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Congenital Long QT Syndromes: Prevalence, Pathophysiology and Management

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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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Conflicts of interests

A. Barsheshet, O. Dotsenko and I. Goldenberg report no conflict of interest relevant to this article. No sources of funding were used to support the writing of the manuscript.

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Authors and Affiliations

  1. Cardiology Department, Rabin Medical Center, Petach Tikva, Israel

    Alon Barsheshet

  2. Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel

    Alon Barsheshet & Ilan Goldenberg

  3. Cardiology Division, University of Rochester Medical Center, Rochester, NY, USA

    Alon Barsheshet, Olena Dotsenko & Ilan Goldenberg

  4. Leviev Heart Center, Sheba Medical Center, 52621, Tel Hashomer, Israel

    Ilan Goldenberg

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  1. Alon Barsheshet
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Correspondence to Ilan Goldenberg.

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Barsheshet, A., Dotsenko, O. & Goldenberg, I. Congenital Long QT Syndromes: Prevalence, Pathophysiology and Management. Pediatr Drugs 16, 447–456 (2014). https://doi.org/10.1007/s40272-014-0090-4

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