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Restrictive cardiomyopathy

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.

Causes

 

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:

  • 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

 

Symptoms

 

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)

 

Exams and Tests

 

A physical exam may show:

  • Enlarged (distended) or bulging neck veins
  • Enlarged liver
  • Lung crackles and abnormal or distant heart sounds in the chest heard through a stethoscope
  • Fluid backup into the hands and feet
  • Signs of heart failure

Tests for restrictive cardiomyopathy include:

  • Cardiac catheterization and coronary angiography
  • Chest CT scan
  • Chest x-ray
  • ECG (electrocardiogram)
  • Echocardiogram and Doppler study
  • MRI of the heart
  • Nuclear heart scan (MUGA, RNV)
  • Serum iron studies
  • Serum or urine protein tests

Restrictive cardiomyopathy may appear similar to constrictive pericarditis. Cardiac catheterization may help confirm the diagnosis. Rarely, a biopsy of the heart may be required.

 

Treatment

 

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
  • Steroids or chemotherapy for some causes

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

 

Outlook (Prognosis)

 

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 to Contact a Medical Professional

 

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

 

 

References

Hare JM. The dilated, restrictive, and infiltrative cardiomyopathies. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 10th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 65.

McKenna W, Elliott P. Diseases of the myocardium and endocardium. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 60.

 
  • Cardiomyopathy overview

    Cardiomyopathy overview

    Animation

  •  

    Cardiomyopathy overview - Animation

    Types, causes, symptoms, health risks, and treatment of cardiomyopathy.

  • Heart, section through the middle

    Heart, section through the middle - illustration

    The interior of the heart is composed of valves, chambers, and associated vessels.

    Heart, section through the middle

    illustration

  • Heart, front view

    Heart, front view - illustration

    The external structures of the heart include the ventricles, atria, arteries and veins. Arteries carry blood away from the heart while veins carry blood into the heart. The vessels colored blue indicate the transport of blood with relatively low content of oxygen and high content of carbon dioxide. The vessels colored red indicate the transport of blood with relatively high content of oxygen and low content of carbon dioxide.

    Heart, front view

    illustration

  • Cardiomyopathy overview

    Animation

  •  

    Cardiomyopathy overview - Animation

    Types, causes, symptoms, health risks, and treatment of cardiomyopathy.

  • Heart, section through the middle

    Heart, section through the middle - illustration

    The interior of the heart is composed of valves, chambers, and associated vessels.

    Heart, section through the middle

    illustration

  • Heart, front view

    Heart, front view - illustration

    The external structures of the heart include the ventricles, atria, arteries and veins. Arteries carry blood away from the heart while veins carry blood into the heart. The vessels colored blue indicate the transport of blood with relatively low content of oxygen and high content of carbon dioxide. The vessels colored red indicate the transport of blood with relatively high content of oxygen and low content of carbon dioxide.

    Heart, front view

    illustration

A Closer Look

 

    Self Care

     

      Tests for Restrictive cardiomyopathy

       

         

        Review Date: 5/5/2016

        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 Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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