Restrictive cardiomyopathy

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Restrictive cardiomyopathy (a.k.a. Obliterative cardiomyopathy)
Classification and external resources
Cardiac amyloidosis very high mag.jpg
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) (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]

Presentation[edit]

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.

Causes[edit]

It is possible to divide the causes into primary and secondary.[3]

Primary[edit]

Secondary[edit]

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]

Treatment[edit]

Therapy for restrictive cardiomyopathy is limited.[5]

Diuretics may help relieve symptoms.[citation needed] 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.

References[edit]

  1. ^ a b c Hancock, EW (September 2001). "Differential diagnosis of restrictive cardiomyopathy and constrictive pericarditis". Heart (British Cardiac Society) 86 (3): 343–9. PMC 1729880. PMID 11514495. Retrieved 15 October 2011. 
  2. ^ "restrictive cardiomyopathy" at Dorland's Medical Dictionary
  3. ^ Crawford, Michael H. (2003). Current diagnosis & treatment in cardiology. New York: Lange Medical Books/McGraw-Hill. p. 188. ISBN 0-8385-1473-1. 
  4. ^ 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
  5. ^ "Restrictive Cardiomyopathy: Cardiomyopathy: Merck Manual Home Health Handbook". Retrieved 2009-01-04. 

External links[edit]