Catecholaminergic polymorphic VT is a rare but important cause of syncope and sudden death in children with structurally normal hearts and normal QT intervals, requiring careful management.
Despite beta-blocker symptom control, sudden death risk persists in CPVT; case reports leave open optimal long-term management.
Syncope in children is primarily related to vagal hyperreactivity, but ventricular tachycardia (VT) way rarely be seen. Catecholaminergic polymorphic VT is a rare entity that can occur in children without heart disease and with a normal QT interval, which may cause syncope and sudden cardiac death. In this report, we describe the clinical features, treatment, and clinical follow-up of three children with syncope associated with physical effort or emotion and catecholaminergic polymorphic VT. Symptoms were controlled with beta-blockers, but one patient died suddenly in the fourth year of follow-up. Despite the rare occurrence, catecholaminergic polymorphic VT is an important cause of syncope and sudden death in children with no identified heart disease and normal QT interval.
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Leite et al. (2001) studied this question.
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