Cardiomyopathy, Restrictive Cardiomyopathy, RCM

    • LV small
      • may be LVH
    • LA large
    • decrease in the ventricular filling
    • EF maintained
Compared to Constrictive Disease
    • contours of the ventricular cavities are
      maintained (do not appear indented- like in constriction)
    • The degree of increase in atrial size is much greater in the RCM than in constrictive pericarditis (CP)
    • myocardial thickness
      is frequently increased in the RCM but normal in the CP
    • the pericardial thickness is usually normal, having a normal value
      of 2 mm or less



Restrictive cardiomyopathy,  is defined as abnormal diastolic function in association with relatively well-preserved systolic function (at least in the early stages of the disease).

heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, arterial systemic hypertension, valvular diseaseor congenital heart disease sufficient to cause the observed myocardial abnormality.1″


Functionally hemodynamic picture strongly resembles constrictive pericarditis.

Precipitous rise of LV pressure with only small increases in volume. On cardiac catheterization, this phenomenon is characterized by a dip-and-plateau contour of early diastolic pressure traces.

RCM are usually characterized by normal or small LV cavity size (<40 mL/m2)





Iron Overload

Hypertrophic Cardiomyopathy

Chloroquin Induced Cardiomyopathy


infiltrative or storage diseases (e.g. amyloidosis and glycogen storage disease);

obliterative or endomyocardial diseases [e.g. endomyocardial fibrosis (EMF), related or not to hypereosinophilia].

Cause Familial/ genetic Non-familial/ non-genetic
Apparently Idiopathic
 Genetic origin X
 Unknown origin X
 AL/prealbumin X
 Genetic (e.g. TTR) X
 Senile X
Other infiltrative diseases (such as Gaucher’s disease, Hurler’s disease) X
Inflammatory cardiomyopathies with a restrictive haemodynamic component: Sarcoidosis, SSc X
Storage diseases
 Haemochromatosis X
 Fabry disease X
 Glycogen storage disease X
Pseudoxanthoma elasticum X
Radiation therapy X
Drugs X
Endomyocardial diseases (with or without hypereosinophilia, carcinoid disease, drug induced) X (rare) X (frequent)
Miscellaneous (radiation, drug-induced, e.g. antracycline toxicity, serotonin, methysergide, ergotamine, mercurial agents, and busulfan) X

RCM, restrictive cardiomyopathy; TTR, transthyretin; SSc, systemic sclerosis.

References and Links

Zuluaga, A et al Restrictive Cardiomyopathies:Evaluation Using Cardiac MRI and Multidetector CT