Restrictive cardiomyopathy

Answer 1

Definition

Restrictive cardiomyopathy refers to a group of disorders in which the heart chambers are unable to properly fill with blood because of stiffness in the heart.

Alternative Names

Cardiomyopathy - restrictive; Infiltrative cardiomyopathy

Causes, incidence, and risk factors

In restrictive cardiomyopathy, the heart is of normal size or only slightly enlarged. However, it cannot relax normally during the time between heartbeats when the blood returns from the body to the heart (diastole).

Later in the disease, the heart may not pump blood efficiently. The abnormal heart function can affect the lungs, liver, and other body systems. Restrictive cardiomyopathy may affect either or both ventricles. It may be associated with a disease of the heart muscle.

The most common causes of restrictive cardiomyopathy are amyloidosis and scarring of the heart from an unknown cause (idiopathic myocardial fibrosis). It frequently occurs 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's syndrome (rare)
• Iron overload (hemochromatosis)
• Radiation fibrosis
• Sarcoidosis
• Scleroderma
• Tumors of the heart

Symptoms

Symptoms of heart failure are most common. Usually, these symptoms develop slowly over time. However, sometimes symptoms start very suddenly and are severe.

Common symptoms are:

• Cough
• Difficulty breathing
  • At night
  • Especially with exertion
  • When lying flat
• Fatigue, poor exercise tolerance
• Irregular or rapid pulse
• Loss of appetite
• Swelling of the abdomen
• Swelling of the feet and ankles

Other symptoms may include:

• Chest pain
• Decreased alertness or concentration
• Failure to thrive (in children)
• Low urine production
• Need to urinate at night (in adults)
• Shock (low blood pressure)

Signs and tests

An examination may show:

• Enlarged (distended) or bulging neck veins
• Enlarged liver
• Lung crackles and abnormal or distant heart sounds when listening to the chest with a stethoscope (auscultation)
• Fluid backup into the hands and feet
• Signs of heart failure

Children will have:

• Difficulty feeding
• Pale skin
• Poor growth
• Weak pulses in the legs and arms

Tests for restrictive cardiomyopathy include:

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

Restrictive cardiomyopathy may be hard to tell apart from constrictive pericarditis. A biopsy of the heart muscle or cardiac catheterization may help confirm the diagnosis.

Treatment

When the cause of any cardiomyopathy can be found, that condition is treated.

Few treatments are known to be effective 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 medications, either aspirin or warfarin
• Chemotherapy (in some situations)
• Diuretics to remove fluid and help improve breathing
• Medications to prevent or control irregular or abnormal heart rhythms
• Steroids for specific causes

A heart transplant may be considered if the heart function is very poor and the patient has many symptoms.

Expectations (prognosis)

People with restrictive cardiomyopathy may be heart transplant candidates. The outlook depends on the cause of the condition, but it is usually poor. Average survival after diagnosis is 9 years.

Complications

• Arrhythmias
• Mitral regurgitation
• Progressive heart failure
• Tricuspid regurgitation

Calling your health care provider

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

References

Hare JM. The dilated, restrictive, and infiltrative cardiomyopathies. In: Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 64.

Wexler RK, Elton T, Pleister A, Feldman D. Cardiomyopathy: An overview. Am Fam Physician. 2009;79:778-784.

Bernstein D. Diseases of the myocardium. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 439.

Answer 2

Definition

Restrictive cardiomyopathy refers to a group of disorders in which the heart chambers are unable to properly fill with blood because of stiffness in the heart.

Alternative Names

Cardiomyopathy - restrictive; Infiltrative cardiomyopathy

Causes, incidence, and risk factors

In restrictive cardiomyopathy, the heart is of normal size or only slightly enlarged. However, it cannot relax normally during the time between heartbeats when the blood returns from the body to the heart (diastole).

Later in the disease, the heart may not pump blood efficiently. The abnormal heart function can affect the lungs, liver, and other body systems. Restrictive cardiomyopathy may affect either or both ventricles. It may be associated with a disease of the heart muscle.

The most common causes of restrictive cardiomyopathy are amyloidosis and scarring of the heart from an unknown cause (idiopathic myocardial fibrosis). It frequently occurs 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's syndrome (rare)
• Iron overload (hemochromatosis)
• Radiation fibrosis
• Sarcoidosis
• Scleroderma
• Tumors of the heart

Symptoms

Symptoms of heart failure are most common. Usually, these symptoms develop slowly over time. However, sometimes symptoms start very suddenly and are severe.

Common symptoms are:

• Cough
• Difficulty breathing
  • At night
  • Especially with exertion
  • When lying flat
• Fatigue, poor exercise tolerance
• Irregular or rapid pulse
• Loss of appetite
• Swelling of the abdomen
• Swelling of the feet and ankles

Other symptoms may include:

• Chest pain
• Decreased alertness or concentration
• Failure to thrive (in children)
• Low urine production
• Need to urinate at night (in adults)
• Shock (low blood pressure)

Signs and tests

An examination may show:

• Enlarged (distended) or bulging neck veins
• Enlarged liver
• Lung crackles and abnormal or distant heart sounds when listening to the chest with a stethoscope (auscultation)
• Fluid backup into the hands and feet
• Signs of heart failure

Children will have:

• Difficulty feeding
• Pale skin
• Poor growth
• Weak pulses in the legs and arms

Tests for restrictive cardiomyopathy include:

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

Restrictive cardiomyopathy may be hard to tell apart from constrictive pericarditis. A biopsy of the heart muscle or cardiac catheterization may help confirm the diagnosis.

Treatment

When the cause of any cardiomyopathy can be found, that condition is treated.

Few treatments are known to be effective 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 medications, either aspirin or warfarin
• Chemotherapy (in some situations)
• Diuretics to remove fluid and help improve breathing
• Medications to prevent or control irregular or abnormal heart rhythms
• Steroids for specific causes

A heart transplant may be considered if the heart function is very poor and the patient has many symptoms.

Expectations (prognosis)

People with restrictive cardiomyopathy may be heart transplant candidates. The outlook depends on the cause of the condition, but it is usually poor. Average survival after diagnosis is 9 years.

Complications

• Arrhythmias
• Mitral regurgitation
• Progressive heart failure
• Tricuspid regurgitation

Calling your health care provider

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

References

Hare JM. The dilated, restrictive, and infiltrative cardiomyopathies. In: Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 64.

Wexler RK, Elton T, Pleister A, Feldman D. Cardiomyopathy: An overview. Am Fam Physician. 2009;79:778-784.

Bernstein D. Diseases of the myocardium. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 439.

Reviewed By

Review Date: 05/17/2010

Michael A. Chen, MD, PhD, Assistant Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.