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ARVC Diagnosis — EECC MCQ

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ModerateCardiomyopathyARVC DiagnosisEECC

A 30-year-old man presents with exercise-induced VT originating from the RV. CMR shows fibrofatty replacement of the RV free wall with regional RV wall motion abnormalities. Genetic testing reveals a desmosomal mutation (PKP2). What is the diagnosis?

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Correct answer: B — Arrhythmogenic right ventricular cardiomyopathy (ARVC) — diagnosed using the revised Task Force Criteria incorporating imaging, ECG, arrhythmia, histological, and genetic criteria

ARVC is a genetically determined cardiomyopathy characterised by fibrofatty replacement of the RV myocardium, predisposing to ventricular arrhythmias and SCD, particularly during exercise. The 2010 revised Task Force Criteria use a scoring system across 6 categories: (1) RV structure/function (CMR: regional wall motion abnormalities, RV dilatation); (2) tissue characterisation (fibrofatty replacement on biopsy); (3) ECG depolarisation (epsilon waves, QRS prolongation in V1-V3); (4) ECG repolarisation (T-wave inversion V1-V3); (5) arrhythmias (VT of LBBB morphology from RV origin); (6) family history/genetics (desmosomal mutations: PKP2, DSP, DSG2, DSC2, JUP). The 2023 ESC Cardiomyopathy Guidelines adopt the broader term NDLVC for cases involving the LV.

Reference: ESC (2023): Guidelines on Cardiomyopathies; 2010 Revised Task Force Criteria