What are the ESC treatment guidelines for long QT syndrome (LQTS) and preventions of sudden cardiac death (SCD)?

Updated: Nov 29, 2017
  • Author: Ali A Sovari, MD, FACP, FACC; Chief Editor: Mikhael F El-Chami, MD  more...
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Answer

The following is a summary of recommendations included in the 2015 ESC guidelines for management of of LQTS and preventions of SCD. [37]

Class I (Level of evidence: B)

Lifestyle changes, such as the following:

  • Avoidance of QT-prolonging drugs
  • Correction of electrolyte abnormalities (hypokalemia, hypomagnesemia, hypocalcemia) that may occur during diarrhea, vomiting, or metabolic conditions
  • Avoidance of genotype-specific triggers for arrhythmias (strenuous swimming, especially in LQTS1, and exposure to loud noises in LQTS2 patients)

Beta-blockers for all patients 

Implantable cardioverter-defibrillator (ICD) placement with the use of beta-blockers for patients with a previous cardiac arrest

Class IIa 

Consider beta-blockers for carriers of an LQTS genetic mutation and normal QT interval. (Level of evidence: B)

Consider ICD implantation in addition to beta-blockers in patients with syncope and/or ventricular tachycardia (VT) while receiving an adequate dose of beta-blockers. (Level of evidence: B)

Left cardiac sympathetic denervation should be considered in patients with symptomatic LQTS when (Level of evidence: C):

  • Beta-blockers are ineffective, not tolerated, or contraindicated
  • ICD therapy is contraindicated or refused
  • Patients on beta-blockers with an ICD experience multiple shocks

Class IIb (Level of evidence: C)

Consider sodium channel blockers (mexiletine, flecainide or ranolazine) as add-on therapy to shorten the QT interval in LQTS3 patients with a QTc longer than 500 ms.

Consider an ICD in addition to beta-blocker therapy in asymptomatic carriers of a pathogenic mutation in KCNH2 or SCN5A when the QTc is longer than 500 ms.

Class III (Level of evidence: C)

Invasive electrophysiological study (EPS) with programmed ventricular stimulation (PVS) is not recommended for SCD risk stratification.

The 2015 ESC recommendations summarized above are consistent with the recommendations of the 2006 joint guidelines of the American College of Cardiology, the American Heart Association, and the ESC (ACC/AHA/ESC). [39]  However, the 2013 HRS/EHRA/APHRS recommendations have one significant variance in that beta-blockers are only recommended in patients who are asymptomatic with a QTc of at least 470 ms and/or symptomatic for syncope or documented ventricular tachycardia/ventricular fibrillation (VT/VF). [36]


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