*The hallmark of restrictive cardiomyopathy: abnormal diastolic function by impeding ventricular filling
→ limit cardiac output and raise filling pressure →impaired systolic function
→ Clinical features: exercise intolerance, dyspnea and elevated systemic venous pressure
*Laboratory examination
-ECG: low-voltage, unspecific ST-T wave changes, arrhythmias.
-X-ray: no pericardial calcification
-Echocardiography: symmetrically thickened LV walls and normal or slightly reduced ventricular volumes and systolic function
Doppler echocardiography: diastolic dysfunction
*Differentiation from: constrictive pericarditis:
RV transvenous endomyocardial biopsy: myocardial infiltration or fibrosis
CTI or CMRI: no thickened pericardium
*Treatment: depends on cause, but is usually disappointed except for hemochromatosis and Fabry’s disease
Hemochromatosis: continuous subcutaneous administration of deferoxamine to reduce body iron stores

In hemochromatosis iron is deposited in the liver cells (hepatocytes), whereas in hemosiderosis iron is deposited in another cell type.

hemosiderosis / hemochromatosis:
iron deposition due to abnormal metabolism or iron overload

1’ abnormal iron metabolism
2’ alcoholic cirrhosis

* multiple blood transfusions (e.g., Hb SS, thalassemia)

Findings:

* osteoporosis
* chondrocalcinosis (CPPD)
* arthropathy
- resembles CPPD arthropathy
- distinctive feature: BEAK-like osteophytes at MCP jts
* mottled increased density of liver + spleen

* Definitions:
- hemosiderosis: hemosiderin deposition within cells
- hemochromatosis: hemosiderin within cells AND interstitium