Cardiovascular · UKMLA & AKT
Arrhythmogenic right ventricular cardiomyopathy
A free high-yield preview for the UKMLA Applied Knowledge Test. Below are the key points to recognise arrhythmogenic right ventricular cardiomyopathy — the full SA Note notes add investigations, management, complications and 10 practice questions.
Key high-yield points
- Genetic desmosomal cardiomyopathy causing fibro-fatty replacement of the RV free wall → re-entrant VT/VF
- Leading cause of sudden cardiac death in young people and athletes; affects ~1 in 1,000-5,000
- Most common mutation: PKP2 (plakophilin-2); other genes: DSP, DSG2, DSC2, JUP (homozygous JUP → Naxos disease: ARVC + woolly hair + palmoplantar keratoderma)
- Inheritance: autosomal dominant with reduced, age-related penetrance
- Triangle of dysplasia - RV inflow tract, outflow tract, and apex are preferentially affected
Exercise is not just an arrhythmia trigger - it actively drives structural progression by amplifying desmosomal disruption. Sports restriction is a treatment, not just a precaution.
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