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Catecholaminergic Polymorphic VT — EECC MCQ

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HardArrhythmia & ElectrophysiologyCatecholaminergic Polymorphic VTEECC

A 38-year-old man with catecholaminergic polymorphic ventricular tachycardia (CPVT) confirmed by RYR2 mutation is reviewed. He had a syncopal episode during exercise 6 months ago. Holter monitoring during exercise showed bidirectional VT at heart rate 130 bpm. He is currently on nadolol 80 mg daily (maximum tolerated dose). Despite this, he had another episode of exercise-induced syncope last week. What is the most appropriate next step?

Educational content. Not a substitute for clinical judgement or local policy.

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Correct answer: EAdd flecainide and reassess

Explanation lettering: E = shown as A · A = shown as D · D = shown as E

Per the 2022 ESC VA/SCD Guidelines, in CPVT patients with recurrent syncope or VT despite maximally tolerated beta-blocker, addition of flecainide should be considered (Class IIa). Flecainide has a specific anti-arrhythmic effect in CPVT by blocking RyR2-mediated calcium release. Switching beta-blocker brand (A) is not the appropriate escalation. An ICD (C) may be considered but medical therapy should be optimised first. Verapamil (D) is not standard in CPVT. LCSD (E) may be considered if flecainide fails.

Reference: ESC (2022): Guidelines for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death