7th March 2024

Pathology

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is perhaps the simplest of the cardiomyopathies to describe as it has one common pathology – there is progressive replacement of myocardial tissues with adipose tissue, typically in the right ventricular inflow, outflow and apex leading to the name.  This leads to a particular morphology with dilation and aneurysm of the right ventricle, best seen on echo or particularly MRI, but with characteristic changes also seen on ECG.

It can also occur in the left ventricle, where a DCM phenotype is more commonly seen.

It is exclusively genetic, largely caused by autosomal dominant genes encoding proteins in the cardiomyocyte desmosome, although it is also seen in some autosomal recessive conditions such as Naxos and Carvajal syndromes.