Learn About Restrictive Cardiomyopathy

What is the definition of Restrictive Cardiomyopathy?

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.

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What are the alternative names for Restrictive Cardiomyopathy?

Cardiomyopathy - restrictive; Infiltrative cardiomyopathy; Idiopathic myocardial fibrosis

What are the causes of Restrictive Cardiomyopathy?

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:

  • Cardiac amyloidosis
  • Carcinoid heart disease
  • Diseases of the heart lining (endocardium), such as endomyocardial fibrosis and Loeffler syndrome (rare)
  • Iron overload (hemochromatosis)
  • Sarcoidosis
  • Scarring after radiation or chemotherapy
  • Scleroderma
  • Tumors of the heart
What are the symptoms of Restrictive Cardiomyopathy?

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:

  • Cough
  • Breathing problems that occur at night, with activity or when lying flat
  • Fatigue and inability to exercise
  • Loss of appetite
  • Swelling of the abdomen
  • Swelling of the feet and ankles
  • Uneven or rapid pulse

Other symptoms may include:

  • Chest pain
  • Inability to concentrate
  • Low urine output
  • Need to urinate at night (in adults)
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What are the current treatments for Restrictive Cardiomyopathy?

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:

  • Blood thinning medicines
  • Chemotherapy (in some situations)
  • Diuretics to remove fluid and help improve breathing
  • Medicines to prevent or control abnormal heart rhythms
Other treatments that may be used depending on the specific type of cardiomyopathy include:
  • Chemotherapeutic agents
  • Medicines such as tafamidis that target abnormal proteins (amyloid fibrils)
  • Liver transplantation

Additional treatments are also under investigation.

A heart transplant may be considered if the heart function is very poor and symptoms are severe.

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What is the outlook (prognosis) for Restrictive Cardiomyopathy?

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.

When should I contact a medical professional for Restrictive Cardiomyopathy?

Contact your health care provider if you have symptoms of restrictive cardiomyopathy.

Heart - section through the middle
Heart - front view
What are the latest Restrictive Cardiomyopathy Clinical Trials?
Pediatric Cardiomyopathy Mutation Analysis

Summary: The goal of this protocol is to obtain information from individuals with cardiomyopathy and from their families in order to elucidate the molecular genetics of this disorder. This will provide the basis for future genetic counseling as well as contribute to elucidating the biology of normal and abnormal cardiac function.

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Prevalence and Significance of ATTR Aortic Valve Amyloidosis in Degenerative Aortic Stenosis

Summary: Cardiac amyloidosis is a restrictive cardiomyopathy with a potentially severe prognosis that can be life-threatening. It is linked in the vast majority of cases to a light chain deposition of immunoglobulin or transthyretin. Although myocardial involvement is predominant, other locations are possible: the atrioventricular conduction system, coronary arteries and valve leaflets. In systematic histo...

What are the Latest Advances for Restrictive Cardiomyopathy?
Surgical Management of The Endomyocardial Fibrosis of Right Ventricle Mimicking Tumor with Recurrent Pulmonary Embolism.
Cardiac Amyloidosis Treatment.
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Diastolic Mitral Regurgitation.
Who are the sources who wrote this article ?

Published Date: May 08, 2022
Published 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.

What are the references for this article ?

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.