|Restrictive cardiomyopathy (a.k.a. Obliterative cardiomyopathy)|
|Classification and external resources|
Restrictive cardiomyopathy (RCM) (a.k.a. Obliterative cardiomyopathy, once known as "constrictive cardiomyopathy") is a form of cardiomyopathy in which the walls are rigid, and the heart is restricted from stretching and filling with blood properly.
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, Churg-Strauss syndrome, cystinosis, lymphoma, Gaucher's disease, hemochromatosis, Fabry's disease, pseudoxanthoma elasticum, hypereosinophilic syndrome, carcinoid, Noonan's syndrome, reactive arthritis, and Werner's syndrome.
Therapy for restrictive cardiomyopathy is limited.
Heart failure resulting from restrictive cardiomyopathy will usually eventually have to be treated by cardiac transplantation.
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- "restrictive cardiomyopathy" at Dorland's Medical Dictionary
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- Stöllberger, C.; Finsterer, J. (2007). "Extracardiac medical and neuromuscular implications in restrictive cardiomyopathy". Clinical Cardiology 30 (8): 375–380. doi:10.1002/clc.20005. PMID 17680617.
- "Restrictive Cardiomyopathy: Cardiomyopathy: Merck Manual Home Health Handbook". Retrieved 2009-01-04.