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

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?

Author information

1
Department of Paediatrics, Oslo University Hospital, Oslo, Norway.
2
Department of Otorhinolaryngology, Oslo University Hospital, Oslo, Norway.
3
Center for Cardiological Innovation, Department of Cardiology and Institute of Surgical Research, Oslo University Hospital, Oslo, Norway; University of Oslo, Oslo, Norway. Electronic address: kristina.haugaa@rr-research.no.

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.

KEYWORDS:

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

PMID:
27451284
DOI:
10.1016/j.hrthm.2016.07.020
[Indexed for MEDLINE]
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