Written by Dimitrios Vrachatis, Specialist Cardiologist, PhD, National and Kapodistrian University of Athens.
What is restrictive cardiomyopathy?
This is a form of cardiomyopathy characterised by a stiff, unyielding myocardium that restricts diastolic filling.
The thickness of the myocardial walls and their overall systolic performance remain almost unaffected. The characteristic finding is restrictive physiology: a disproportionate increase in pressure within the heart chambers for a small increase in volume.
How common is it?
Its exact epidemiology is not known, but it is considered the least common cardiomyopathy.
What are the symptoms?
The patient may have no symptoms or may experience shortness of breath, fatigue, leg swelling, weight gain, a feeling of fullness and other symptoms.
What causes it?
Restrictive cardiomyopathy can result from a number of familial conditions, such as Anderson–Fabry disease, or non-familial causes, such as previous radiotherapy, sarcoidosis, endomyocardial fibrosis, carcinoid disease, anthracycline toxicity and scleroderma. Sometimes no cause is found (idiopathic).
In the strict sense, the condition is not characterised as hereditary.
How is it diagnosed?
Restrictive cardiomyopathy diagnosis is not always easy. It must be distinguished from constrictive pericarditis, with which it shares common features. Investigations may include:
- An electrocardiogram.
- An echocardiogram.
- Cardiac MRI.
- A chest X-ray.
- Right heart catheterisation and other tests.