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Síndrome do QT longo

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  • Theory
  • Diagnosis
  • Management
  • Follow up
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Last reviewed: 20 Aug 2025
Last updated: 29 Aug 2023

Summary

Определение

Анамнез и осмотр

Ключевые диагностические факторы

  • história de mutação genética conhecida
  • uso de medicamentos ou circunstâncias conhecidos por aumentar o intervalo QT
  • síncope durante elevações do tônus adrenérgico
  • síncope durante excitação ou surpresa
  • sintomas arrítmicos pós-parto
  • síncope no repouso e durante bradicardia
  • síncope cardíaca
  • palpitações
  • paralisia periódica
  • características dismórficas
  • surdez neurossensorial
Полная информация

Другие диагностические факторы

  • tontura
  • angina
  • fadiga
  • oligúria
  • fraqueza muscular
  • tetania
  • dormência
  • sinal de Chvostek
  • sinal de Trousseau
  • membros frios e pálidos
  • hipotensão
  • confusão
Полная информация

Факторы риска

  • mutações no gene KCNQ1
  • mutações no gene KCNH2
  • mutações no gene SCN5A
  • medicamentos que prolongam o intervalo QT
  • hipocalemia
  • hipomagnesemia
  • hipocalcemia
  • bradiarritmias
  • lesões no sistema nervoso central
  • sexo feminino
  • desnutrição
Полная информация

Диагностические исследования

Исследования, которые показаны в первую очередь

  • eletrocardiograma (ECG) para QTL1
  • eletrocardiograma (ECG) para QTL2
  • eletrocardiograma (ECG) para QTL3
  • eletrocardiograma (ECG) para hipocalemia e hipomagnesemia
  • eletrocardiograma (ECG) para hipocalcemia
  • Eletrocardiograma para bloqueio atrioventricular (AV) total
  • potássio sérico
  • magnésio sérico
  • cálcio sérico
Полная информация

Исследования, проведение которых нужно рассмотреть

  • monitor Holter
  • teste de tolerância ao exercício
  • ecocardiografia
  • teste genético
  • teste de adrenalina
Полная информация

Алгоритм лечения

Острый

SQTL adquirida

síndrome do QT longo (SQTL) congênita sem evento cardíaco prévio

síndrome do QT longo (SQTL) congênita com evento cardíaco prévio

Составители

Авторы

Mehmet K. Aktas, MD, MBA

Associate Professor of Medicine

University of Rochester Medical Center

Rochester

NY

Раскрытие информации

MKA declares that he has received research grants from Astra Zeneca, Medtronic, and Boston Scientific. MKA has served as consultant for Abbott and Huxley Medical. MKA holds a patent for ‘ECG Clock Electrocardiogram Based Diagnostic Device and Method’ (US patent #10,085,667). He is the author of a reference cited in this topic.

James P. Daubert, MD

Professor of Medicine

Duke University Medical Center

Duke Clinical Research Institute

Durham

NC

Раскрытие информации

JPD declares that he has received honoraria for advisory boards, steering committees, data safety monitoring boards, events committees, and lecture fees from Abbott, Acutus Medical, Affera Inc., Biosense Webster, Biotronik, Boston Scientific, Farapulse, Gilead Sciences Inc., Medtronic, Microport, Phillips, and Vytronus; and research grants from Abbott, Biosense Webster, Boston Scientific, Farapulse, and Medtronic. He receives royalty payments from Wiley, Springboard and McGraw Hill for textbook authorship and educational materials. JPD declares that he has no stock, stock options, or other forms of ownership. He is the author of a reference cited in this topic.

Рецензенты

Sami Viskin, MD

Director of Cardiac Hospitalization

Department of Cardiology

Tel Aviv Medical Center

Tel Aviv

Israel

Disclosures

SV declares that he has no competing interests.

Elizabeth S. Kaufman, MD

Associate Professor

Heart and Vascular Center

MetroHealth Campus

Case Western Reserve University

Cleveland

OH

Disclosures

ESK declares that she has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126.Full text  Abstract

Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013 Dec;10(12):1932-63.Full text  Abstract

Wilde AAM, Semsarian C, Márquez MF, et al. European Heart Rhythm Association (EHRA)/Heart Rhythm Society (HRS)/Asia Pacific Heart Rhythm Society (APHRS)/Latin American Heart Rhythm Society (LAHRS) expert consensus statement on the state of genetic testing for cardiac diseases. Europace. 2022 Sep 1;24(8):1307-67.Full text  Abstract

Roden DM. Clinical practice. Long-QT syndrome. N Engl J Med. 2008 Jan 10;358(2):169-76. Abstract

Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force on clinical practice guidelines and the Heart Rhythm Society. Circulation. 2018 Sep 25;138(13):e272-391.Full text  Abstract

Sauer AJ, Moss AJ, McNitt S, et al. Long QT syndrome in adults. J Am Coll Cardiol. 2007 Jan 23;49(3):329-37. Abstract

Schwartz PJ, Moss AJ, Vincent GM, et al. Diagnostic criteria for the long QT syndrome. An update. Circulation. 1993 Aug;88(2):782-4. Abstract

Schwartz PJ, Crotti L. QTc behavior during exercise and genetic testing for the long-QT syndrome. Circulation. 2011 Nov 15;124(20):2181-4.Full text  Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Síndrome do QT longo images

  • Differentials

    • Cardiopatia estrutural adquirida
    • Síncope neurocardiogênica (vasovagal)
    • Síncope neurológica
    More Differentials

  • Guidelines

    • European Heart Rhythm Association (EHRA)/Heart Rhythm Society (HRS)/Asia Pacific Heart Rhythm Society (APHRS)/Latin American Heart Rhythm Society (LAHRS) Expert Consensus Statement on the state of genetic testing for cardiac diseases
    • 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death
    More Guidelines

  • Patient information

    Ataque cardíaco: o que é?

    Ataque cardíaco: perguntas a fazer ao seu médico

    More Patient information

  • Calculators

    Correção do intervalo QT

    More Calculators
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