Cardiomyopathy - restrictive; Infiltrative cardiomyopathy; Idiopathic myocardial fibrosis
Restrictive cardiomyopathy refers to a set of changes in how the heart muscle functions. These changes cause the heart to fill poorly (more common) or squeeze poorly (less common). Sometimes, both problems are present.
In a case of restrictive cardiomyopathy, the heart muscle is of normal size or slightly enlarged. Most of the time, it also pumps normally. However, it does not relax normally during the time between heartbeats when the blood returns from the body (diastole).
Although the main problem is abnormal filling of the heart, the heart may not pump blood strongly when the disease progresses. The abnormal heart function can affect the lungs, liver, and other body systems. Restrictive cardiomyopathy may affect either or both of the lower heart chambers (ventricles). Restrictive cardiomyopathy is a rare condition. The most common causes are amyloidosis and scarring of the heart from an unknown cause. It also can occur after a heart transplant.
Other causes of restrictive cardiomyopathy include:
Symptoms of heart failure are most common. These symptoms often develop slowly over time. However, symptoms sometimes start very suddenly and are severe.
Common symptoms are:
Other symptoms may include:
A physical exam may show:
Tests for restrictive cardiomyopathy include:
Restrictive cardiomyopathy may appear similar to constrictive pericarditis. Cardiac catheterization may help confirm the diagnosis. Rarely, a biopsy of the heart may be required.
The condition causing the cardiomyopathy is treated when it can be found.
Few treatments are known to work well for restrictive cardiomyopathy. The main goal of treatment is to control symptoms and improve quality of life.
The following treatments may be used to control symptoms or prevent problems:
Additional treatments are also under investigation.
A heart transplant may be considered if the heart function is very poor and symptoms are severe.
People with this condition often develop heart failure that gets worse. Problems with heart rhythm or "leaky" heart valves may also occur.
People with restrictive cardiomyopathy may be heart transplant candidates. The outlook depends on the cause of the condition, but it is usually poor. Survival after diagnosis may exceed 10 years.
Contact your health care provider if you have symptoms of restrictive cardiomyopathy.
Heidenreich PA, Bozkurt B, Aguilar D, et al. 2022 AHA/ACC/HFSA guideline for the management of heartfailure: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2022;79(17):1757-1780. PMID: 35379504 pubmed.ncbi.nlm.nih.gov/35379504/.
Hershberger RE. The dilated, restrictive, and infiltrative cardiomyopathies. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Philadelphia, PA: Elsevier; 2022:chap 52.
McKenna WJ, Elliott PM. Diseases of the myocardium and endocardium. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 54.BACK TO TOP
Review Date: 5/8/2022
Reviewed By: Michael A. Chen, MD, PhD, Associate Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, WA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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