Restrictive Cardiomyopathies
From Ask Dr Wiki
Definition
- A group of disorders that affect myocardial function by either primary myocyte dysfunction and/or extracellular infiltration and fibrosis
- Predominant feature is impaired ventricular filling/diastolic dysfunction with preservation of systolic function (early in disease).
- Most are rapidly progressive and irreversible with transplantation being the only treatment option for advanced disease.
Etiology of RCM
Primary
- Loeffler’s
- Idiopathic
- Endomyocardial Fibrosis
Secondary
- Infiltrative
- Sarcoidosis
- Amyloidosis
- Post-Radiation
- Storage Disease
- Fabry’s
- Hemochromatosis
- Glycogen Storage
