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Familial ventricular tachycardia

MedGen UID:
Concept ID:
Disease or Syndrome
SNOMED CT: Familial ventricular tachycardia (233906007)
Gene (location): GNAI2 (3p21.31)
Monarch Initiative: MONDO:0008648
OMIM®: 192605


An instance of ventricular tachycardia that is caused by an inherited modification of the individual's genome. [from MONDO]

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).
Paroxysmal ventricular tachycardia
MedGen UID:
Concept ID:
Disease or Syndrome
Episodes of ventricular tachycardia that have a sudden onset and ending.
Right bundle branch block
MedGen UID:
Concept ID:
Disease or Syndrome
A conduction block of the right branch of the bundle of His. This manifests as a prolongation of the QRS complex (greater than 0.12 s) with delayed activation of the right ventricle and terminal delay on the EKG.
MedGen UID:
Concept ID:
Disease or Syndrome
A myocardial disorder in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality.

Professional guidelines


Wilde AAM, Amin AS, Postema PG
Heart 2022 Mar;108(5):332-338. Epub 2021 May 26 doi: 10.1136/heartjnl-2020-318259. PMID: 34039680Free PMC Article
Verdonschot JAJ, Hazebroek MR, Krapels IPC, Henkens MTHM, Raafs A, Wang P, Merken JJ, Claes GRF, Vanhoutte EK, van den Wijngaard A, Heymans SRB, Brunner HG
Circ Genom Precis Med 2020 Oct;13(5):476-487. Epub 2020 Sep 3 doi: 10.1161/CIRCGEN.120.003031. PMID: 32880476
Kodera S, Kiyosue A, Ando J, Akazawa H, Morita H, Watanabe M, Komuro I
Int Heart J 2017 Dec 12;58(6):847-852. Epub 2017 Nov 17 doi: 10.1536/ihj.17-365. PMID: 29151496

Recent clinical studies

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