It is characterised by fatty and fibrofatty tissue infiltrating predominantly the right ventricle.
It may be inherited in an autosomal dominant fashion with variable penetrance.
Presentation may be:
- sudden death
ECG may be normal, or:
- T wave inversion V1-V3
- epsilon wave = terminal notch in QRS
The investigation of choice is MRI.
An MRCP-gem to remember is Naxos disease, which is the association of arrhythmogenic right ventricular cardiomyopathy with palmoplantar keratosis and wooley hair.
Now for some pulmonary hypertension...