Restrictive cardiomyopathy

Restrictive cardiomyopathy
Synonyms	Obliterative cardiomyopathy, infiltrative cardiomyopathy, constrictive cardiomyopathy
Micrograph of cardiac amyloidosis, a cause of restrictive cardiomyopathy. Congo red stain.
Classification and external resources
Specialty	cardiology
ICD-10	I42.5
ICD-9-CM	425.4
DiseasesDB	11390
MedlinePlus	000189
eMedicine	med/291
MeSH	D002313
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Restrictive cardiomyopathy (RCM) is a form of cardiomyopathy in which the walls are rigid, and the heart is restricted from stretching and filling with blood properly. It is the least common of John Forrest Goodwin's three original subtypes of cardiomyopathy, which includes hypertrophic and dilated as well as restrictive.

It should not be confused with constrictive pericarditis, a disease which presents similarly but is very different in treatment and prognosis.

Rhythmicity and contractility of the heart may be normal, but the stiff walls of the heart chambers (atria and ventricles) keep them from adequately filling, reducing preload and end-diastolic volume.

Thus, blood flow is reduced, and blood volume that would normally enter the heart is backed up in the circulatory system. In time, restrictive cardiomyopathy patients develop diastolic dysfunction and eventually heart failure.

Untreated hearts with RCM often develop the following characteristics: Biatrial enlargement, thickened LV walls (with normal chamber size), thickened RV free wall (with normal chamber size), elevated right atrial pressure (>12mmHg), moderate pulmonary hypertension, normal systolic function, poor diastolic function, typically Grade III - IV Diastolic heart failure.

It is possible to divide the causes into primary and secondary.

Other causes include scleroderma, eosinophilic granulomatosis with polyangiitis, cystinosis, lymphoma, Gaucher's disease, Fabry's disease, pseudoxanthoma elasticum, hypereosinophilic syndrome, carcinoid, Noonan's syndrome, reactive arthritis, and Werner's syndrome.

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