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Familial ventricular tachycardia

MedGen UID:
83309
Concept ID:
C0340485
Disease or Syndrome
Synonym: VENTRICULAR TACHYCARDIA, FAMILIAL POLYMORPHIC
SNOMED CT: Familial ventricular tachycardia (233906007)
 
Gene (location): GNAI2 (3p21.31)
 
Monarch Initiative: MONDO:0008648
OMIM®: 192605

Definition

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:
38841
Concept ID:
C0085298
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:
18315
Concept ID:
C0030591
Disease or Syndrome
Episodes of ventricular tachycardia that have a sudden onset and ending.
Right bundle branch block
MedGen UID:
88445
Concept ID:
C0085615
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.
Cardiomyopathy
MedGen UID:
209232
Concept ID:
C0878544
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

PubMed

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
Stiles MK, Wilde AAM, Abrams DJ, Ackerman MJ, Albert CM, Behr ER, Chugh SS, Cornel MC, Gardner K, Ingles J, James CA, Jimmy Juang JM, Kääb S, Kaufman ES, Krahn AD, Lubitz SA, MacLeod H, Morillo CA, Nademanee K, Probst V, Saarel EV, Sacilotto L, Semsarian C, Sheppard MN, Shimizu W, Skinner JR, Tfelt-Hansen J, Wang DW
Heart Rhythm 2021 Jan;18(1):e1-e50. Epub 2020 Oct 19 doi: 10.1016/j.hrthm.2020.10.010. PMID: 33091602Free 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

Recent clinical studies

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