Long QT syndrome

Last reviewed: 23 Aug 2023
Last updated: 29 Aug 2023



History and exam

Key diagnostic factors

  • history of known gene mutation
  • use of drugs or circumstances known to increase the QT interval
  • syncope during heightened adrenergic tones
  • syncope during arousal or surprise
  • arrhythmic symptoms postpartum
  • syncope at rest and during bradycardia
  • cardiac syncope
  • palpitations
  • periodic paralysis
  • dysmorphic features
  • sensorineural deafness
More key diagnostic factors

Other diagnostic factors

  • dizziness
  • angina
  • fatigue
  • oliguria
  • muscle weakness
  • tetany
  • numbness
  • Chvostek's sign
  • Trousseau's sign
  • cold and pale extremities
  • hypotension
  • confusion
Other diagnostic factors

Risk factors

  • KCNQ1 gene mutations
  • KCNH2 gene mutations
  • SCN5A gene mutations
  • QT interval-prolonging drugs
  • hypokalemia
  • hypomagnesemia
  • hypocalcemia
  • bradyarrhythmias
  • central nervous system lesions
  • female sex
  • malnutrition
More risk factors

Diagnostic investigations

1st investigations to order

  • ECG for LQT1
  • ECG for LQT2
  • ECG for LQT3
  • ECG for hypokalemia and hypomagnesemia
  • ECG for hypocalcemia
  • ECG for complete atrioventricular (AV) block
  • serum potassium
  • serum magnesium
  • serum calcium
More 1st investigations to order

Investigations to consider

  • Holter monitor
  • exercise tolerance test
  • echocardiography
  • genetic testing
  • epinephrine test
More investigations to consider

Treatment algorithm


acquired LQTS

congenital LQTS without previous cardiac event

congenital LQTS with previous cardiac event



Mehmet K. Aktas, MD, MBA

Associate Professor of Medicine

University of Rochester Medical Center




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




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.

Peer reviewers

Sami Viskin, MD

Director of Cardiac Hospitalization

Department of Cardiology

Tel Aviv Medical Center

Tel Aviv



SV declares that he has no competing interests.

Elizabeth S. Kaufman, MD

Associate Professor

Heart and Vascular Center

MetroHealth Campus

Case Western Reserve University




ESK declares that she has no competing interests.

  • Long QT syndrome images
  • Differentials

    • Acquired structural heart disease
    • Neurocardiogenic (vasovagal) syncope
    • Neurologic syncope
    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 leaflets

    Heart attack: what is it?

    What you can do to prevent another heart attack

    More Patient leaflets
  • Calculators

    QT Interval Correction

    More Calculators
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