7th March 2024

Pathology

Restrictive cardiomyopathy (RCM) is an umbrella term for pathologies in which there is increased stiffness of the myocardium, leading to increased pressure with only small increases in volume. It is thought to be one of the least common forms of cardiomyopathy.

A number of pathologies can lead to restrictive physiology; Genetic causes include troponin I abnormalities, familial amyloidosis, and Fabry’s disease, while acquired causes can include light chain amyloidosis, drug induced RCM, carcinoid syndrome, anthracycline chemotherapy and radiation. Haemochromatosis can be either genetically inherited through the HFE gene or acquired through repeated blood transfusions, as can sometimes be seen in thalassaemia.

It has previously been taught that systolic function is preserved in patients with these disorders, but contraction is rarely normal in these conditions and abnormalities of systolic function can be demonstrated on myocardial deformation imaging.