Advanced arrhythmias and channelopathies - arrhythmogenic right ventricular cardiomyopathy
Description
- Genetic desmosomal cardiomyopathy - progressive fibrofatty replacement of myocardium, arrhythmia out of proportion to ventricular dysfunction
- Terminology has broadened: "arrhythmogenic cardiomyopathy" (ACM), because the left ventricle is involved in the majority
- Right-dominant (classic ARVC) | Biventricular (commonest) | Left-dominant (ALVC) - often misdiagnosed as myocarditis or DCM
- Replacement progresses epicardium -> endocardium (the reverse of ischaemic scar)
- Hence the value of epicardial mapping and ablation
- "Triangle of dysplasia" - RV inflow, outflow and apex (modern imaging shows basal RV and subtricuspid region, not apex, are the earliest)
- A leading cause of sudden cardiac death in the young and in athletes
6 more sections, plus exam facts
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