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Restrictive cardiomyopathy
Classification and external resources

Micrograph of cardiac amyloidosis, a cause of restrictive cardiomyopathy. Congo red stain.
ICD-10 I42.5
ICD-9 425.4
DiseasesDB 11390
MedlinePlus 000189
eMedicine med/291
MeSH D002313

Restrictive cardiomyopathy (RCM) is a form of cardiomyopathy in which the walls are rigid,[1] and the heart is restricted from stretching and filling with blood properly.

It is the least common cardiomyopathy.

Contents

Presentation

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.

Causes

It is possible to divide the causes into primary and secondary.[2]

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Primary

Secondary

  • interstitial
    • postradiation fibrosis

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.[3]

Treatment

Therapy for restrictive cardiomyopathy is limited. [4]

Diuretics may help relieve symptoms.

References

  1. ^ restrictive cardiomyopathy at Dorland's Medical Dictionary
  2. ^ Crawford, Michael H. (2003). Current diagnosis & treatment in cardiology. New York: Lange Medical Books/McGraw-Hill. pp. 188. ISBN 0-8385-1473-1.  
  3. ^ 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.   edit
  4. ^ "Restrictive Cardiomyopathy: Cardiomyopathy: Merck Manual Home Edition". http://www.merck.com/mmhe/sec03/ch026/ch026d.html. Retrieved 2009-01-04.  

External links


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