Restrictive cardiomyopathy

Restrictive cardiomyopathy (RCM) (a.k.a Obliterative cardiomyopathy) 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 cardiomyopathy.

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.

Primary

 * Löffler's endocarditis
 * endocardial fibroelastosis

Secondary

 * infiltrative
 * cardiac amyloidosis
 * haemochromatosis
 * sarcoidosis


 * 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.

Treatment
Therapy for restrictive cardiomyopathy is limited.

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.