Long QT Syndrome 2

Alternative Names

  • LQT2
  • Long QT Syndrome 2, Acquired, Susceptibility to
  • Long QT Syndrome 1/2, Digenic
  • LQT1/2, Digenic
  • Long QT Syndrome 2/3, Digenic
  • LQT2/3, Digenic
  • Long QT Syndrome 2/5, Digenic
  • LQT2/5, Digenic
  • Long QT Syndrome 2/9, Digenic
  • LQT2/9, Digenic
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WHO-ICD-10 version:2010

Diseases of the circulatory system

Other forms of heart disease

OMIM Number

613688

Mode of Inheritance

Autosomal dominant

Gene Map Locus

7q36.1,12q12

Description

Long QT syndrome (LQTS) is a hereditary cardiac disease characterized by a prolongation of the QT interval at basal ECG and by a high risk of life-threatening arrhythmias. It can be caused by mutations in several cardiac ion channel genes. There are eight types of LQT syndrome based on the gene in which causative mutations occur. The most prevalent forms are LQT1, LQT2, and LQT3. Half of individuals with LQT2 remain asymptomatic until the age of 16. Symptoms are triggered by a variety of causes including emotions, auditory experience, and exercise. Furthermore, some cases may occur during rest and sleep.

The diagnosis of LQT2 is based on electrocardiogram (ECG) abnormalities; notched T waves are most commonly seen in this type. Patients with LQT2 have symptom relief from beta-blocker therapy. Symptoms can also be improved in patients who maintain their potassium levels and modify their lifestyles. Treatment with potassium may be recommended.

Epidemiology in the Arab World

View Map
Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
613688.1.1LebanonMaleYes Sudden cardiac death; Death in early ad...NM_000238.3:c.1837A>GHeterozygousAutosomal, DominantPoulsen et al. 2015
613688.1.2LebanonFemaleYes Prolonged QTc intervalNM_000238.3:c.1837A>GHeterozygousAutosomal, DominantPoulsen et al. 2015 Sibling of 613688.1....
613688.1.3LebanonMaleYes Syncope; Prolonged QTc intervalNM_000238.3:c.1837A>GHeterozygousAutosomal, DominantPoulsen et al. 2015 Father of 613688.1.1
613688.2.1Saudi ArabiaFemaleNoNo Bilateral tonic-clonic seizure; Prolonge...NM_000238.4:c.2362G>AHomozygousAutosomal, RecessiveBhuiyan et al. 2009
613688.3.1Saudi ArabiaFemaleNoNo Bilateral tonic-clonic seizure; Prolonge...NM_000238.4:c.1609C>T, NM_000238.4:c.2342dupHeterozygousAutosomal, RecessiveBhuiyan et al. 2009
613688.4.1Saudi ArabiaMaleYesYes Polymorphic ventricular tachycardia; Pro...NM_000238.4:c.3208C>THomozygousAutosomal, RecessiveBhuiyan et al. 2009 Proband. HIs mother ...
613688.4.2Saudi ArabiaFemaleYesYes Arrhythmia; StillbirthNM_000238.4:c.3208C>THomozygousAutosomal, RecessiveBhuiyan et al. 2009 Sister of 613688.4.1
613688.5.1Saudi ArabiaMaleNoNo Bradycardia; Cardiac arrest; Prolonged Q...NM_000238.4:c.3208C>THomozygousAutosomal, RecessiveBhuiyan et al. 2009
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