Catecholaminergic polymorphic ventricular tachycardia managed as orthostatic dysregulation and epilepsy in 11- and 15-year-old sisters

Pediatrics International : Official Journal of the Japan Pediatric Society
Shuhei FujitaKiyoshi Hatasaki

Abstract

In pediatric patients, syncope commonly occurs as vasovagal syncope, or in epilepsy or orthostatic dysregulation. Cardiogenic syncope is rare but it is lethal, and needs to be promptly diagnosed and treated. We describe the cases of 11- and 15-year-old sisters with frequent syncope during exercise and emotional stress since the age of 10 and 12, respectively. There were no abnormalities on 12-lead electrocardiogram (ECG) at rest. They were first diagnosed with orthostatic dysregulation and epilepsy. Because of recurrent exercise-induced syncope, cardiac examinations were performed. On treadmill exercise stress test, bidirectional ventricular tachycardia was induced in the 11-year-old girl, which degenerated into ventricular fibrillation; frequent polymorphic premature ventricular contractions were induced in her elder sister. They were diagnosed with catecholaminergic polymorphic ventricular tachycardia (CPVT) and started on oral beta-blockers and exercise restriction. It is important to suspect CPVT in pediatric exercise-induced syncope, and to recognize that CPVT does not show ECG abnormalities at rest.

References

Aug 4, 2004·The Journal of Pediatrics·Martial M MassinPaul Gérard
Apr 19, 2013·Circulation Journal : Official Journal of the Japanese Circulation Society·Mihoko KawamuraMinoru Horie
Sep 3, 2013·The Journal of Pediatrics·Justin T Tretter, Rae-Ellen W Kavey
Aug 19, 2014·International Journal of Cardiology·Jennifer KozlovskiChristopher Semsarian
Jan 19, 2016·European Journal of Pediatrics·Alban-Elouen BaruteauAnne M Dubin
Aug 10, 2016·The Journal of Pediatrics·Aarti DalalRobert Campbell
Oct 21, 2016·Journal of Arrhythmia·Naokata Sumitomo

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