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Long QT syndrome(LQTS)

MedGen UID:
44193
Concept ID:
C0023976
Disease or Syndrome
Synonym: LQTS
SNOMED CT: Long Q-T syndrome (9651007); Long QT syndrome (9651007); Prolonged Q-T interval syndrome (9651007)
 
Genes (locations): KCNE1 (21q22.12); KCNE2 (21q22.11); KCNH2 (7q36.1); KCNQ1 (11p15.5-15.4); SCN5A (3p22.2); TRDN (6q22.31)
Related genes: ALG10B, AKAP9, SNTA1, SCN4B, KCNJ5, CAV3
 
Monarch Initiative: MONDO:0002442

Definition

Romano-Ward syndrome is a condition that causes a disruption of the heart's normal rhythm (arrhythmia). This disorder is a form of long QT syndrome, which is a heart condition that causes the heart (cardiac) muscle to take longer than usual to recharge between beats. The term "long QT" refers to a specific pattern of heart activity that is detected with an electrocardiogram (ECG or EKG), which is a test used to measure the electrical activity of the heart. In people with long QT syndrome, the part of the heartbeat known as the QT interval is abnormally long. Abnormalities in the time it takes to recharge the heart lead to abnormal heart rhythms.

The arrhythmia associated with Romano-Ward syndrome can lead to fainting (syncope) or cardiac arrest and sudden death. However, some people with Romano-Ward syndrome never experience any health problems associated with the condition.

Fifteen types of long QT syndrome have been defined based on their genetic cause. Some types of long QT syndrome involve other cardiac abnormalities or problems with additional body systems. Romano-Ward syndrome encompasses those types that involve only a long QT interval without other abnormalities. [from MedlinePlus Genetics]

Professional guidelines

PubMed

Strasburger JF, Eckstein G, Butler M, Noffke P, Wacker-Gussmann A
J Clin Pharmacol 2022 Sep;62 Suppl 1(Suppl 1):S53-S66. doi: 10.1002/jcph.2129. PMID: 36106782Free PMC Article
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
Schwartz PJ, Crotti L, Insolia R
Circ Arrhythm Electrophysiol 2012 Aug 1;5(4):868-77. doi: 10.1161/CIRCEP.111.962019. PMID: 22895603Free PMC Article

Suggested Reading

PubMed

Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, Blom N, Brugada J, Chiang CE, Huikuri H, Kannankeril P, Krahn A, Leenhardt A, Moss A, Schwartz PJ, Shimizu W, Tomaselli G, Tracy C; Document Reviewers, Ackerman M, Belhassen B, Estes NA 3rd, Fatkin D, Kalman J, Kaufman E, Kirchhof P, Schulze-Bahr E, Wolpert C, Vohra J, Refaat M, Etheridge SP, Campbell RM, Martin ET, Quek SC; Heart Rhythm Society; European Heart Rhythm Association; Asia Pacific Heart Rhythm Society
Europace 2013 Oct;15(10):1389-406. Epub 2013 Aug 30 doi: 10.1093/europace/eut272. PMID: 23994779

Recent clinical studies

Etiology

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
Galić E, Bešlić P, Kilić P, Planinić Z, Pašalić A, Galić I, Ćubela VV, Pekić P
Acta Clin Croat 2021 Dec;60(4):739-748. doi: 10.20471/acc.2021.60.04.22. PMID: 35734489Free PMC Article
Wallace E, Howard L, Liu M, O'Brien T, Ward D, Shen S, Prendiville T
Pediatr Cardiol 2019 Oct;40(7):1419-1430. Epub 2019 Aug 22 doi: 10.1007/s00246-019-02151-x. PMID: 31440766Free PMC Article
Giudicessi JR, Noseworthy PA, Ackerman MJ
Circulation 2019 Jun 11;139(24):2711-2713. Epub 2019 Jun 10 doi: 10.1161/CIRCULATIONAHA.119.039598. PMID: 31180747
Shah SR, Park K, Alweis R
Curr Probl Cardiol 2019 Mar;44(3):92-106. Epub 2018 May 10 doi: 10.1016/j.cpcardiol.2018.04.002. PMID: 29784533

Diagnosis

Schwartz PJ
Heart Rhythm 2024 Jul;21(7):991-992. doi: 10.1016/j.hrthm.2024.04.037. PMID: 38960553
Galić E, Bešlić P, Kilić P, Planinić Z, Pašalić A, Galić I, Ćubela VV, Pekić P
Acta Clin Croat 2021 Dec;60(4):739-748. doi: 10.20471/acc.2021.60.04.22. PMID: 35734489Free PMC Article
Shah SR, Park K, Alweis R
Curr Probl Cardiol 2019 Mar;44(3):92-106. Epub 2018 May 10 doi: 10.1016/j.cpcardiol.2018.04.002. PMID: 29784533
Abrams DJ, Macrae CA
Circulation 2014 Apr 8;129(14):1524-9. doi: 10.1161/CIRCULATIONAHA.113.003985. PMID: 24709866
Schwartz PJ, Crotti L, Insolia R
Circ Arrhythm Electrophysiol 2012 Aug 1;5(4):868-77. doi: 10.1161/CIRCEP.111.962019. PMID: 22895603Free PMC Article

Therapy

Woosley RL, Heise CW
Heart Rhythm 2024 Jun;21(6):969-970. doi: 10.1016/j.hrthm.2024.02.007. PMID: 38816146
Shimizu T, Kawai S, Kawada A, Wakamiya T, Nakano Y, Watanabe S, Iwamoto M
Pediatr Int 2023 Jan;65(1):e15517. doi: 10.1111/ped.15517. PMID: 36847291
Jackson H, Huisman LA, Sanatani S, Arbour LT
CMAJ 2011 Aug 9;183(11):1272-5. Epub 2011 Apr 11 doi: 10.1503/cmaj.100138. PMID: 21482651Free PMC Article
Vincent GM, Timothy K, Zhang L
Card Electrophysiol Rev 2002 Feb;6(1-2):57-60. doi: 10.1023/a:1017935121656. PMID: 11984019
Schwartz PJ, Bonazzi O, Locati E, Napolitano C, Sala S
Ann N Y Acad Sci 1992 Jan 27;644:112-41. doi: 10.1111/j.1749-6632.1992.tb31007.x. PMID: 1348609

Prognosis

Kamp TJ, Hamdan MH, January CT
J Am Heart Assoc 2020 Jun 16;9(12):e016887. Epub 2020 May 28 doi: 10.1161/JAHA.120.016887. PMID: 32463308Free PMC Article
Ellison SR
Prim Care 2015 Mar;42(1):57-76. doi: 10.1016/j.pop.2014.09.012. PMID: 25634705
Ackerman MJ
Pediatr Rev 1998 Jul;19(7):232-8. doi: 10.1542/pir.19-7-232. PMID: 9654946
Perry JC
Pacing Clin Electrophysiol 1997 Aug;20(8 Pt 2):2061-4. doi: 10.1111/j.1540-8159.1997.tb03628.x. PMID: 9272509
Schwartz PJ
Curr Probl Cardiol 1997 Jun;22(6):297-351. doi: 10.1016/s0146-2806(97)80009-6. PMID: 9189673

Clinical prediction guides

Mazzanti A, Trancuccio A, Kukavica D, Pagan E, Wang M, Mohsin M, Peterson D, Bagnardi V, Zareba W, Priori SG
Europace 2022 Apr 5;24(4):614-619. doi: 10.1093/europace/euab238. PMID: 34505884
Galić E, Bešlić P, Kilić P, Planinić Z, Pašalić A, Galić I, Ćubela VV, Pekić P
Acta Clin Croat 2021 Dec;60(4):739-748. doi: 10.20471/acc.2021.60.04.22. PMID: 35734489Free PMC Article
Giudicessi JR, Noseworthy PA, Ackerman MJ
Circulation 2019 Jun 11;139(24):2711-2713. Epub 2019 Jun 10 doi: 10.1161/CIRCULATIONAHA.119.039598. PMID: 31180747
Sandau KE, Funk M, Auerbach A, Barsness GW, Blum K, Cvach M, Lampert R, May JL, McDaniel GM, Perez MV, Sendelbach S, Sommargren CE, Wang PJ; American Heart Association Council on Cardiovascular and Stroke Nursing; Council on Clinical Cardiology; and Council on Cardiovascular Disease in the Young
Circulation 2017 Nov 7;136(19):e273-e344. Epub 2017 Oct 3 doi: 10.1161/CIR.0000000000000527. PMID: 28974521
Itoh H, Crotti L, Aiba T, Spazzolini C, Denjoy I, Fressart V, Hayashi K, Nakajima T, Ohno S, Makiyama T, Wu J, Hasegawa K, Mastantuono E, Dagradi F, Pedrazzini M, Yamagishi M, Berthet M, Murakami Y, Shimizu W, Guicheney P, Schwartz PJ, Horie M
Eur Heart J 2016 May 7;37(18):1456-64. Epub 2015 Dec 28 doi: 10.1093/eurheartj/ehv695. PMID: 26715165Free PMC Article

Recent systematic reviews

Galić E, Bešlić P, Kilić P, Planinić Z, Pašalić A, Galić I, Ćubela VV, Pekić P
Acta Clin Croat 2021 Dec;60(4):739-748. doi: 10.20471/acc.2021.60.04.22. PMID: 35734489Free PMC Article
Beach SR, Gross AF, Hartney KE, Taylor JB, Rundell JR
Gen Hosp Psychiatry 2020 Nov-Dec;67:42-50. Epub 2020 Aug 22 doi: 10.1016/j.genhosppsych.2020.08.008. PMID: 32979582
Adler A, Novelli V, Amin AS, Abiusi E, Care M, Nannenberg EA, Feilotter H, Amenta S, Mazza D, Bikker H, Sturm AC, Garcia J, Ackerman MJ, Hershberger RE, Perez MV, Zareba W, Ware JS, Wilde AAM, Gollob MH
Circulation 2020 Feb 11;141(6):418-428. Epub 2020 Jan 27 doi: 10.1161/CIRCULATIONAHA.119.043132. PMID: 31983240Free PMC Article
Vandael E, Vandenberk B, Vandenberghe J, Willems R, Foulon V
Int J Clin Pharm 2017 Feb;39(1):16-25. Epub 2016 Dec 23 doi: 10.1007/s11096-016-0414-2. PMID: 28012118
Tampi RR, Balderas M, Carter KV, Tampi DJ, Moca M, Knudsen A, May J
Psychosomatics 2015 Jan-Feb;56(1):36-43. Epub 2014 Sep 6 doi: 10.1016/j.psym.2014.09.002. PMID: 25619672

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