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?

Abstract

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.

• Authors: Andreas Früh, Geir Siem, Henrik Holmström, Gaute Døhlen, Kristina H Haugaa
• Journal: Heart rhythm (Heart Rhythm) Vol. 13 Issue 11 Pg. 2186-2192 (11 2016) ISSN: 1556-3871 [Electronic] United States
• PMID: 27451284 (Publication Type: Journal Article)
• Copyright: Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

Chemical References

• Adrenergic beta-Antagonists
• KCNQ1 Potassium Channel

Topics

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