The Jervell and Lange-Nielsen syndrome; atrial pacing combined with ß-blocker therapy, a favorable approach in young high-risk patients with long QT syndrome?

Heart Rhythm. 2016 Nov;13(11):2186-2192. doi: 10.1016/j.hrthm.2016.07.020. Epub 2016 Jul 20.


Background: Patients with Jervell and Lange-Nielsen syndrome (JLNS) exhibit severe phenotypes that are characterized by congenital deafness, very long QT intervals, and high risk of life-threatening arrhythmias. Current treatment strategies include high doses of beta-blocker medication, left cardiac sympathetic denervation, and ICD placement, which is challenging in young children.

Objective: The purpose of this study was to evaluate the safety and effect of pacing in addition to beta-blocker treatment in children with JLNS.

Methods: All genetically confirmed patients with JLNS born since 1999 in Norway were included in the study. Data on history of long QT syndrome-related symptoms, QT interval, and beta-blocker and pacemaker treatment were recorded.

Results: A total of 9 patients with QT intervals ranging from 510 to 660 ms were identified. Eight patients developed long QT syndrome-related symptoms, and 1 patient died before diagnosis. The survivors received beta-blocker medication. Seven patients also received a pacemaker; 1 had a ventricular lead and 6 had atrial leads. The patient with the ventricular lead died during follow-up. The 6 patients with atrial leads survived without events at a mean follow-up of 6.9 years after pacemaker implantation. Two patients received prophylactic upgrade to a 2-chamber ICD.

Conclusion: No arrhythmic events occurred in 6 very young JLNS patients who received atrial pacing in combination with increased doses of beta-blockers during 7-year follow-up. If confirmed in additional patients, this treatment strategy may prevent life-threatening arrhythmias in this high-risk patient group and may act as a bridge to insertion of a 2-chamber ICD when left cardiac sympathetic denervation is not available.

Keywords: Arrhythmia; Cardiac pacing; Genetics; Implantable cardioverter–defibrillator; Jervell and Lange-Nielsen syndrome; Long QT syndrome.

MeSH terms

  • Adolescent
  • Adrenergic beta-Antagonists / administration & dosage*
  • Arrhythmias, Cardiac* / genetics
  • Arrhythmias, Cardiac* / prevention & control
  • Cardiac Pacing, Artificial / methods*
  • Child
  • Child, Preschool
  • Cochlear Implantation / methods
  • Deafness / congenital
  • Deafness / surgery
  • Defibrillators, Implantable
  • Dose-Response Relationship, Drug
  • Electrocardiography / methods
  • Female
  • Heart Conduction System / drug effects
  • Heart Conduction System / physiopathology
  • Humans
  • Infant
  • Jervell-Lange Nielsen Syndrome* / complications
  • Jervell-Lange Nielsen Syndrome* / epidemiology
  • Jervell-Lange Nielsen Syndrome* / physiopathology
  • Jervell-Lange Nielsen Syndrome* / therapy
  • KCNQ1 Potassium Channel / genetics*
  • Male
  • Mutation
  • Norway / epidemiology
  • Outcome and Process Assessment, Health Care
  • Sympathectomy / methods*


  • Adrenergic beta-Antagonists
  • KCNQ1 Potassium Channel