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Long QT syndrome type 3

MedGen UID:
444047
•Concept ID:
C2931401
•
Disease or Syndrome
SNOMED CT: Long QT syndrome type 3 (1208866002)

Professional guidelines

PubMed

Type 3 long QT syndrome: Is the effectiveness of treatment with beta-blockers population-specific?
Hermida A, Gourraud JB, Denjoy I, Fressart V, Kyndt F, Maltret A, Khraiche D, Klug D, Mabo P, Sacher F, Maury P, Winum P, Defaye P, Clerici G, Babuty D, Elbez Y, Morgat C, Surget E, Messali A, De Jode P, Clédel A, Minois D, Maison-Blanche P, Bloch A, Leenhardt A, Probst V, Extramiana F
Heart Rhythm 2024 Mar;21(3):313-320. Epub 2023 Nov 11 doi: 10.1016/j.hrthm.2023.11.007. PMID: 37956775
Role of chronic continuous intravenous lidocaine in the clinical management of patients with malignant type 3 long QT syndrome.
Bains S, Lador A, Neves R, Bos JM, Giudicessi JR, Cannon BC, Ackerman MJ
Heart Rhythm 2022 Jan;19(1):81-87. Epub 2021 Sep 16 doi: 10.1016/j.hrthm.2021.09.016. PMID: 34537410
SCN5A mutations in 442 neonates and children: genotype-phenotype correlation and identification of higher-risk subgroups.
Baruteau AE, Kyndt F, Behr ER, Vink AS, Lachaud M, Joong A, Schott JJ, Horie M, Denjoy I, Crotti L, Shimizu W, Bos JM, Stephenson EA, Wong L, Abrams DJ, Davis AM, Winbo A, Dubin AM, Sanatani S, Liberman L, Kaski JP, Rudic B, Kwok SY, Rieubland C, Tfelt-Hansen J, Van Hare GF, Guyomarc'h-Delasalle B, Blom NA, Wijeyeratne YD, Gourraud JB, Le Marec H, Ozawa J, Fressart V, Lupoglazoff JM, Dagradi F, Spazzolini C, Aiba T, Tester DJ, Zahavich LA, Beauséjour-Ladouceur V, Jadhav M, Skinner JR, Franciosi S, Krahn AD, Abdelsayed M, Ruben PC, Yung TC, Ackerman MJ, Wilde AA, Schwartz PJ, Probst V
Eur Heart J 2018 Aug 14;39(31):2879-2887. doi: 10.1093/eurheartj/ehy412. PMID: 30059973

Recent clinical studies

Etiology

Independent validation and clinical implications of the risk prediction model for long QT syndrome (1-2-3-LQTS-Risk).
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
The role of mexiletine in the management of long QT syndrome.
Li G, Zhang L
J Electrocardiol 2018 Nov-Dec;51(6):1061-1065. Epub 2018 Aug 31 doi: 10.1016/j.jelectrocard.2018.08.035. PMID: 30497731
Long-term flecainide therapy in type 3 long QT syndrome.
Chorin E, Taub R, Medina A, Flint N, Viskin S, Benhorin J
Europace 2018 Feb 1;20(2):370-376. doi: 10.1093/europace/euw439. PMID: 28339995
Clinical Aspects of Type 3 Long-QT Syndrome: An International Multicenter Study.
Wilde AA, Moss AJ, Kaufman ES, Shimizu W, Peterson DR, Benhorin J, Lopes C, Towbin JA, Spazzolini C, Crotti L, Zareba W, Goldenberg I, Kanters JK, Robinson JL, Qi M, Hofman N, Tester DJ, Bezzina CR, Alders M, Aiba T, Kamakura S, Miyamoto Y, Andrews ML, McNitt S, Polonsky B, Schwartz PJ, Ackerman MJ
Circulation 2016 Sep 20;134(12):872-82. Epub 2016 Aug 26 doi: 10.1161/CIRCULATIONAHA.116.021823. PMID: 27566755Free PMC Article
Sodium channel mutations and arrhythmias.
Ruan Y, Liu N, Priori SG
Nat Rev Cardiol 2009 May;6(5):337-48. doi: 10.1038/nrcardio.2009.44. PMID: 19377496

Diagnosis

Combined mexiletine and flecainide for severe long QT syndrome type 3.
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
Independent validation and clinical implications of the risk prediction model for long QT syndrome (1-2-3-LQTS-Risk).
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
Dynamic QT response to cold-water face immersion in long-QT syndrome type 3.
Takahashi K, Shimizu W, Makita N, Nakayashiro M
Pediatr Int 2020 Aug;62(8):899-906. Epub 2020 Aug 6 doi: 10.1111/ped.14319. PMID: 32449227Free PMC Article
Long-term flecainide therapy in type 3 long QT syndrome.
Chorin E, Taub R, Medina A, Flint N, Viskin S, Benhorin J
Europace 2018 Feb 1;20(2):370-376. doi: 10.1093/europace/euw439. PMID: 28339995
Clinical Aspects of Type 3 Long-QT Syndrome: An International Multicenter Study.
Wilde AA, Moss AJ, Kaufman ES, Shimizu W, Peterson DR, Benhorin J, Lopes C, Towbin JA, Spazzolini C, Crotti L, Zareba W, Goldenberg I, Kanters JK, Robinson JL, Qi M, Hofman N, Tester DJ, Bezzina CR, Alders M, Aiba T, Kamakura S, Miyamoto Y, Andrews ML, McNitt S, Polonsky B, Schwartz PJ, Ackerman MJ
Circulation 2016 Sep 20;134(12):872-82. Epub 2016 Aug 26 doi: 10.1161/CIRCULATIONAHA.116.021823. PMID: 27566755Free PMC Article

Therapy

Therapeutic Efficacy of Mexiletine for Long QT Syndrome Type 2: Evidence From Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes, Transgenic Rabbits, and Patients.
Crotti L, Neves R, Dagradi F, Musu G, Giannetti F, Bos JM, Barbieri M, Cerea P, Giovenzana FLF, Torchio M, Mura M, Gnecchi M, Conte G, Auricchio A, Sala L, Odening KE, Ackerman MJ, Schwartz PJ
Circulation 2024 Aug 13;150(7):531-543. Epub 2024 Jun 28 doi: 10.1161/CIRCULATIONAHA.124.068959. PMID: 38939955
Combined mexiletine and flecainide for severe long QT syndrome type 3.
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
The role of mexiletine in the management of long QT syndrome.
Li G, Zhang L
J Electrocardiol 2018 Nov-Dec;51(6):1061-1065. Epub 2018 Aug 31 doi: 10.1016/j.jelectrocard.2018.08.035. PMID: 30497731
Long-term flecainide therapy in type 3 long QT syndrome.
Chorin E, Taub R, Medina A, Flint N, Viskin S, Benhorin J
Europace 2018 Feb 1;20(2):370-376. doi: 10.1093/europace/euw439. PMID: 28339995
Clinical Aspects of Type 3 Long-QT Syndrome: An International Multicenter Study.
Wilde AA, Moss AJ, Kaufman ES, Shimizu W, Peterson DR, Benhorin J, Lopes C, Towbin JA, Spazzolini C, Crotti L, Zareba W, Goldenberg I, Kanters JK, Robinson JL, Qi M, Hofman N, Tester DJ, Bezzina CR, Alders M, Aiba T, Kamakura S, Miyamoto Y, Andrews ML, McNitt S, Polonsky B, Schwartz PJ, Ackerman MJ
Circulation 2016 Sep 20;134(12):872-82. Epub 2016 Aug 26 doi: 10.1161/CIRCULATIONAHA.116.021823. PMID: 27566755Free PMC Article

Prognosis

Therapeutic Efficacy of Mexiletine for Long QT Syndrome Type 2: Evidence From Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes, Transgenic Rabbits, and Patients.
Crotti L, Neves R, Dagradi F, Musu G, Giannetti F, Bos JM, Barbieri M, Cerea P, Giovenzana FLF, Torchio M, Mura M, Gnecchi M, Conte G, Auricchio A, Sala L, Odening KE, Ackerman MJ, Schwartz PJ
Circulation 2024 Aug 13;150(7):531-543. Epub 2024 Jun 28 doi: 10.1161/CIRCULATIONAHA.124.068959. PMID: 38939955
Independent validation and clinical implications of the risk prediction model for long QT syndrome (1-2-3-LQTS-Risk).
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
High Prevalence of Late-Appearing T-Wave in Patients With Long QT Syndrome Type 8.
Fukuyama M, Ohno S, Ozawa J, Kato K, Makiyama T, Nakagawa Y, Horie M
Circ J 2020 Mar 25;84(4):559-568. Epub 2020 Mar 12 doi: 10.1253/circj.CJ-19-1101. PMID: 32161207
Multiple mechanisms underlie increased cardiac late sodium current.
Kroncke BM, Yang T, Roden DM
Heart Rhythm 2019 Jul;16(7):1091-1097. Epub 2019 Jan 21 doi: 10.1016/j.hrthm.2019.01.018. PMID: 30677491Free PMC Article
Eleclazine exhibits enhanced selectivity for long QT syndrome type 3-associated late Na(+) current.
El-Bizri N, Xie C, Liu L, Limberis J, Krause M, Hirakawa R, Nguyen S, Tabuena DR, Belardinelli L, Kahlig KM
Heart Rhythm 2018 Feb;15(2):277-286. Epub 2017 Oct 7 doi: 10.1016/j.hrthm.2017.09.028. PMID: 29017927

Clinical prediction guides

Therapeutic Efficacy of Mexiletine for Long QT Syndrome Type 2: Evidence From Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes, Transgenic Rabbits, and Patients.
Crotti L, Neves R, Dagradi F, Musu G, Giannetti F, Bos JM, Barbieri M, Cerea P, Giovenzana FLF, Torchio M, Mura M, Gnecchi M, Conte G, Auricchio A, Sala L, Odening KE, Ackerman MJ, Schwartz PJ
Circulation 2024 Aug 13;150(7):531-543. Epub 2024 Jun 28 doi: 10.1161/CIRCULATIONAHA.124.068959. PMID: 38939955
Modeling incomplete penetrance in long QT syndrome type 3 through ion channel heterogeneity: an in silico population study.
Miller JA, Moise N, Weinberg SH
Am J Physiol Heart Circ Physiol 2023 Feb 1;324(2):H179-H197. Epub 2022 Dec 9 doi: 10.1152/ajpheart.00430.2022. PMID: 36487185Free PMC Article
Independent validation and clinical implications of the risk prediction model for long QT syndrome (1-2-3-LQTS-Risk).
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
Purkinje system hyperexcitability and ventricular arrhythmia risk in type 3 long QT syndrome.
Barake W, Giudicessi JR, Asirvatham SJ, Ackerman MJ
Heart Rhythm 2020 Oct;17(10):1768-1776. Epub 2020 May 23 doi: 10.1016/j.hrthm.2020.05.019. PMID: 32454217
Eleclazine exhibits enhanced selectivity for long QT syndrome type 3-associated late Na(+) current.
El-Bizri N, Xie C, Liu L, Limberis J, Krause M, Hirakawa R, Nguyen S, Tabuena DR, Belardinelli L, Kahlig KM
Heart Rhythm 2018 Feb;15(2):277-286. Epub 2017 Oct 7 doi: 10.1016/j.hrthm.2017.09.028. PMID: 29017927

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