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Arrhythmogenic right ventricular dysplasia 6(ARVC6; ARVD6)

MedGen UID:
Concept ID:
Disease or Syndrome
Synonyms: Arrhythmogenic right ventricular dysplasia, familial, 6; Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy 6
Monarch Initiative: MONDO:0011460
OMIM®: 604401

Elizabeth McNally  |  Heather MacLeod  |  Lisa Dellefave-Castillo   view full author information

Additional descriptions

From GeneReviews Overview
Arrhythmogenic right ventricular cardiomyopathy (ARVC) – previously referred to as arrhythmogenic right ventricular dysplasia (ARVD) – is characterized by progressive fibrofatty replacement of the myocardium that predisposes to ventricular tachycardia and sudden death in young individuals and athletes. It primarily affects the right ventricle, and it may also involve the left ventricle. The presentation of disease is highly variable even within families, and some affected individuals may not meet established clinical criteria. The mean age at diagnosis is 31 years (±13; range: 4-64 years).
From MedlinePlus Genetics
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a form of heart disease that usually appears in adulthood. ARVC is a disorder of the myocardium, which is the muscular wall of the heart. This condition causes part of the myocardium to break down over time, increasing the risk of an abnormal heartbeat (arrhythmia) and sudden death.

ARVC may not cause any symptoms in its early stages. However, affected individuals may still be at risk of sudden death, especially during strenuous exercise. When symptoms occur, they most commonly include a sensation of fluttering or pounding in the chest (palpitations), light-headedness, and fainting (syncope). Over time, ARVC can also cause shortness of breath and abnormal swelling in the legs or abdomen. If the myocardium becomes severely damaged in the later stages of the disease, it can lead to heart failure.  https://medlineplus.gov/genetics/condition/arrhythmogenic-right-ventricular-cardiomyopathy

Clinical features

From HPO
Sudden cardiac death
MedGen UID:
Concept ID:
Pathologic Function
The heart suddenly and unexpectedly stops beating resulting in death within a short time period (generally within 1 h of symptom onset).
Premature ventricular contraction
MedGen UID:
Concept ID:
Disease or Syndrome
Premature ventricular contractions (PVC) or ventricular extrasystoles are premature contractions of the heart that arise in response to an impulse in the ventricles rather than the normal impulse from the sinoatrial (SA) node.
Right ventricular cardiomyopathy
MedGen UID:
Concept ID:
Disease or Syndrome
Right ventricular dysfunction (global or regional) with functional and morphological right ventricular abnormalities, with or without left ventricular disease.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines


Alblaihed L, Kositz C, Brady WJ, Al-Salamah T, Mattu A
Am J Emerg Med 2023 Mar;65:146-153. Epub 2022 Dec 12 doi: 10.1016/j.ajem.2022.12.010. PMID: 36638611
Stava TT, Leren TP, Bogsrud MP
Eur J Prev Cardiol 2022 Oct 18;29(13):1789-1799. doi: 10.1093/eurjpc/zwac102. PMID: 35653365
Haykal M, Matsumori A, Saleh A, Fayez M, Negm H, Shalaby M, Bassuony S
Expert Rev Cardiovasc Ther 2021 Jun;19(6):493-499. Epub 2021 Jun 2 doi: 10.1080/14779072.2021.1917383. PMID: 33861939

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