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

Echocardiography

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

References

1. Belloni E, De cobelli F, Esposito A et-al. MRI of cardiomyopathy. AJR Am J Roentgenol. 2008;191 (6): 1702-10. doi:10.2214/AJR.07.3997 - Pubmed citation
2. Vanden driesen RI, Slaughter RE, Strugnell WE. MR findings in cardiac amyloidosis. AJR Am J Roentgenol. 2006;186 (6): 1682-5. doi:10.2214/AJR.04.0871 - Pubmed citation
3. Boxt LM. Cardiac MR imaging: a guide for the beginner. Radiographics. 19 (4): 1009-25. Radiographics (full text) - Pubmed citation
4. Nihoyannopoulos P, Dawson D. Restrictive cardiomyopathies. Eur J Echocardiogr. 2009;10 (8): iii23-33. doi:10.1093/ejechocard/jep156 - Pubmed citation
5. Cotroneo J, Sleik KM, Rene rodriguez E et-al. Hydroxychloroquine-induced restrictive cardiomyopathy. Eur J Echocardiogr. 2007;8 (4): 247-51. doi:10.1016/j.euje.2006.02.002 - Pubmed citation
6. Masui T, Finck S, Higgins CB. Constrictive pericarditis and restrictive cardiomyopathy: evaluation with MR imaging. Radiology. 1992;182 (2): 369-73. Radiology (abstract) - Pubmed citation
7. Elliott P, Andersson B, Arbustini E et-al. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur. Heart J. 2008;29 (2): 270-6. doi:10.1093/eurheartj/ehm342 - Pubmed citation