Cardiovascular · UKMLA & AKT
Arrhythmogenic right ventricular cardiomyopathy
A free high-yield preview for the UKMLA Applied Knowledge Test, covering how to recognise this condition. The full SA Note notes add investigations, management and complications, with practice questions on this topic.
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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