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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 - 1 Nov 2016

Früh Andreas, Siem Geir, Holmström Henrik, Døhlen Gaute, Haugaa Kristina H

Abstract excerpt

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...

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PMID: 27451284
DOI: 10.1016/j.hrthm.2016.07.020

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