Wednesday, 17 November 2010

MRCP revision battle 52.5: Arrhythmogenic right ventricular cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy is the second most common cause of sudden cardiac death in young people after HCM.

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:
  • palpitations
  • syncope
  • sudden death

ECG may be normal, or:
  • T wave inversion V1-V3
  • epsilon wave = terminal notch in QRS

The investigation of choice is MRI.

Treatment includes:
  • sotolol
  • ablation
  • ICD

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...