Buzz

• • 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

 

Definition

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 disease, or 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)

Types

Amyloidosis

Sarcoidosis

Loefflers

Iron Overload

Hypertrophic Cardiomyopathy

Chloroquin Induced Cardiomyopathy

Basically

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].

References and Links

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

• TCV
  • Cardiomyopathy Map