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Propranolol syrup to tablets change triggers electrical storm in Jervell-Lange-Nielsen syndrome
Javier Ramos-Jiménez1, Inmaculada Sánchez-Perez2, Nicolás Moreno-Mata3
1Department of Cardiology, University Hospital Ramón y Cajal, Madrid, Spain.
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.
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