Propranolol
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Cardiac electrical storm: case report A 10-year-old girl developed cardiac electrical storm after changing propranolol syrup to tablet during treatment for Jervell and Lange-Nielsen syndrome. The girl was admitted to the paediatric ICU due to multiple episodes of Torsade de Pointes which were treated by implantable cardiac defibrillator. Her medical history was significant for Jervell and Lange-Nielsen syndrome, implantable cardiac defibrillator, shocks, hypochlorhydria, hypergastrinaemia, bilateral sensorineural deafness and iron deficiency anaemia. Seven days prior to this admission, her treatment was changed from oral propranolol syrup 30mg every 8h (1.2 mg/kg/8h) to propranolol tablets at the same dosage. On admission, her baseline ECG revealed left anterior fascicular block, sinus rhythm at 78 beats/min and QTc interval of 604ms. Biochemical studies revealed no metabolic alterations. During the first 4h in the paediatric ICU, she presented 17 new episodes of Torsade de Pointes requiring orotracheal intubation. It was reported that her propranolol syrup contained 25% citric acid which likely resulted in greater bioavailability despite hypochlorhydria. Thus, switching to tablet formulation might have led to decreased bioavailability which likely contributed to arrhythmic storm. The girl was treated with esmolol perfusion, sedation and rapid overdrive pacing resulting in control of her cardiac electrical storm. However, due to ongoing episode, she underwent successful T1–T5 sympathectomy and partial ablation of the stellate ganglion on admission day 3. Esmolol was changed to propranolol 0.3 mg/kg/day with favourable outcome. She was extubated on admission day 6 and discharged home after 21 days of admission. Thereafter, she received oral propranolol syrup 60mg every 8h. During 17 months of follow-up, she experienced no further arrhythmic episode. Ramos-Jimenez J, et al. Propranolol syrup to tablets change triggers electrical storm in Jervell-Lange-Nielsen syndrome. Cardiology in the Young : 13 Oct 2020. Available 803515216 from: URL: http://doi.org/10.1017/S1047951120003285
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Reactions 14 Nov 2020 No. 1830
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