Restrictive cardiomyopathy (RCM) (a.k.a Obliterative cardiomyopathy, once known as "constrictive cardiomyopathy") is a form of cardiomyopathy

Cardiomyopathy

Cardiomyopathy, which literally means "heart muscle disease," is the deterioration of the function of the myocardium for any reason. People with cardiomyopathy are often at risk of arrhythmia or sudden cardiac death or both. Cardiomyopathy can often go undetected, making it especially dangerous to...

 in which the walls are rigid, 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

Hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy is a disease of the myocardium in which a portion of the myocardium is hypertrophied without any obvious cause...

 and dilated

Dilated cardiomyopathy

Dilated cardiomyopathy or DCM is a condition in which the heart becomes weakened and enlarged and cannot pump blood efficiently. The decreased heart function can affect the lungs, liver, and other body systems....

 as well as restrictive.

It should not be confused with constrictive pericarditis

Constrictive pericarditis

In many cases, constrictive pericarditis is a late sequela, in other words a condition that is the consequence of a previous disease, of an inflammatory condition of the pericardium...

, a disease which presents similarly but is very different in treatment and prognosis.

Presentation

Rhythmicity and contractility

Contractility

Myocardial contractility is the intrinsic ability of the heart to contract independent of preload and afterload. Changes in the ability to produce force during contraction result from different degrees of binding between myosin and actin filaments...

 of the heart may be normal, but the stiff walls of the heart chambers (atria

Atria

Atria may refer to:*Atrium , an anatomical structure of the heart*Atrium , a large open space within a building*Atria or Alpha Trianguli Australis, a star in the constellation Triangulum Australe...

 and ventricles

Ventricle (heart)

In the heart, a ventricle is one of two large chambers that collect and expel blood received from an atrium towards the peripheral beds within the body and lungs. The Atria primes the Pump...

) keep them from adequately filling, reducing preload and end-diastolic volume

End-diastolic volume

In cardiovascular physiology, end-diastolic volume is the volume of blood in the right and/or left ventricle at end Load or filling in . Because greater EDVs cause greater distention of the ventricle, EDV is often used synonymously with preload, which refers to the length of the sarcomeres in...

.

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.

Secondary

• infiltrative
  • cardiac amyloidosis
    Cardiac amyloidosis
    Cardiac amyloidosis may refer to:* Cardiac manifestations of AL amyloidosis* Cardiac manifestations of transthyretin-related hereditary amyloidosis* Isolated atrial amyloidosis...
    
    
  • haemochromatosis
    Haemochromatosis
    Haemochromatosis type 1 is a hereditary disease characterized by excessive intestinal absorption of dietary iron resulting in a pathological increase in total body iron stores. Humans, like most animals, have no means to excrete excess iron...
    
    
  • sarcoidosis
    Sarcoidosis
    Sarcoidosis , also called sarcoid, Besnier-Boeck disease or Besnier-Boeck-Schaumann disease, is a disease in which abnormal collections of chronic inflammatory cells form as nodules in multiple organs. The cause of sarcoidosis is unknown...
    
    

• interstitial
  • postradiation fibrosis

Other causes include scleroderma

Scleroderma

Systemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma.-Skin symptoms:...

, Churg-Strauss syndrome

Churg-Strauss syndrome

Churg–Strauss syndrome is a medium and small vessel autoimmune vasculitis, leading to necrosis. It involves mainly the blood vessels of the lungs , gastrointestinal system, and peripheral nerves, but also affects the heart, skin and kidneys. It is a rare disease that is non-inheritable and...

, cystinosis

Cystinosis

Cystinosis is a lysosomal storage disease characterized by the abnormal accumulation of the amino acid cystine. It is a genetic disorder that typically follows an autosomal recessive inheritance pattern. Cystinosis is the most common cause of Fanconi syndrome in the pediatric age group...

, lymphoma

Lymphoma

Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...

, Gaucher's disease

Gaucher's disease

Gaucher's disease is a genetic disease in which a fatty substance accumulates in cells and certain organs.Gaucher's disease is the most common of the lysosomal storage diseases. It is caused by a hereditary deficiency of the enzyme glucosylceramidase. The enzyme acts on the fatty acid...

, hemochromatosis, Fabry's disease

Fabry's disease

Fabry disease is a rare X-linked recessive lysosomal storage disease, which can cause a wide range of systemic symptoms...

, pseudoxanthoma elasticum

Pseudoxanthoma elasticum

Pseudoxanthoma elasticum , also known as Grönblad–Strandberg syndrome, is a genetic disease that causes fragmentation and mineralization of elastic fibers in some tissues. The most common problems arise in the skin and eyes, and later in blood vessels in the form of premature atherosclerosis...

, hypereosinophilic syndrome

Hypereosinophilic syndrome

The hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow.HES is a diagnosis of exclusion, after clonal...

, carcinoid

Carcinoid

Carcinoid is a slow-growing type of neuroendocrine tumor, originating in the cells of the neuroendocrine system.In 2000, the World Health Organization redefined "carcinoid", but this new definition has not been accepted by all practitioners. This has led to some complexity in distinguishing...

, Noonan's syndrome, reactive arthritis

Reactive arthritis

Reactive arthritis , is classified as an autoimmune condition that develops in response to an infection in another part of the body. Coming into contact with bacteria and developing an infection can trigger the disease. Reiter's syndrome has symptoms similar to various other conditions collectively...

, 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.

External links

• Overview at Merck Manual