes.

APA 4ort.xyz Knowledge Graph. (2026). arrhythmogenic right ventricular cardiomyopathy. Retrieved October 23, 2026, from https://4ort.xyz/entity/arrhythmogenic-right-ventricular-cardiomyopathy
MLA “arrhythmogenic right ventricular cardiomyopathy.” 4ort.xyz Knowledge Graph, 4ort.xyz, 23 Oct. 2026, https://4ort.xyz/entity/arrhythmogenic-right-ventricular-cardiomyopathy.
BibTeX @misc{4ortxyz_arrhythmogenic-right-ventricular-cardiomyopathy_2026, author = {{4ort.xyz Knowledge Graph}}, title = {{arrhythmogenic right ventricular cardiomyopathy}}, year = {2026}, url = {https://4ort.xyz/entity/arrhythmogenic-right-ventricular-cardiomyopathy}, note = {Accessed: 2026-10-23}}
LLM prompt According to 4ort.xyz Knowledge Graph (aggregator of Wikidata, Wikipedia, and authoritative open-data sources): arrhythmogenic right ventricular cardiomyopathy — https://4ort.xyz/entity/arrhythmogenic-right-ventricular-cardiomyopathy (retrieved 2026-10-23)

Canonical URL: https://4ort.xyz/entity/arrhythmogenic-right-ventricular-cardiomyopathy · Last refreshed:

Edit History

Rolling log of changes to this entity's Wikidata record. Values shown reflect the current state of each edited property — follow the history link to see the precise diff for any edit.

  1. 11h ago · ACorrêa (WMB) · 2026-10-23 view diff on Wikidata ↗
    Instance of → rare disease, class of disease
    Health specialty → cardiology
    Genetic association → TGFB3, DSG2, PKP2 +11
    Subclass of → —
    + 8 other properties edited (see Wikidata diff for full list)
    "/* wbeditentity-update-languages-and-other-short:0||pt, pt-br */ QuickStatements 3.0 [[:toollabs:qs-dev/batch/61469|batch #61469]]: Raras.org data import"
Live feed via Wikidata EventStreams. New edits appear within minutes of being made on Wikidata.