Restrictive cardiomyopathy involves non-dilated non-hypertrophied ventricles with impaired ventricular filling.
Causes
- Familial non-infiltrative cardiomyopathy (inherited genetic disorders)
- Infiltrative:
- Amyloidosis
- Sarcoidosis
- Gaucher disease
- Hurler syndrome
- Fatty infiltration
- Storage:
- Haemochromatosis
- Fabry disease
- Glycogen storage disorders
- Others:
- Diabetic cardiomyopathy
- Scleroderma
- Hypereosinophilic syndrome (Löffler's)
- Radiation
- Chemotherapy (anthracyclines like Doxorubicin and Daunorubicin)
PC
Presentation is often that of heart failure or sometimes similar to that of constrictive pericarditis. Up to 75% of patients will have associated atrial fibrillation.
Investigations
- Echocardiogram (which shows thickened ventricular walls and valves)
- cardiac MRI (which is useful for distinguishing between restrictive cardiomyopathy and constrictive pericarditis).