restrictive cardiomyopathy


Also found in: Dictionary, Thesaurus, Acronyms, Encyclopedia, Wikipedia.

Restrictive Cardiomyopathy

 

Definition

Cardiomyopathy is an ongoing disease process that damages the muscle wall of the lower chambers of the heart. Restrictive cardiomyopathy is a form of cardiomyopathy in which the walls of the heart become rigid.

Description

Restrictive cardiomyopathy is the least common type of cardiomyopathy in the United States. The stiffened heart walls cannot stretch properly to allow enough blood to fill the ventricles between heartbeats. As the stiffening worsens, heart failure occurs. The blood backs up into the blood vessels, causing fluid buildup in tissues (congestion and edema).

Causes and symptoms

Restrictive cardiomyopathy can be caused by a number of diseases. Often, the cause is unknown. The rigidity of the heart walls may be caused by fibrosis, the replacement of muscle cells with tough, fibrous tissue. In some disorders, proteins and other substances are deposited in the heart wall. Amyloidosis is the accumulation of a protein material, called amyloid, in the tissue of the heart wall and other organs. In hemochromatosis, there is too much iron in the body and some of the excess iron can build up in the heart. Sarcoidosis causes the formation of many small lesions, called granulomas, in the heart wall and other tissues of the body. These granulomas contain inflammatory white blood cells and other cells that decrease the flexibility of the heart.
People with restrictive cardiomyopathy usually feel tired and weak, and have shortness of breath, especially during exercise. If blood is backing up in the circulation they may also experience edema (large amounts of fluid in tissues) of the legs and feet.

Diagnosis

The diagnosis is usually based on a physical examination, echocardiography, and other tests as needed. The physician listens to the heart with a stethoscope to detect abnormal heart rhythms and heart sounds.
Echocardiography uses sound waves to make images of the heart. These images provide information about the structures of the heart and its heart valves. Echocardiography can also be used to find out how much blood the heart is pumping. It determines the amount of blood in the ventricle, called the ventricular volume, and the amount of blood the ventricle pumps each time it beats, called the ejection fraction. A healthy heart pumps at least one half the amount of blood in the left ventricle with each heartbeat.
Computed tomography scan (CT scan) and magnetic resonance imaging (MRI) are imaging tests that can also provide information about the structure of the heart. However, these tests are rarely needed for diagnosis.
Cardiac catheterization may be needed to confirm a diagnosis or cause. In cardiac catheterization, a small tube called a catheter is inserted into an artery and passed into the heart. It is used to measure pressure in the heart and the amount of blood pumped by the heart. A small tissue sample (biopsy) of the heart muscle can be removed through the catheter for microscopic examination. Fibrous tissue or deposits in the heart muscle can be identified in this biopsy.

Treatment

There is no effective treatment for restrictive cardiomyopathy. Treatment of a causative disease may reduce or stop the damage to the heart, but existing damage cannot be reversed. Medications may be used to lessen the workload on the heart and to control the heart rhythm. Drugs normally used to treat other types of cardiomyopathy and heart failure may cause problems for patients with restrictive cardiomyopathy. For example, medicines that reduce the heart's workload may lower blood pressure too much.
A heart transplant may be necessary for patients who develop severe heart failure.

Key terms

Amyloidosis — Build up of amyloid, a protein substance, in tissues of the body, including the heart.
Cardiac catheterization — A diagnostic test for evaluating heart disease; a catheter is inserted into an artery and passed into the heart.
Edema — Swelling caused by fluid buildup in tissues.
Fibrosis — Replacement of normal tissue with tough, fibrous tissue.
Hemochromatosis — A disease in which there is too much iron in the body; iron deposits can build up in the heart muscle and other tissues.
Sarcoidosis — A chronic disease causing the formation of many small lesions called granulomas in the heart wall and other tissues of the body.

Prognosis

The prognosis for patients with restrictive cardiomyopathy is poor. If the disease process causing the problem can be treated, the damage to the heart muscle may be stopped. Also, medicines may relieve symptoms. However, for most patients, restrictive cardiomyopathy eventually causes heart failure. A heart transplant may be necessary when heart failure becomes too severe to treat with medicines.

Prevention

Obtaining early treatment for diseases that might cause restrictive cardiomyopathy might prevent or slow the development of heart wall stiffness. Anyone experiencing symptoms of shortness of breath, tiredness, and weakness should see a physician.

Resources

Organizations

American Heart Association. 7320 Greenville Ave. Dallas, TX 75231. (214) 373-6300. http://www.americanheart.org.
National Heart, Lung and Blood Institute. P.O. Box 30105, Bethesda, MD 20824-0105. (301) 251-1222. http://www.nhlbi.nih.gov.
Texas Heart Institute. Heart Information Service. P.O. Box 20345, Houston, TX 77225-0345. http://www.tmc.edu/thi.
Gale Encyclopedia of Medicine. Copyright 2008 The Gale Group, Inc. All rights reserved.

cardiomyopathy

 [kahr″de-o-mi-op´ah-the]
a general diagnostic term designating primary myocardial disease.
alcoholic cardiomyopathy a congestive cardiomyopathy resulting in cardiac enlargement and low cardiac output occurring in chronic alcoholics; the heart disease in beriberi (thiamine deficiency) is also associated with alcoholism.
congestive cardiomyopathy a syndrome characterized by cardiac enlargement, especially of the left ventricle, myocardial dysfunction, and congestive heart failure.
hypertrophic cardiomyopathy an increase in heart muscle weight, particularly of the left ventricle and often involving the interventricular septum; it may affect the flow of blood from an atrium into the ventricle or out from the ventricle. This type of cardiomyopathy is frequently associated with idiopathic hypertrophic subaortic stenosis. Called also asymmetrical septal hypertrophy.
hypertrophic obstructive cardiomyopathy a form of hypertrophic cardiomyopathy in which the location of the septal hypertrophy causes obstructive interference to left ventricular outflow. See also asymmetrical septal hypertrophy.
infiltrative cardiomyopathy myocardial disease resulting from deposition in the heart tissue of abnormal substances, as may occur in amyloidosis, hemochromatosis, and other disorders.
primary cardiomyopathy that in which the basic pathologic process involves the myocardium itself and not other cardiac structures; the condition is of unknown etiology and not part of a disease affecting other organs.
restrictive cardiomyopathy a form in which the ventricular walls are excessively rigid, impeding ventricular filling; it is marked by abnormal diastolic function but normal or nearly normal systolic function.
secondary cardiomyopathy any form that is due to another cardiovascular disorder or is a manifestation of a systemic disease such as sarcoidosis.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

re·stric·tive car·di·o·my·op·a·thy

a diverse group of conditions characterized by restriction of diastolic filling; often confused with constrictive pericarditis and the infiltrative cardiomyopathies; left ventricular size and systolic function may be preserved but dyspnea results primarily from increased left ventricular diastolic pressure; signs of right ventricular failure may be prominent.
Farlex Partner Medical Dictionary © Farlex 2012

restrictive cardiomyopathy

Infiltrative cardiomyopathy A group of myocardial disorders characterized by an inability of the ventricles to fill and pump blood efficiently–the least common cardiomyopathy in the US Etiology Myocardial and endomyocardial diseases Clinical If left and right atrial are chronic, systemic and pulmonary HTN→congestion and/or edema; Sx of right-sided congestion–↑ jugular venous pressure, dependent edema, liver congestion or ascites are common. Cf Hypertrophic cardiomyopathy.
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.
References in periodicals archive ?
Genetic linkage of a novel autosomal dominant restrictive cardiomyopathy locus.
Clinical spectrum of restrictive cardiomyopathy in children.
Ren et al., "Regional left atrial function differentiation in patients with constrictive pericarditis and restrictive cardiomyopathy: a study using speckle tracking echocardiography," The International Journal of Cardiovascular Imaging, vol.
Sorajja, "Invasive hemodynamics of constrictive pericarditis, restrictive cardiomyopathy, and cardiac tamponade," Cardiology Clinics, vol.
Endomyocardial fibrosis is an idiopathic disorder characterized by patchy fibrosis of the endocardial surface of the heart, resulting in restrictive cardiomyopathy. It is considered part of a spectrum of single-disease processes that includes Loeffler endocarditits (nontropical eosinophilic endomyocardial fibrosis or fibroplastic parietal endocarditis with eosinophilia).
Type Subtypes Cardiomyopathies Hypertrophic cardiomyopathy Dilated cardiomyopathy Restrictive cardiomyopathy Arrhythmogenic right-ventricular cardiomyopathy Unclassified Specific Ischemic cardiomyopathy cardiomyopathies Valvular cardiomyopathy Hypertensive cardiomyopathy Inflammatory cardiomyopathy Myocarditis Idiopathic Infective Autoimmune Metabolic cardiomyopathy Endocrine Familial storage disease and infiltrations Deficiency Amyloidosis General system disease Connective tissue disorders Infiltrations and granulomas Muscular dystrophies Neuromuscular disorders Sensitivity and toxic reactions Peripartal cardiomyopathy Table 2.
Restrictive Cardiomyopathy with overlapping Constrictive Pericarditis is a rarely accounted clinical condition and only isolated case reports have been cited.3 A number of studies using different techniques have been attempted to distinguish between the two conditions.1 However, no technique is totally reliable and some patients may have mixed features of both conditions.
Restrictive, obliterative and infiltrative cardiomyopathy: restrictive cardiomyopathy. Hurst Heart.
Restrictive Cardiomyopathy. In this disease, the heart becomes constricted in its pumping ability due to loss of elasticity, which makes the heart function in a stiff manner.
* Restrictive cardiomyopathy, which accounts for approximately 10 percent of the primary heart muscle diseases diagnosed in Dr.
A spokesman for Glasgow's Yorkhill Hospital confirmed that Shelly's condition - restrictive cardiomyopathy - is extremely rare.