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CPVT Diagnosis — EECC MCQ

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ModerateArrhythmia & ElectrophysiologyCPVT DiagnosisEECC

A 55-year-old man with structurally normal heart presents with recurrent palpitations. Holter reveals frequent episodes of non-sustained polymorphic VT occurring during exercise. His resting ECG is normal and baseline QTc is 380 ms. Genetic testing reveals an RYR2 mutation. What is the diagnosis?

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Correct answer: C — Catecholaminergic polymorphic ventricular tachycardia (CPVT) — triggered by adrenergic stimulation during exercise or emotional stress, with characteristic bidirectional or polymorphic VT on a normal resting ECG

CPVT is a rare inherited channelopathy caused by mutations in genes regulating intracellular calcium handling (RYR2 in autosomal dominant form, CASQ2 in autosomal recessive). Characteristic features: (1) normal resting ECG (no QT prolongation, no Brugada pattern); (2) adrenergically-triggered arrhythmias — progressive with exercise: isolated PVCs → bigeminy → bidirectional VT (alternating QRS axis beat-to-beat) → polymorphic VT → VF; (3) onset in childhood/adolescence; (4) structurally normal heart. The 2022 ESC VA/SCD Guidelines recommend: (1) beta-blocker therapy (nadolol preferred — Class I); (2) flecainide as add-on for breakthrough arrhythmias on beta-blocker (Class IIa); (3) ICD for cardiac arrest survivors; (4) avoidance of competitive sport and strenuous exercise. Exercise testing is both diagnostic and used for monitoring.

Reference: ESC (2022): Guidelines on VA and SCD Prevention