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ARVC with Cardiac Arrest — RACP Adult Medicine MCQ

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EasyCardiologyARVC with Cardiac ArrestRACP Adult Medicine

A 35-year-old man presents after a cardiac arrest during a football match. He was resuscitated with defibrillation (VF on the AED). ECG post-resuscitation shows normal QTc and no Brugada pattern. Echo shows severe RV dilatation with regional wall motion abnormalities and fibrofatty replacement on cardiac MRI. Genetic testing reveals a desmoplakin mutation. What is the most appropriate management?

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Correct answer: E — ICD implantation

Cardiac arrest survivor with RV dilatation, regional wall motion abnormalities, fibrofatty replacement on MRI, and desmosomal gene mutation is arrhythmogenic right ventricular cardiomyopathy (ARVC). Secondary prevention ICD is mandatory (Class I) after cardiac arrest. Competitive sports must be ceased permanently. Beta-blockers are used as adjunctive antiarrhythmic therapy. Cascade screening of first-degree relatives is essential.

Reference: ESC – 2022 – SCD Prevention; 2020 Revised ARVC Task Force Criteria