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Review
. 2022 May;8(5):687-706.
doi: 10.1016/j.jacep.2022.02.017.

Congenital Long QT Syndrome

Andrew D Krahn  1 , Zachary Laksman  2 , Raymond W Sy  3 , Pieter G Postema  4 , Michael J Ackerman  5 , Arthur A M Wilde  6 , Hui-Chen Han  7
Affiliations
Free article
Review

Congenital Long QT Syndrome

Andrew D Krahn et al. JACC Clin Electrophysiol. 2022 May.
Free article

Abstract

Congenital long QT syndrome (LQTS) encompasses a group of heritable conditions that are associated with cardiac repolarization dysfunction. Since its initial description in 1957, our understanding of LQTS has increased dramatically. The prevalence of LQTS is estimated to be ∼1:2,000, with a slight female predominance. The diagnosis of LQTS is based on clinical, electrocardiogram, and genetic factors. Risk stratification of patients with LQTS aims to identify those who are at increased risk of cardiac arrest or sudden cardiac death. Factors including age, sex, QTc interval, and genetic background all contribute to current risk stratification paradigms. The management of LQTS involves conservative measures such as the avoidance of QT-prolonging drugs, pharmacologic measures with nonselective β-blockers, and interventional approaches such as device therapy or left cardiac sympathetic denervation. In general, most forms of exercise are considered safe in adequately treated patients, and implantable cardioverter-defibrillator therapy is reserved for those at the highest risk. This review summarizes our current understanding of LQTS and provides clinicians with a practical approach to diagnosis and management.

Keywords: arrhythmia; inherited; sudden death.

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Conflict of interest statement

Funding Support and Author Disclosures The study was supported by the Heart in Rhythm Organization (Dr Krahn, Principal Investigator), which receives support from the Canadian Institute of Health Research (RN380020-406814). Dr Krahn has received support from the Sauder Family and Heart and Stroke Foundation Chair in Cardiology (Vancouver, BC), the Paul Brunes Chair in Heart Rhythm Disorders (Vancouver, BC), and the Paul Albrechtson Foundation (Winnipeg, MB). Dr Laksman has received support from The University of British Columbia, Department of Medicine and the School of Biomedical Engineering, The University of British Columbia Cardiology Academic Practice Plan. Dr Ackerman receives support from The Mayo Clinic Windland Smith Rice Comprehensive Sudden Cardiac Death Program. Dr Wilde has received support from the Netherlands CardioVascular Research Initiative”: the Dutch Heart Foundation, Dutch Federation of University Medical Centres, the Netherlands Organisation for Health Research and Development, and the Royal Netherlands Academy of Sciences (PREDICT2). Dr Han is supported by a Robert and Elizabeth Albert Travel Grant from the RACP Foundation, Australia. Dr Laksman is a consultant for Abbott, Medtronic, and Boston Scientific. Dr Ackerman is a consultant for Abbott, ARMGO Pharma, Boston Scientific, Daiichi-Sankyo, Invitae, LQT Therapeutics, Medtronic, and UpToDate; and holds equity in Alivecor and Anumana. Dr Wilde is a member of the scientific advisory board for LQT therapeutics. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

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