Specialty cardiology ICD-9-CM 425.4 MedlinePlus 000189 | ICD-10 I42.5 DiseasesDB 11390 | |
 | ||
Synonyms Obliterative cardiomyopathy, infiltrative cardiomyopathy, constrictive cardiomyopathy | ||
Restrictive cardiomyopathy (RCM) 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 of John Forrest Goodwin's three original subtypes of cardiomyopathy, which includes hypertrophic and dilated as well as restrictive.
Contents
It should not be confused with constrictive pericarditis, a disease which presents similarly but is very different in treatment and prognosis.
Signs and symptoms
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
It is possible to divide the causes into primary and secondary.
Primary
Secondary
Other causes include scleroderma, eosinophilic granulomatosis with polyangiitis, cystinosis, lymphoma, Gaucher's disease, Fabry's disease, pseudoxanthoma elasticum, hypereosinophilic syndrome, carcinoid, Noonan's syndrome, reactive arthritis, and Werner's syndrome.
Treatment
Treatment of restrictive cardiomyopathy should focus on management of causative conditions (for example, using corticosteroids if the cause is sarcoidosis), and slowing the progression of cardiomyopathy. Diuretics, vasodilators, angiotensin-converting enzyme inhibitors, and anticoagulation may be indicated for managing restrictive cardiomyopathy.
Calcium channel blockers are generally contraindicated due to their negative inotropic effect, particularly in cardiomyopathy caused by amyloidosis. Digoxin, calcium channel blocking drugs and beta-adrenergic blocking agents provide little benefit, except in the subgroup of restrictive cardiomyopathy with atrial fibrillation.
Heart failure resulting from restrictive cardiomyopathy will usually eventually have to be treated by cardiac transplantation or left ventricular assist device.