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Paroxysmal familial ventricular fibrillation

MedGen UID:
Concept ID:
Disease or Syndrome
Synonyms: Idiopathic Ventricular Fibrillation; Idiopathic ventricular fibrillation, non Brugada type; Paroxysmal ventricular fibrillation
SNOMED CT: Paroxysmal familial ventricular fibrillation (233915000)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
Concept ID:
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
Concept ID:
Source: Orphanet
clinical entity without genetic inheritance.
Related genes: SCN5A, DPP6
Monarch Initiative: MONDO:0100234
Orphanet: ORPHA228140


A rare, genetic, cardiac rhythm disease characterized by ventricular fibrillation in the absence of any structural or functional heart disease, or known repolarization abnormalities. The presence of J waves is associated with a higher risk of nocturnal ventricular fibrillation events and a higher risk of recurrence. [from ORDO]

Professional guidelines


van der Crabben SN, Wilde AAM
Herzschrittmacherther Elektrophysiol 2024 Mar;35(1):19-24. Epub 2024 Feb 9 doi: 10.1007/s00399-024-00994-3. PMID: 38334831Free PMC Article
Mascia G, Bona RD, Ameri P, Canepa M, Porto I, Parati G, Crotti L, Brignole M
Europace 2021 Jul 18;23(7):996-1002. doi: 10.1093/europace/euaa370. PMID: 33367713
Mellor G, Behr ER
Heart Rhythm 2017 Jul;14(7):1041-1042. Epub 2017 Feb 10 doi: 10.1016/j.hrthm.2017.02.004. PMID: 28192205

Recent clinical studies


McRae JR, Wagner GS, Rogers MC, Canent RV
J Pediatr 1974 Apr;84(4):515-8. doi: 10.1016/s0022-3476(74)80670-0. PMID: 4834245

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