Pediatric Drugs

, Volume 16, Issue 6, pp 447–456

Congenital Long QT Syndromes: Prevalence, Pathophysiology and Management

Therapy in Practice

DOI: 10.1007/s40272-014-0090-4

Cite this article as:
Barsheshet, A., Dotsenko, O. & Goldenberg, I. Pediatr Drugs (2014) 16: 447. doi:10.1007/s40272-014-0090-4


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.

Copyright information

© Springer International Publishing Switzerland 2014

Authors and Affiliations

  • Alon Barsheshet
    • 1
    • 2
    • 3
  • Olena Dotsenko
    • 3
  • Ilan Goldenberg
    • 2
    • 3
    • 4
  1. 1.Cardiology DepartmentRabin Medical CenterPetach TikvaIsrael
  2. 2.Sackler Faculty of MedicineTel-Aviv UniversityTel AvivIsrael
  3. 3.Cardiology DivisionUniversity of Rochester Medical CenterRochesterUSA
  4. 4.Leviev Heart Center, Sheba Medical CenterTel HashomerIsrael