Arrhythmogenic Cardiomyopathy Medical Slides

Organize the 6 diagnostic categories as a grid: (1) global/regional dysfunction on imaging, (2) tissue characterization (fibrofatty replacement on biopsy), (3) repolarization abnormalities (T-wave inversions V1-V3), (4) depolarization abnormalities (epsilon waves, terminal activation duration >55 ms), (5) arrhythmias (LBBB-morphology VT, >500 PVCs/24h), (6) family history/genetics. Definite diagnosis = 2 major, or 1 major + 2 minor, or 4 minor from different categories.

Emphasize that competitive and high-intensity exercise accelerates disease progression and increases arrhythmic risk 5-fold in ACM gene carriers. The 2015 ESC guidelines and 2019 HRS expert consensus recommend restricting competitive sports and limiting exercise intensity. Present the mechanistic rationale: repetitive RV volume and pressure overload during exercise accelerates fibrofatty replacement at the desmosomal junction through mechanical stress on weakened cell-cell connections.

Emphasize the evolution from "ARVC" to "ACM" to capture left-dominant and biventricular forms. Left-dominant ACM (often from DSP or FLNC mutations) presents with LV dysfunction, LGE on CMR in a ring-like subepicardial pattern, and VT with RBBB morphology — it can be misdiagnosed as myocarditis or DCM. Include it as a teaching point to avoid anchoring bias on the classic right-dominant phenotype.