| Restrictive cardiomyopathy (a.k.a Obliterative cardiomyopathy) | |
|---|---|
| Classification and external resources | |
Micrograph of cardiac amyloidosis, a cause of restrictive cardiomyopathy. Congo red stain. |
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| ICD-10 | I42.5 |
| ICD-9 | 425.4 |
| DiseasesDB | 11390 |
| MedlinePlus | 000189 |
| eMedicine | med/291 |
| MeSH | D002313 |
Restrictive cardiomyopathy (RCM) (a.k.a Obliterative cardiomyopathy, once known as "constrictive cardiomyopathy"[1]) is a form of cardiomyopathy in which the walls are rigid,[2] and the heart is restricted from stretching and filling with blood properly.
It is the least common of Goodwin's three original subtypes of cardiomyopathy, which includes hypertrophic and dilated as well as restrictive.[1]
It should not be confused with constrictive pericarditis, a disease which presents similarly but is very different in treatment and prognosis.[1]
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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.
So blood flow is reduced, and blood 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.
It is possible to divide the causes into primary and secondary.[3]
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.[4]
Therapy for restrictive cardiomyopathy is limited.[5]
Diuretics may help relieve symptoms. Calcium Channel Blockers may improve diastolic function in selected individuals.
Heart failure resulting from restrictive cardiomyopathy will usually eventually have to be treated by cardiac transplantation.
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