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Published in:

01-12-2014 | Therapy in Practice

Congenital Long QT Syndromes: Prevalence, Pathophysiology and Management

Authors: Alon Barsheshet, Olena Dotsenko, Ilan Goldenberg

Published in: Pediatric Drugs | Issue 6/2014

Abstract

Long QT syndrome is a genetic disorder associated with life threatening ventricular arrhythmias and sudden death. This inherited arrhythmic disorder exhibits genetic heterogeneity, incomplete penetrance, and variable expressivity. During the past two decades there have been major advancements in understanding the genotype-phenotype correlations in LQTS. This genotype-phenotype relationship can lead to improved management of LQTS. However, development of genotype-specific or mutation-specific management strategies is very challenging. This review describes the pathophysiology of LQTS, genotype-phenotype correlations, and focuses on the management of LQTS. In general, the treatment of LQTS consists of lifestyle modifications, medical therapy with beta-blockers, device and surgical therapy. We further summarize current data on the efficacy of pharmacological treatment options for the three most prevalent LQTS variants including beta-blockers in LQT1, LQT2 and LQT3, sodium channel blockers and ranolazine for LQT3, potassium supplementation and spironolactone for LQT2, and possibly sex hormone-based therapy for LQT2.

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Title: Congenital Long QT Syndromes: Prevalence, Pathophysiology and Management
Authors: Alon Barsheshet
Olena Dotsenko
Ilan Goldenberg
Publication date: 01-12-2014
Publisher: Springer International Publishing
Published in: Pediatric Drugs / Issue 6/2014
Print ISSN: 1174-5878
Electronic ISSN: 1179-2019
DOI: https://doi.org/10.1007/s40272-014-0090-4

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Congenital Long QT Syndromes: Prevalence, Pathophysiology and Management

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

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