Restrictive Cardiomyopathy

A restrictive cardiomyopathy is a sub type of cardiomyopathy which is characterised by a marked decrease in ventricular compliance. It is predominantly a disease of diastolic dysfunction where the systolic (contractile) of the myocardium is usually unaffected.

Recognised causes which can give a restrictive type pattern include

* cardiac amyloidosis : thought to be the most common cause outside the tropics 2
* cardiac sarcoidosis
* haemochromatosis 4
* scleroderma 4
* tumour invasion / infiltration / metastases
* hyper eosinophillic (Loffler) syndrome
* drugs : hydroxychloroquine 5, methylsergide 6 , anthracyclines 6
* Anderson-Fabry disease
* Glycogen storage diseases
* idiopathic

Radiographic features

Chest radiograph

The heart size can be normal. Sometimes there may be evidence of biatrial dilatation


Shows diastolic dysfunction

Cardiac MRI

Useful for differentiating between constrictive pericarditis. May show biatrial enlargement with mininal or no ventricular elargement. Cine MRI will show altered diastolic filling.

Differential diagnoses

  • constrictive pericarditis : there can be septal flattening on cine MRI 1 and the pericardium can be thicker than 4mm 1,3
  • endomyocardial fibrosis : ( sometimes classified as a restrictive type )

Article Source : Dr Yuranga Weerakkody, Radiopaedia


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