Long QT Syndrome 16

Alternative Names

  • LQT16

Associated Genes

Calmodulin 3
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WHO-ICD-10 version:2010

Diseases of the circulatory system

Other forms of heart disease

OMIM Number

618782

Mode of Inheritance

Autosomal dominant

Gene Map Locus

19q13.32

Description

LQT16 Long QT syndrome-16 (LQT16) is characterized by a markedly prolonged corrected QT (QTc) interval and 2:1 atrioventricular (AV) block, with onset in the perinatal period. Patients experience bradycardia or ventricular tachyarrhythmias that may result in syncope, cardiac arrest, and/or sudden death. Patients with LQT14, LQT15, or LQT16, resulting from mutation in calmodulin genes CALM1, CALM2, or CALM3, respectively, typically have a more severe phenotype, with earlier onset, profound QT prolongation, and a high predilection for cardiac arrest and sudden death, than patients with mutations in other genes.

Epidemiology in the Arab World

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Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
618782.1Saudi ArabiaMaleNoNo Bradycardia; Syncope; 2:1 atrioventricul...NM_005184.4:c.421G>AHeterozygousAutosomal, DominantWren et al. 2019 Patient from 'family...
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