Hostname: page-component-848d4c4894-nmvwc Total loading time: 0 Render date: 2024-06-29T15:14:11.359Z Has data issue: false hasContentIssue false
  • English
  • Français

Propranolol syrup to tablets change triggers electrical storm in Jervell-Lange-Nielsen syndrome

Published online by Cambridge University Press:  13 October 2020

Javier Ramos-Jiménez Open the ORCID record for Javier Ramos-Jiménez [Opens in a new window] ,
Inmaculada Sánchez-Perez ,
Nicolás Moreno-Mata  and
Javier Moreno-Planas
Javier Ramos-Jiménez*
Affiliation:
Department of Cardiology, University Hospital Ramón y Cajal, Madrid, Spain
Inmaculada Sánchez-Perez
Affiliation:
Department of Paediatric Cardiology, University Hospital Ramón y Cajal, Madrid, Spain
Nicolás Moreno-Mata
Affiliation:
Department of Thoracic Surgery, University Hospital Ramón y Cajal, Madrid, Spain
Javier Moreno-Planas
Affiliation:
Department of Cardiology, University Hospital Ramón y Cajal, Madrid, Spain
*
Author for correspondence: Javier Ramos Jiménez, MD, Cardiology Department, University Hospital Ramón y Cajal, Ctra. de Colmenar, Km 9,100, C.P.: 28034, Madrid, Spain. Tel: +34 691 680 034; Fax: +34 91 336 85 15. E-mail: jramj90@gmail.com
Get access Rights & Permissions [Opens in a new window]

Abstract

A 10-year-old girl with genetically confirmed Jervell-Lange-Nielsen syndrome treated with beta-blocker and developed electrical storm after changing propranolol syrup to tablets. Jervell-Lange-Nielsen is characterised by long QT and congenital sensorineural deafness, with high risk of malignant arrhythmias at early ages. Gastric involvement and achlorhydria may be present, with subsequent alteration of medication bioavailability which can trigger severe arrhythmic complications.

Keywords

Jervell-Lange-Nielsen syndromelong QT intervalelectrical stormbioavailabilitythoracic sympathectomy
Type
Brief Report
Information
Cardiology in the Young , Volume 31 , Issue 1 , January 2021 , pp. 141 - 143
Check for updates
Copyright
© The Author(s), 2020. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Schwartz Peter, J, Carla, S, Lia, C, et al. The Jervell and Lange-Nielsen syndrome. Circulation 2006; 113: 783–790.CrossRefGoogle Scholar
Rice, KS, Dickson, G, Lane, M, et al. Elevated serum gastrin levels in Jervell and Lange-Nielsen syndrome: a marker of severe KCNQ1 dysfunction? Heart Rhythm 2011; 8: 551–554.Google ScholarPubMed
Sweetman, SC. Martindale: The Complete Drug Reference. London, Chicago: Pharmaceutical Press, 2009: 1381–1382.Google Scholar
Al-Khatib Sana, M, Stevenson William, G, Ackerman Michael, J, Bryant William, J, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Circulation. 2018; 138: e272–391.Google Scholar