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Long QT syndrome 4(LQT4)

MedGen UID:
331449
•Concept ID:
C1833154
•
Disease or Syndrome
Synonym: LQT4
 
Monarch Initiative: MONDO:0800323
OMIM®: 106410; 600919

Recent clinical studies

Etiology

Mothers with long QT syndrome are at increased risk for fetal death: findings from a multicenter international study.
Cuneo BF, Kaizer AM, Clur SA, Swan H, Herberg U, Winbo A, Rydberg A, Haugaa K, Etheridge S, Ackerman MJ, Dagradi F, Killen SAS, Wacker-Gussmann A, Benson DW, Wilde AAM, Pan Z, Lam A, Spazzolini C, Horigome H, Schwartz PJ; Fetal LQTS Consortium
Am J Obstet Gynecol 2020 Mar;222(3):263.e1-263.e11. Epub 2019 Sep 11 doi: 10.1016/j.ajog.2019.09.004. PMID: 31520628
All Along the Watchtower: a Case of Long QT Syndrome Misdiagnosis Secondary to Genetic Testing Misinterpretation.
Helm BM, Ayers MD, Kean AC
J Genet Couns 2018 Dec;27(6):1515-1522. Epub 2018 Aug 16 doi: 10.1007/s10897-018-0287-8. PMID: 30117070
Video-assisted thoracoscopic cardiac denervation: a potential novel therapeutic option for children with intractable ventricular arrhythmias.
Atallah J, Fynn-Thompson F, Cecchin F, DiBardino DJ, Walsh EP, Berul CI
Ann Thorac Surg 2008 Nov;86(5):1620-5. doi: 10.1016/j.athoracsur.2008.07.006. PMID: 19049760
Major quantitative trait locus for resting heart rate maps to a region on chromosome 4.
Martin LJ, Comuzzie AG, Sonnenberg GE, Myklebust J, James R, Marks J, Blangero J, Kissebah AH
Hypertension 2004 May;43(5):1146-51. Epub 2004 Mar 1 doi: 10.1161/01.HYP.0000122873.42047.17. PMID: 14993199

Diagnosis

All Along the Watchtower: a Case of Long QT Syndrome Misdiagnosis Secondary to Genetic Testing Misinterpretation.
Helm BM, Ayers MD, Kean AC
J Genet Couns 2018 Dec;27(6):1515-1522. Epub 2018 Aug 16 doi: 10.1007/s10897-018-0287-8. PMID: 30117070
Inherited long QT syndrome: phenotype and therapy to use and avoid.
Conti CR
Clin Cardiol 2006 Oct;29(10):429-30. doi: 10.1002/clc.4960291002. PMID: 17063944Free PMC Article
Sudden unexplained death: heritability and diagnostic yield of cardiological and genetic examination in surviving relatives.
Tan HL, Hofman N, van Langen IM, van der Wal AC, Wilde AA
Circulation 2005 Jul 12;112(2):207-13. Epub 2005 Jul 5 doi: 10.1161/CIRCULATIONAHA.104.522581. PMID: 15998675

Therapy

Mothers with long QT syndrome are at increased risk for fetal death: findings from a multicenter international study.
Cuneo BF, Kaizer AM, Clur SA, Swan H, Herberg U, Winbo A, Rydberg A, Haugaa K, Etheridge S, Ackerman MJ, Dagradi F, Killen SAS, Wacker-Gussmann A, Benson DW, Wilde AAM, Pan Z, Lam A, Spazzolini C, Horigome H, Schwartz PJ; Fetal LQTS Consortium
Am J Obstet Gynecol 2020 Mar;222(3):263.e1-263.e11. Epub 2019 Sep 11 doi: 10.1016/j.ajog.2019.09.004. PMID: 31520628
Inherited long QT syndrome: phenotype and therapy to use and avoid.
Conti CR
Clin Cardiol 2006 Oct;29(10):429-30. doi: 10.1002/clc.4960291002. PMID: 17063944Free PMC Article

Prognosis

All Along the Watchtower: a Case of Long QT Syndrome Misdiagnosis Secondary to Genetic Testing Misinterpretation.
Helm BM, Ayers MD, Kean AC
J Genet Couns 2018 Dec;27(6):1515-1522. Epub 2018 Aug 16 doi: 10.1007/s10897-018-0287-8. PMID: 30117070
Video-assisted thoracoscopic cardiac denervation: a potential novel therapeutic option for children with intractable ventricular arrhythmias.
Atallah J, Fynn-Thompson F, Cecchin F, DiBardino DJ, Walsh EP, Berul CI
Ann Thorac Surg 2008 Nov;86(5):1620-5. doi: 10.1016/j.athoracsur.2008.07.006. PMID: 19049760

Clinical prediction guides

Mothers with long QT syndrome are at increased risk for fetal death: findings from a multicenter international study.
Cuneo BF, Kaizer AM, Clur SA, Swan H, Herberg U, Winbo A, Rydberg A, Haugaa K, Etheridge S, Ackerman MJ, Dagradi F, Killen SAS, Wacker-Gussmann A, Benson DW, Wilde AAM, Pan Z, Lam A, Spazzolini C, Horigome H, Schwartz PJ; Fetal LQTS Consortium
Am J Obstet Gynecol 2020 Mar;222(3):263.e1-263.e11. Epub 2019 Sep 11 doi: 10.1016/j.ajog.2019.09.004. PMID: 31520628

Supplemental Content

Table of contents

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    Reviews

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