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Long QT syndrome 10(LQT10)

MedGen UID:: 394836
•Concept ID:: C2678484
•: Disease or Syndrome

Synonym:	LQT10

Gene (location): Gene(s) directly associated with this condition or phenotype.	SCN4B (11q23.3)

Monarch Initiative:	MONDO:0012737
OMIM®:	611819

Definition

Any long QT syndrome in which the cause of the disease is a mutation in the SCN4B gene. [from MONDO]

Clinical features

From HPO

Sudden cardiac death

MedGen UID:: 38841
•Concept ID:: C0085298
•: Pathologic Function

The heart suddenly and unexpectedly stops beating resulting in death within a short time period (generally within 1 h of symptom onset).

See: Feature record | Search on this feature

Atrial fibrillation

MedGen UID:: 445
•Concept ID:: C0004238
•: Finding

An atrial arrhythmia characterized by disorganized atrial activity without discrete P waves on the surface EKG, but instead by an undulating baseline or more sharply circumscribed atrial deflections of varying amplitude an frequency ranging from 350 to 600 per minute.

See: Feature record | Search on this feature

Atrioventricular block

MedGen UID:: 13956
•Concept ID:: C0004245
•: Disease or Syndrome

Delayed or lack of conduction of atrial depolarizations through the atrioventricular node to the ventricles.

See: Feature record | Search on this feature

Prolonged QT interval

MedGen UID:: 57494
•Concept ID:: C0151878
•: Finding

Increased time between the start of the Q wave and the end of the T wave as measured by the electrocardiogram (EKG).

See: Feature record | Search on this feature

T-wave alternans

MedGen UID:: 745313
•Concept ID:: C1998313
•: Finding

A periodic beat-to-beat variation in the amplitude or shape of the T wave in an EKG.

See: Feature record | Search on this feature

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Abnormality of the cardiovascular system
Constitutional symptom
- Sudden cardiac death

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

Congenital long QT syndrome
- Congenital long QT syndrome
  - Congenital long QT syndrome
    - Congenital long QT syndrome
      - Congenital long QT syndrome
        Congenital long QT syndrome
        Long QT syndrome 10

Professional guidelines

PubMed

Spotlight on the 2022 ESC guideline management of ventricular arrhythmias and prevention of sudden cardiac death: 10 novel key aspects.

Könemann H, Dagres N, Merino JL, Sticherling C, Zeppenfeld K, Tfelt-Hansen J, Eckardt L
Europace 2023 May 19;25(5) doi: 10.1093/europace/euad091. PMID: 37102266 Free PMC Article

Delamanid and QT prolongation in the treatment of multidrug-resistant tuberculosis.

Gupta R, Geiter LJ, Hafkin J, Wells CD
Int J Tuberc Lung Dis 2015 Oct;19(10):1261-2. doi: 10.5588/ijtld.15.0541. PMID: 26459547

Genetic testing for long-QT syndrome: distinguishing pathogenic mutations from benign variants.

Kapa S, Tester DJ, Salisbury BA, Harris-Kerr C, Pungliya MS, Alders M, Wilde AA, Ackerman MJ
Circulation 2009 Nov 3;120(18):1752-60. Epub 2009 Oct 19 doi: 10.1161/CIRCULATIONAHA.109.863076. PMID: 19841300 Free PMC Article

See all (124)

Recent clinical studies

Etiology

Antipsychotics and risk of QT prolongation: a pharmacovigilance study.

Bordet C, Garcia P, Salvo F, Touafchia A, Galinier M, Sommet A, Montastruc F
Psychopharmacology (Berl) 2023 Jan;240(1):199-202. Epub 2022 Dec 14 doi: 10.1007/s00213-022-06293-4. PMID: 36515735

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

Managing drug-induced QT prolongation in clinical practice.

Khatib R, Sabir FRN, Omari C, Pepper C, Tayebjee MH
Postgrad Med J 2021 Jul;97(1149):452-458. Epub 2020 Oct 29 doi: 10.1136/postgradmedj-2020-138661. PMID: 33122341 Free PMC Article

The measurement of the QT interval.

Postema PG, Wilde AA
Curr Cardiol Rev 2014 Aug;10(3):287-94. doi: 10.2174/1573403x10666140514103612. PMID: 24827793 Free PMC Article

A comparison of the risk of QT prolongation among SSRIs.

Funk KA, Bostwick JR
Ann Pharmacother 2013 Oct;47(10):1330-41. Epub 2013 Oct 21 doi: 10.1177/1060028013501994. PMID: 24259697

See all (1063)

Diagnosis

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

Polymorphic Ventricular Tachycardia: Terminology, Mechanism, Diagnosis, and Emergency Therapy.

Viskin S, Chorin E, Viskin D, Hochstadt A, Schwartz AL, Rosso R
Circulation 2021 Sep 7;144(10):823-839. doi: 10.1161/CIRCULATIONAHA.121.055783. PMID: 34491774

Enhancing rare variant interpretation in inherited arrhythmias through quantitative analysis of consortium disease cohorts and population controls.

Walsh R, Lahrouchi N, Tadros R, Kyndt F, Glinge C, Postema PG, Amin AS, Nannenberg EA, Ware JS, Whiffin N, Mazzarotto F, Škorić-Milosavljević D, Krijger C, Arbelo E, Babuty D, Barajas-Martinez H, Beckmann BM, Bézieau S, Bos JM, Breckpot J, Campuzano O, Castelletti S, Celen C, Clauss S, Corveleyn A, Crotti L, Dagradi F, de Asmundis C, Denjoy I, Dittmann S, Ellinor PT, Ortuño CG, Giustetto C, Gourraud JB, Hazeki D, Horie M, Ishikawa T, Itoh H, Kaneko Y, Kanters JK, Kimoto H, Kotta MC, Krapels IPC, Kurabayashi M, Lazarte J, Leenhardt A, Loeys BL, Lundin C, Makiyama T, Mansourati J, Martins RP, Mazzanti A, Mörner S, Napolitano C, Ohkubo K, Papadakis M, Rudic B, Molina MS, Sacher F, Sahin H, Sarquella-Brugada G, Sebastiano R, Sharma S, Sheppard MN, Shimamoto K, Shoemaker MB, Stallmeyer B, Steinfurt J, Tanaka Y, Tester DJ, Usuda K, van der Zwaag PA, Van Dooren S, Van Laer L, Winbo A, Winkel BG, Yamagata K, Zumhagen S, Volders PGA, Lubitz SA, Antzelevitch C, Platonov PG, Odening KE, Roden DM, Roberts JD, Skinner JR, Tfelt-Hansen J, van den Berg MP, Olesen MS, Lambiase PD, Borggrefe M, Hayashi K, Rydberg A, Nakajima T, Yoshinaga M, Saenen JB, Kääb S, Brugada P, Robyns T, Giachino DF, Ackerman MJ, Brugada R, Brugada J, Gimeno JR, Hasdemir C, Guicheney P, Priori SG, Schulze-Bahr E, Makita N, Schwartz PJ, Shimizu W, Aiba T, Schott JJ, Redon R, Ohno S, Probst V; Nantes Referral Center for inherited cardiac arrhythmia, Behr ER, Barc J, Bezzina CR
Genet Med 2021 Jan;23(1):47-58. Epub 2020 Sep 7 doi: 10.1038/s41436-020-00946-5. PMID: 32893267 Free PMC Article

Development and validation of a risk score to predict QT interval prolongation in hospitalized patients.

Tisdale JE, Jaynes HA, Kingery JR, Mourad NA, Trujillo TN, Overholser BR, Kovacs RJ
Circ Cardiovasc Qual Outcomes 2013 Jul;6(4):479-87. Epub 2013 May 28 doi: 10.1161/CIRCOUTCOMES.113.000152. PMID: 23716032 Free PMC Article

Genetic testing for long-QT syndrome: distinguishing pathogenic mutations from benign variants.

See all (820)

Therapy

Antipsychotics and risk of QT prolongation: a pharmacovigilance study.

Bordet C, Garcia P, Salvo F, Touafchia A, Galinier M, Sommet A, Montastruc F
Psychopharmacology (Berl) 2023 Jan;240(1):199-202. Epub 2022 Dec 14 doi: 10.1007/s00213-022-06293-4. PMID: 36515735

Right-Sided Pneumothorax After Cardioverter-Defibrillator Implantation.

Morales-Estrella J, Huded C, Bhargava M, Gildea T, Sampaio Rodrigues T, Almeida FA
Heart Lung Circ 2019 Oct;28(10):e137-e138. Epub 2019 Apr 5 doi: 10.1016/j.hlc.2019.03.011. PMID: 31006594

Citalopram, QTc Prolongation, and Torsades de Pointes.

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

Meta-analysis of selective serotonin reuptake inhibitor-associated QTc prolongation.

Beach SR, Kostis WJ, Celano CM, Januzzi JL, Ruskin JN, Noseworthy PA, Huffman JC
J Clin Psychiatry 2014 May;75(5):e441-9. doi: 10.4088/JCP.13r08672. PMID: 24922496

A comparison of the risk of QT prolongation among SSRIs.

Funk KA, Bostwick JR
Ann Pharmacother 2013 Oct;47(10):1330-41. Epub 2013 Oct 21 doi: 10.1177/1060028013501994. PMID: 24259697

See all (786)

Prognosis

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

Development and validation of a risk score to predict QT interval prolongation in hospitalized patients.

Paralogous annotation of disease-causing variants in long QT syndrome genes.

Ware JS, Walsh R, Cunningham F, Birney E, Cook SA
Hum Mutat 2012 Aug;33(8):1188-1191. Epub 2012 Jun 7 doi: 10.1002/humu.22114. PMID: 22581653 Free PMC Article

Genetic testing for long-QT syndrome: distinguishing pathogenic mutations from benign variants.

QT interval dispersion in dialysis patients.

Wu VC, Lin LY, Wu KD
Nephrology (Carlton) 2005 Apr;10(2):109-12. doi: 10.1111/j.1440-1797.2005.00391.x. PMID: 15877667

See all (550)

Clinical prediction guides

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

Development and validation of a risk score to predict QT interval prolongation in hospitalized patients.

Paralogous annotation of disease-causing variants in long QT syndrome genes.

Ware JS, Walsh R, Cunningham F, Birney E, Cook SA
Hum Mutat 2012 Aug;33(8):1188-1191. Epub 2012 Jun 7 doi: 10.1002/humu.22114. PMID: 22581653 Free PMC Article

Genetic testing for long-QT syndrome: distinguishing pathogenic mutations from benign variants.

QT interval dispersion in dialysis patients.

Wu VC, Lin LY, Wu KD
Nephrology (Carlton) 2005 Apr;10(2):109-12. doi: 10.1111/j.1440-1797.2005.00391.x. PMID: 15877667

See all (682)

Recent systematic reviews

Comparison of methods for correcting QT interval in athletes and young people: A systematic review.

Mahendran S, Gupta I, Davis J, Davis AJ, Orchard JW, Orchard JJ
Clin Cardiol 2023 Sep;46(9):1106-1115. Epub 2023 Jul 20 doi: 10.1002/clc.24093. PMID: 37470093 Free PMC Article

Evaluation of the QT interval in patients with drug-induced QT prolongation and torsades de pointes.

Krisai P, Vlachos K, Ramirez FD, Nakatani Y, Nakashima T, Takagi T, Kamakura T, Surget E, André C, Cheniti G, Welte N, Chauvel R, Tixier R, Duchateau J, Pambrun T, Derval N, Hocini M, Jaïs P, Haïssaguerre M, Sacher F
J Cardiovasc Electrophysiol 2020 Oct;31(10):2696-2701. Epub 2020 Jul 28 doi: 10.1111/jce.14687. PMID: 32700358

Safety of azithromycin in pediatrics: a systematic review and meta-analysis.

Zeng L, Xu P, Choonara I, Bo Z, Pan X, Li W, Ni X, Xiong T, Chen C, Huang L, Qazi SA, Mu D, Zhang L
Eur J Clin Pharmacol 2020 Dec;76(12):1709-1721. Epub 2020 Jul 17 doi: 10.1007/s00228-020-02956-3. PMID: 32681202 Free PMC Article

Prolonged corrected QT interval in predicting atrial fibrillation: A systematic review and meta-analysis.

Zhang N, Gong M, Tse G, Zhang Z, Meng L, Yan BP, Zhang L, Wu G, Xia Y, Xin-Yan G, Li G, Liu T
Pacing Clin Electrophysiol 2018 Mar;41(3):321-327. Epub 2018 Feb 16 doi: 10.1111/pace.13292. PMID: 29380395

Drug-induced QT prolongation and torsades de pointes: evaluation of a QT nomogram.

Chan A, Isbister GK, Kirkpatrick CM, Dufful SB
QJM 2007 Oct;100(10):609-15. Epub 2007 Sep 19 doi: 10.1093/qjmed/hcm072. PMID: 17881416

See all (20)

MedGen

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Long QT syndrome 10(LQT10)

Definition

Clinical features

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Genetic Testing Registry

Clinical resources

Practice guidelines

Molecular resources

Consumer resources

Reviews

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