U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

Idiopathic ventricular tachycardia is a generic term that describes the various forms of ventricular arrhythmias that occur in patients without structural heart disease and in the absence of long QT syndrome (summary by Lerman et al., 1998). Familial ventricular tachycardia is usually attributable to recognized conditions such as arrhythmogenic right ventricular dysplasia, hypertrophic cardiomyopathy, familial cardiomyopathy, or one of the long QT syndromes. There are families with ventricular tachycardia in which no recognized underlying condition has been identified. The features of the disorder are variable from family to family (summary by Fisher et al., 1999). See also catecholaminergic polymorphic ventricular tachycardia (CPVT; 604772). [from OMIM]

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

Lenarczyk R, Zeppenfeld K, Tfelt-Hansen J, Heinzel FR, Deneke T, Ene E, Meyer C, Wilde A, Arbelo E, Jędrzejczyk-Patej E, Sabbag A, Stühlinger M, di Biase L, Vaseghi M, Ziv O, Bautista-Vargas WF, Kumar S, Namboodiri N, Henz BD, Montero-Cabezas J, Dagres N, Peichl P, Frontera A, Tzeis S, Merino JL, Soejima K, de Chillou C, Tung R, Eckardt L, Maury P, Hlivak P, Tereshchenko LG, Kojodjojo P, Atié J
Europace 2024 Mar 30;26(4) doi: 10.1093/europace/euae049. PMID: 38584423Free PMC Article
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

Recent clinical studies

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...