Long QT Syndrome 1

Alternative Names

  • LQT1
  • Ward-Romano Syndrome
  • WRS
  • Romano-Ward Syndrome
  • RWS
  • Ventricular Fibrillation with Prolonged QT Interval
  • Long QT Syndrome 1/2
  • LQT1/2
  • Long QT Syndrome 1, Acquired, Susceptibility to
Back to search Result
WHO-ICD-10 version:2010

Diseases of the circulatory system

Other forms of heart disease

OMIM Number

192500

Mode of Inheritance

Autosomal dominant

Gene Map Locus

11p15.5-p15.4

Description

Long QT Syndrome is a cardiac disorder, characterized by a delay of the cardiac muscles in recharging the electrical stimulus between beats. This leads to a prolonged interval between two consecutive beats, referred to as the QT interval. While a prolonged QT interval is not harmful per se, it can result in arrhythmia, which may lead to cardiac arrests, and sudden death. LQTS1 is caused by heterozygous mutations in the KCNQ1 gene, which encodes a voltage-gated potassium channel. 

Epidemiology in the Arab World

View Map
Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
192500.1.1Saudi ArabiaMaleYesYes Syncope; Prolonged QTc intervalNM_000218.2:c.387-5T>AHeterozygousAutosomal, DominantBhuiyan et al. 2009
192500.1.2Saudi ArabiaMaleYesNM_000218.2:c.387-5T>AHeterozygousAutosomal, DominantBhuiyan et al. 2009 Father of 192500.1.1
192500.1.3Saudi ArabiaFemaleYesNM_000218.2:c.387-5T>AHeterozygousAutosomal, DominantBhuiyan et al. 2009 Mother of 192500.1.1
192500.2.1Saudi ArabiaMaleNoYes Generalized-onset seizure; Cyanotic epi...NM_000218.2:c.387-5T>AHeterozygousAutosomal, DominantBhuiyan et al. 2009
192500.3.01Saudi ArabiaFemaleYesNo Syncope; Prolonged QTc intervalNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013
192500.3.02Saudi ArabiaFemaleYesNoNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013 Mother of 192500.3.0...
192500.3.03Saudi ArabiaFemaleYesNoNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013 Daughter of 192500.3...
192500.3.04Saudi ArabiaMaleYesNoNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013 Brother of 192500.3....
192500.3.05Saudi ArabiaMaleYesNo Syncope; Prolonged QTc intervalNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013 Brother of 192500.3....
192500.3.06Saudi ArabiaMaleYesNoNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013 Brother of 192500.3....
192500.3.07Saudi ArabiaMaleYesNoNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013 Nephew of 192500.3.0...
192500.3.08Saudi ArabiaMaleYesNoNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013 Nephew of 192500.3.0...
192500.3.09Saudi ArabiaMaleYesNoNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013 Nephew of 192500.3.0...
192500.3.10Saudi ArabiaMaleYesNoNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013 Nephew of 192500.3.0...
192500.3.11Saudi ArabiaMaleYesNoNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013 Nephew of 192500.3.0...
192500.3.12Saudi ArabiaMaleYesNoNM_000218.2:c.773A>CHeterozygousAutosomal, DominantShinwari et al. 2013 Nephew of 192500.3.0...
192500.4LebanonFemaleNo Torsade de pointes; Syncope; Anemia; Dee...NM_000218.3:c.1591C>T, NM_000238.4:c.967G>AHeterozygousRefaat et al. 2016 Patient has long-sta...
192500.G.1TunisiaUnknown Prolonged QTc intervalNM_000335.4:c.5366A>GHeterozygousAutosomal, DominantBenhorin et al. 1998 Group of 23 family m...

Other Reports

Kuwait

Vurgese et al. (2006) described a 35-year old Arab patient with a rare diagnosis of Guillain Barre syndrome in comorbidity with Romano Ward syndrome.

[Vurgese T, Mapkar OA, Surrun SK. Guillain-Barre syndrome in a patient with Romano-Ward syndrome: a case report. Kuwait Med J. 2006; 38(1):53-5.]

Lebanon

Schulze-Bahr et al. (1997) performed haplotype analysis in a Lebanese family with Jervell Lange-Nielsen syndrome, but failed to detect linkage at LQTS 1. Using this approach, they also excluded two other ion-channel genes involved in autosomal-dominant LQTS, HERG (LQTS2) and SCN5A (LQTS3).

Oman

Subramanyan and Venugopalan (2002) reported a consanguineous family with a malignant form of long QT syndrome that resulted in the deaths of four children.  

Saudi Arabia

Gorgels et al. (1998) described a 2-year old boy who was diagnosed with a rare condition of LQTS with impaired AV conduction. 

 

Tunisia

Benhorin et al. (1993) referred to the unpublished work of Kerem and colleagues who indicated the existence of more than one genetic form of LQT and excluded linkage with HRAS1 in a very large affected Jewish family originating from the island of Jerba near Tunis. 

© CAGS 2024. All rights reserved.