The restrictive forms are among the least common of the described cardiomyopathies. Restrictive cardiomyopathy is defined as heart muscle disease that results in "restricted" ventricular filling, with normal or decreased diastolic volume of either or both ventricles. Systolic function is usually normal, and ventricular wall thickness may be normal or increased, depending on the underlying cause. The hemodynamic hallmarks include (1) elevated LV and RV end-diastolic pressure, (2) normal LV systolic function (ejection fraction greater than 50 percent), and (3) an abrupt and rapid rise in early-diastolic ventricular pressure following a marked decline at the onset of diastole. The rapid rise and abrupt plateau in the early-diastolic ventricular pressure trace produce a characteristic "square-root sign" or "dip-and-plateau" filling pattern due to increased myocardial stiffness. This pattern is not diagnostic, however, and may be seen in constrictive pericarditis, with which restrictive cardiomyopathy is commonly confused. Differentiation between the two is critical because constrictive pericarditis can be cured surgically. The diagnosis of restrictive cardiomyopathy should be considered in a patient presenting with CHF but no evidence of cardiomegaly or systolic dysfunction. 11
Restrictive cardiomyopathy may result from systemic disorders (I§b!e51-4.), but most cases are idiopathic. The idiopathic form is sometimes familial, with autosomal dominant transmission. There has been no clearly demonstrated predilection for gender or ethnicity.
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