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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)
 
Related genes: ALG10B, AKAP9, KCNE2, SNTA1, SCN5A, SCN4B, KCNQ1, KCNJ5, KCNH2, KCNE1, 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

Jowais J, Castro-Gonzalez LM, Golluscio A, Tieleman DP, Larsson HP
Br J Pharmacol 2026 Sep;183(17):5407-5420. Epub 2026 Jun 15 doi: 10.1111/bph.70532. PMID: 42298290
Farjam M, Yazdanpanah MH, Fereydouni N
Clin Cardiol 2026 Jun;49(6):e70370. doi: 10.1002/clc.70370. PMID: 42274161Free PMC Article
Sui F, Yan D, Meng X, Duan C, Zhang Y, Gao W, Song Z
Europace 2026 Jun 2;28(6) doi: 10.1093/europace/euag133. PMID: 42216678Free 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

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Harmanci N, Kaltus Z, Eroglu E, Cengelli Unel C, Yigitaslan S, Sirmagul B
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Diagnosis

Miyazaki Y, Aiba T, Kato K, Fukunaga H, Morita H, Noma T, Takahashi T, Mizukami A, Nakamura T, Oka S, Wakamiya A, Ueda N, Nakajima K, Kamakura T, Wada M, Ishibashi K, Inoue Y, Miyamoto K, Nagase S, Kusano K
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Zhou X, Yang W, Wang G, Hu Y, Lin X, Liu Y, Zhang P, Chen B
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Meloni A, Pistoia L, Ruffo GB, Longo F, Rosso R, Rossi V, Messina G, Peritore G, Zerbini M, Vallone N, Fina P, Positano V, Barison A
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Therapy

Chen Y, Gu Q, Zhao M, Zhao A, Kong Z, Shen H, Zhang Y, Wang S, Li L, Xie H, Liu P, Zhang H, Chen D
Cardiovasc Toxicol 2026 Sep 6;26(10) doi: 10.1007/s12012-026-10174-z. PMID: 42701947Free PMC Article
Aydın E, Kurt D, Gök G
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Dominguez-Gomez P, Zingaro A, Balzotti C, Leitner M, Jacobo-Piqueras N, Vazquez M, Rast G, Aguado-Sierra J
Regul Toxicol Pharmacol 2026 Nov;171:106138. Epub 2026 Jun 1 doi: 10.1016/j.yrtph.2026.106138. PMID: 42219143
Matsumoto Y, Mori Y, Kageyama S, Sato H
Ren Fail 2026 Dec;48(1):2641970. Epub 2026 Mar 12 doi: 10.1080/0886022X.2026.2641970. PMID: 41820013Free PMC Article

Prognosis

Docken SS, Marquis MJ, Ngo K, Wada Y, Kita S, Yarov-Yarovoy V, Clancy CE, Vorobyov I, Lewis TJ, Furutani K, Sack JT
J Gen Physiol 2026 Nov 2;158(6) Epub 2026 Aug 27 doi: 10.1085/jgp.202614015. PMID: 42658189
Taib C, El Makhzen N, Abriel H
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Jing W, Li W, Wang J, Wang Q, Zhu Q, Pan S, Li S, Chu N, Pang Y, Nie W
Int J Antimicrob Agents 2026 Oct;67(10):107923. Epub 2026 Jul 11 doi: 10.1016/j.ijantimicag.2026.107923. PMID: 42435993
Meloni A, Pistoia L, Ruffo GB, Longo F, Rosso R, Rossi V, Messina G, Peritore G, Zerbini M, Vallone N, Fina P, Positano V, Barison A
Eur J Haematol 2026 Oct;117(4):1026-1034. Epub 2026 Jun 15 doi: 10.1111/ejh.70242. PMID: 42297450Free PMC Article
Dominguez-Gomez P, Zingaro A, Balzotti C, Leitner M, Jacobo-Piqueras N, Vazquez M, Rast G, Aguado-Sierra J
Regul Toxicol Pharmacol 2026 Nov;171:106138. Epub 2026 Jun 1 doi: 10.1016/j.yrtph.2026.106138. PMID: 42219143

Clinical prediction guides

Docken SS, Marquis MJ, Ngo K, Wada Y, Kita S, Yarov-Yarovoy V, Clancy CE, Vorobyov I, Lewis TJ, Furutani K, Sack JT
J Gen Physiol 2026 Nov 2;158(6) Epub 2026 Aug 27 doi: 10.1085/jgp.202614015. PMID: 42658189
Zhou X, Yang W, Wang G, Hu Y, Lin X, Liu Y, Zhang P, Chen B
Comput Methods Programs Biomed 2026 Oct;285:109542. Epub 2026 Jul 11 doi: 10.1016/j.cmpb.2026.109542. PMID: 42442037
Jing W, Li W, Wang J, Wang Q, Zhu Q, Pan S, Li S, Chu N, Pang Y, Nie W
Int J Antimicrob Agents 2026 Oct;67(10):107923. Epub 2026 Jul 11 doi: 10.1016/j.ijantimicag.2026.107923. PMID: 42435993
Dominguez-Gomez P, Zingaro A, Balzotti C, Leitner M, Jacobo-Piqueras N, Vazquez M, Rast G, Aguado-Sierra J
Regul Toxicol Pharmacol 2026 Nov;171:106138. Epub 2026 Jun 1 doi: 10.1016/j.yrtph.2026.106138. PMID: 42219143
Matsumoto Y, Mori Y, Kageyama S, Sato H
Ren Fail 2026 Dec;48(1):2641970. Epub 2026 Mar 12 doi: 10.1080/0886022X.2026.2641970. PMID: 41820013Free PMC Article

Recent systematic reviews

Akman C, Ucar AB, Girgin MC, Erdem B, Yilmaz G, Beyoglu R, Karcioglu O
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Parizad R, Batta A, Hatwal J, Brar A, Mohan B
Heart Int 2026;20(1):20-34. Epub 2026 Jun 2 doi: 10.17925/HI.2026.20.1.3. PMID: 42293057Free PMC Article
Carneiro IMC, Castelo Branco PES, Franco AHS, Oliveira AP, Aguiar AMA
Rev Paul Pediatr 2026;44:e2025134. Epub 2026 Mar 23 doi: 10.1590/1984-0462/2026/44/2025134. PMID: 41880414Free PMC Article
Bonanni F, Capodici A, Gentile F, Moschetti F, Passino C, Di Paolo M, Olivotto I, Emdin M, Giannoni A
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