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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).
See: Feature record | Search on this feature
Paroxysmal ventricular tachycardia
MedGen UID:
18315
•Concept ID:
C0030591
•
Disease or Syndrome
Episodes of ventricular tachycardia that have a sudden onset and ending.
See: Feature record | Search on this feature
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.
See: Feature record | Search on this feature
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.
See: Feature record | Search on this feature
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Professional guidelines

PubMed

Diagnosis, management and therapeutic strategies for congenital long QT syndrome.
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
2020 APHRS/HRS expert consensus statement on the investigation of decedents with sudden unexplained death and patients with sudden cardiac arrest, and of their families.
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
Implications of Genetic Testing in Dilated Cardiomyopathy.
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

Etiology

Images in cardiology: familial ventricular tachycardia with mild ventricular dysfunction: a 15 year follow up of two African American brothers with arrhythmogenic right ventricular dysplasia.
Perzanowski C, Crespo G, Yazdanfar S
Heart 2000 Dec;84(6):658. doi: 10.1136/heart.84.6.658. PMID: 11083749Free PMC Article

Diagnosis

Images in cardiology: familial ventricular tachycardia with mild ventricular dysfunction: a 15 year follow up of two African American brothers with arrhythmogenic right ventricular dysplasia.
Perzanowski C, Crespo G, Yazdanfar S
Heart 2000 Dec;84(6):658. doi: 10.1136/heart.84.6.658. PMID: 11083749Free PMC Article
A case of familial ventricular tachycardia.
Betsuyaku T, Sakurai M, Yoshida I, Yokoshiki H, Ito T, Yotsukura A, Kitabatake A
Jpn Circ J 1995 Mar;59(3):171-5. doi: 10.1253/jcj.59.171. PMID: 7602753

Prognosis

Images in cardiology: familial ventricular tachycardia with mild ventricular dysfunction: a 15 year follow up of two African American brothers with arrhythmogenic right ventricular dysplasia.
Perzanowski C, Crespo G, Yazdanfar S
Heart 2000 Dec;84(6):658. doi: 10.1136/heart.84.6.658. PMID: 11083749Free PMC Article

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    • Gene
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    • GTR(Clinical)
      Clinical tests in GTR
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    • OMIM
      Related records in OMIM
    • OMIM(Genes)
      OMIM records containing genes associated with phenotypes registered in MedGen
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