Congenital Long QT Syndrome: A Focus on Risk Stratification and Management

Congenital long QT syndrome (LQTs) is an inherited cardiac condition resulting from cardiac repolarization abnormalities. Since the initial description of congenital LQTs by Jervell and Lange-Nielsen in 1957, our understanding of this condition has increased dramatically. A diagnosis of congenital L...

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Main Authors: Deepti Ranganathan, Steffany Grondin, Raouane Hadjeres, Jacqueline Joza
Format: Article
Language:English
Published: IMR Press 2025-06-01
Series:Reviews in Cardiovascular Medicine
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Online Access:https://www.imrpress.com/journal/RCM/26/6/10.31083/RCM36779
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author Deepti Ranganathan
Steffany Grondin
Raouane Hadjeres
Jacqueline Joza
author_facet Deepti Ranganathan
Steffany Grondin
Raouane Hadjeres
Jacqueline Joza
author_sort Deepti Ranganathan
collection DOAJ
description Congenital long QT syndrome (LQTs) is an inherited cardiac condition resulting from cardiac repolarization abnormalities. Since the initial description of congenital LQTs by Jervell and Lange-Nielsen in 1957, our understanding of this condition has increased dramatically. A diagnosis of congenital LQTs is based on the medical history of the patient, alongside electrogram features, and a genetic variant that is identified in approximately 75% of cases. The appropriate risk stratification involves a multitude of factors, with β-blockers being the cornerstone of therapy. Recent developments, such as the incorporation of artificial intelligence (AI) for electrocardiogram (ECG) interpretation, genotype–phenotype-specific therapies, and emerging gene therapies, may potentially make personalized medicine in LQTs a reality in the near future. This review summarizes our current understanding of congenital LQTs, with a focus on risk stratification, current therapeutic interventions, and emerging developments in the management of congenital LQTs.
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institution Kabale University
issn 1530-6550
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publishDate 2025-06-01
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series Reviews in Cardiovascular Medicine
spelling doaj-art-01f8629f13aa4bd89bad26819e0f960c2025-08-20T03:28:59ZengIMR PressReviews in Cardiovascular Medicine1530-65502025-06-012663677910.31083/RCM36779S1530-6550(25)01871-XCongenital Long QT Syndrome: A Focus on Risk Stratification and ManagementDeepti Ranganathan0Steffany Grondin1Raouane Hadjeres2Jacqueline Joza3Department of Cardiology, McGill University Health Centre, Montreal, QC H4A 3J1, CanadaDepartment of Cardiology, McGill University Health Centre, Montreal, QC H4A 3J1, CanadaDepartment of Cardiology, McGill University Health Centre, Montreal, QC H4A 3J1, CanadaDepartment of Cardiology, McGill University Health Centre, Montreal, QC H4A 3J1, CanadaCongenital long QT syndrome (LQTs) is an inherited cardiac condition resulting from cardiac repolarization abnormalities. Since the initial description of congenital LQTs by Jervell and Lange-Nielsen in 1957, our understanding of this condition has increased dramatically. A diagnosis of congenital LQTs is based on the medical history of the patient, alongside electrogram features, and a genetic variant that is identified in approximately 75% of cases. The appropriate risk stratification involves a multitude of factors, with β-blockers being the cornerstone of therapy. Recent developments, such as the incorporation of artificial intelligence (AI) for electrocardiogram (ECG) interpretation, genotype–phenotype-specific therapies, and emerging gene therapies, may potentially make personalized medicine in LQTs a reality in the near future. This review summarizes our current understanding of congenital LQTs, with a focus on risk stratification, current therapeutic interventions, and emerging developments in the management of congenital LQTs.https://www.imrpress.com/journal/RCM/26/6/10.31083/RCM36779long qt syndromeqt prolongationrisk stratification
spellingShingle Deepti Ranganathan
Steffany Grondin
Raouane Hadjeres
Jacqueline Joza
Congenital Long QT Syndrome: A Focus on Risk Stratification and Management
Reviews in Cardiovascular Medicine
long qt syndrome
qt prolongation
risk stratification
title Congenital Long QT Syndrome: A Focus on Risk Stratification and Management
title_full Congenital Long QT Syndrome: A Focus on Risk Stratification and Management
title_fullStr Congenital Long QT Syndrome: A Focus on Risk Stratification and Management
title_full_unstemmed Congenital Long QT Syndrome: A Focus on Risk Stratification and Management
title_short Congenital Long QT Syndrome: A Focus on Risk Stratification and Management
title_sort congenital long qt syndrome a focus on risk stratification and management
topic long qt syndrome
qt prolongation
risk stratification
url https://www.imrpress.com/journal/RCM/26/6/10.31083/RCM36779
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AT raouanehadjeres congenitallongqtsyndromeafocusonriskstratificationandmanagement
AT jacquelinejoza congenitallongqtsyndromeafocusonriskstratificationandmanagement