Topic Overview
What is restrictive cardiomyopathy?
Restrictive
cardiomyopathy is a serious problem that makes your heart muscle stiff. When
your heart muscle is stiff, it can't stretch to allow enough blood to enter its
lower chambers, the
ventricles. So blood that would normally enter the
heart backs up in your circulatory system.
Most of the time, this
leads to
heart failure. Heart failure does not mean that your
heart stops pumping. It means that your heart can't pump enough blood to meet
your body's needs.
What causes restrictive cardiomyopathy?
Often the
cause is never found. But we do know that there are a number of diseases or
problems that can lead to restrictive cardiomyopathy. These include:
- Cardiac amyloidosis, a buildup of an
abnormal protein in the heart muscle.
-
Hemochromatosis
, a buildup of iron in the heart
muscle.
-
Sarcoidosis
, a rare type of heart inflammation.
-
Radiation therapy
and
chemotherapy, used to treat cancer.
-
Carcinoid syndrome
, a rare disease that causes certain
chemicals to be released into the blood stream. These chemicals can stiffen
heart muscle.
- Löeffler’s syndrome and endomyocardial fibrosis,
conditions that can cause scar tissue in the heart.
- Genetic
factors. You can inherit diseases, including Gaucher disease and
Fabry's disease, that can lead to restrictive
cardiomyopathy. But these diseases can be treated to prevent restrictive
cardiomyopathy.
What are the symptoms?
You may not have any
symptoms at first. Or you may have mild symptoms, such as feeling very tired or
weak.
If your heart gets weaker, you will develop heart failure.
When this happens, you will feel other symptoms, including:
- Shortness of breath, especially with
activity.
- Tiredness.
- Trouble breathing when you lie
down.
- Swelling in your legs.
- Chest pain.
Heart failure that suddenly gets worse is an emergency.
Get medical help right away if you:
- Have severe shortness of
breath.
- Have a fast or uneven heartbeat.
- Cough up
foamy, pink mucus.
- Have chest pain.
How is restrictive cardiomyopathy diagnosed?
Your
doctor will ask questions about your symptoms and past health. He or she will
want to know about recent illnesses and about heart disease in your family.
Your doctor will listen to your heart and lungs and check your legs for fluid
buildup.
You may also have other tests, including:
In some cases, a doctor may want to look at a small
sample of heart tissue, called a
biopsy, to make a definite diagnosis.
How is it treated?
You will probably need to take
several medicines to treat heart failure caused by restrictive cardiomyopathy.
It is very important to take your medicines exactly as your doctor tells you to
and to keep taking them. If you don't, your heart failure could get worse.
Lifestyle changes are an important part of your treatment. Taking
these steps can help slow down heart failure.
- Limit how much salt you eat. Salt causes
water to build up in your body and makes it harder for your heart to pump.
Limit your fluid intake if your doctor tells you to.
- Limit your
physical activity. Talk to your doctor about the best balance of rest and
activity.
- Limit how much alcohol you drink.
Your doctor may suggest a mechanical device to help your
heart pump blood or prevent life-threatening irregular heart rhythms. Such
devices include a
pacemaker or
implantable cardioverter-defibrillator (ICD). If your
condition is very bad, a heart transplant may be an option.
Keeping track of your symptoms every day is an important part of your
treatment. Call your doctor if:
- You have a sudden weight gain such as
3 lb (1.4 kg) or more in 2 to 3
days.
- Your ability to exercise changes.
- You have
any sudden change in your symptoms.
What can you expect with restrictive cardiomyopathy?
Most of the time, restrictive cardiomyopathy leads to heart failure.
Heart failure usually gets worse over time, but treatment can slow the disease
and help you feel better and live longer. If your doctor finds the cause of
your restrictive cardiomyopathy, then the cause will also be treated, if
possible.
Some people develop other problems, including:
-
Stroke
.
-
Heart attack
.
- A blood
clot in the lung, called a
pulmonary embolism.
- Sudden cardiac death,
which means the heart suddenly stops working. This may be more likely to happen
to people who have serious rhythm problems (arrhythmias) in
one of the lower heart chambers (ventricles).
If your disease is getting worse over time, you may want
to think about making end-of-life decisions. It can be comforting to know that
you will get the type of care you want.
Frequently Asked Questions
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Learning about restrictive cardiomyopathy:
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Being diagnosed:
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Getting treatment:
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Ongoing concerns:
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Living with restrictive cardiomyopathy:
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End-of-life issues:
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Symptoms
Symptoms of
restrictive cardiomyopathy are usually the same as
those typically seen in
heart failure and include:
- Weight gain caused by water retention and
fluid buildup (edema), especially in the legs, ankles, and
feet.
- Abdominal swelling, tenderness, or pain, which may result
from the buildup of fluid in the abdomen (ascites) and from blood that backs up
in the liver.
- Difficulty breathing during normal activities or exercise that
did not cause breathing problems before.
- A dry, hacking cough,
especially when lying down. At night, you may wake up coughing with a rapid
heart rate and a feeling of being suffocated (paroxysmal nocturnal
dyspnea).
- Dizziness, fainting, or feeling tired or
weak.
-
Heart palpitations
.
- Increased urination at
night.
Sudden heart failure can develop,
causing congestion and rapid fluid buildup in the lungs (pulmonary edema). This is an emergency medical situation
and requires immediate care. Symptoms develop quickly and include:
- Severe shortness of breath.
- An
irregular or rapid heartbeat.
- Coughing up foamy, pink mucus.
Sudden heart failure is different from gradual heart
failure and may be triggered by:
- Eating too much salt.
- Not following
directions for taking medicines.
- An irregular or rapid heartbeat
that comes on suddenly.
- Having a heart attack.
- Drinking alcohol or taking drugs like cocaine.
Complications of restrictive cardiomyopathy can also be
very serious. They include:
- A blood clot that develops in the heart. The
clot can break loose, travel through the bloodstream, become lodged in a blood
vessel, and block blood supply to:
- The brain, resulting in a
stroke.
- The heart, causing a
heart attack.
- The lungs, causing a
pulmonary embolism.
- A limb or elsewhere in
the body, possibly causing a loss of circulation and tissue damage.
- Episodes of rapid, abnormal heart rhythms
(arrhythmia), especially
atrial fibrillation.
- Sudden cardiac
death.
Exams and Tests
If
restrictive cardiomyopathy is identified correctly and
right away, symptoms can be treated more successfully. To diagnose restrictive
cardiomyopathy, your doctor will ask about your medical history. Be prepared to
discuss any conditions or diseases that you or your family members have had. In
addition, you should be able to describe your symptoms and how often you
experience them.
Your doctor will also do a thorough physical
examination, including listening to your heart and lungs with a stethoscope.
Signs of
heart failure can include:
- Unusual sounds,
heart murmurs, or extra sounds called gallops, which
may mean you have a problem with the heart's walls or valves. Pulmonary
rales—crackles or bubbling sounds heard in the chest—may mean there is fluid
buildup in the lungs.
- Fluid buildup (edema), especially in the legs
and feet.
- Bulging neck veins.
It is important to remember that people of all ages can
develop restrictive cardiomyopathy. When symptoms of
cardiomyopathy first occur, several tests can help
your doctor diagnose whether another condition, such as
sarcoidosis (the formation of nodules) or
hemochromatosis (a buildup of iron in the heart
muscle), is causing symptoms.
Echocardiogram: An
echocardiogram, sometimes called an echo, is an
ultrasound exam that uses high-pitched sound waves to
create an image of the heart on a television screen. This painless and
noninvasive test—which determines if your lower heart chambers (ventricles) are
filling too rapidly, a specific sign of restrictive cardiomyopathy—is the
easiest way to diagnose restrictive cardiomyopathy.
Electrocardiogram: An
electrocardiogram (ECG, EKG) records the electrical
activity in the heart as impulses move through it during contraction and
relaxation. An electrocardiogram can determine whether heart muscle is damaged
and may also suggest other possible causes of cardiomyopathy, such as a heart
attack or a buildup of protein in the heart muscle (cardiac
amyloidosis).
Chest X-ray: A
chest X-ray shows the size and shape of the heart and
whether there is fluid buildup in the lungs. In a heart affected by restrictive
cardiomyopathy, the upper heart chambers (atria) may sometimes appear enlarged,
although usually the overall heart size is normal or only slightly
enlarged.
Imaging tests: More complex
tests, called
magnetic resonance imaging (MRI) and
computed tomography (CT), may be used to evaluate
restrictive cardiomyopathy. The major benefit of these tests is that they can
help distinguish between restrictive cardiomyopathy and
constrictive pericarditis. If the sac around the heart
(pericardium) is thickened, the symptoms are more
likely due to constrictive pericarditis than to restrictive
cardiomyopathy.
Cardiac catheterization or coronary angiogram: During
cardiac catheterization, a thin, flexible tube called
a catheter is threaded through an artery or vein in the arm or groin and into
the blood vessels of the heart to measure pressure in the heart chambers. In
restrictive cardiomyopathy, there is typically a higher-than-normal pressure
inside the heart chambers. Dye can also be injected through the catheter to see
how the heart chambers are pumping and whether heart valves are leaking. The
process of injecting dye into the coronary arteries is called coronary
angiography. Cardiac catheterization may help determine whether you have
constrictive pericarditis or restrictive cardiomyopathy.
Other procedures: Sometimes a sample (biopsy) of the
heart tissue—usually done during cardiac catheterization—or the pericardium is
the only way to determine the cause of restrictive cardiomyopathy (for example,
whether it is caused by hemochromatosis or sarcoidosis).
Treatment Overview
In most cases,
restrictive cardiomyopathy is a progressive disease in
which the cause of the disease is not known (idiopathic restrictive
cardiomyopathy) and so cannot be directly treated. Typically, the heart muscle
continues to stiffen and lose function and strength, and
heart failure (inability of the heart to pump enough
blood) develops. In these cases, treatment involves trying to decrease the
heart's workload.
In some cases the cause of restrictive
cardiomyopathy can be identified, such as in
carcinoid syndrome,
sarcoidosis, and amyloidosis, although the treatment
is generally ineffective.
Corticosteroids may offer limited benefit in treating
sarcoidosis and amyloidosis. In most of these cases, the condition is
progressive, and treatment involves trying to manage heart failure
symptoms.
But in other cases where the cause is diagnosed early
and can be treated, further heart damage may be prevented. For example, in
restrictive cardiomyopathy caused by
hemochromatosis (a buildup of iron in the heart
muscle), treatment may involve the use of drugs that help eliminate excess iron
(chelating agents) and/or
phlebotomy (withdrawing blood). In another example, an
inherited problem called
Fabry's disease can be treated to prevent restrictive
cardiomyopathy from happening.
If heart failure has developed,
your doctor will prescribe medicines to manage its symptoms and complications.
They may include:
-
Diuretics, which help eliminate fluid
buildup in the lungs and elsewhere in the body.
-
Angiotensin-converting enzyme (ACE) inhibitors or
angiotensin II receptor blockers (ARBs) to improve
blood flow and reduce the heart's workload. ARBs may be used when a person
cannot tolerate ACE inhibitors or when ACE inhibitors are not controlling
symptoms.
-
Beta-blockers, which slow the heart rate and reduce
blood pressure.
-
Anticoagulants, to help prevent blood
clots from forming in the heart. People with restrictive cardiomyopathy,
especially those with
atrial fibrillation, are at risk for developing clots,
which can travel through the bloodstream to other places in the body and cause
a
heart attack,
pulmonary embolism, or
stroke.
-
Digoxin, which can
help increase the strength of heart contractions. These medicines are sometimes
used, although with caution in those people who have amyloidosis, because they
can lead to serious arrhythmias.
-
Calcium channel blockers, which slow your heart rate and lower blood pressure. These
medicines may be used to treat diastolic heart failure, when the heart has
problems filling with blood.
Surgery
An artificial
pacemaker may be surgically placed in the chest. Scar
tissue that develops in restrictive cardiomyopathy may block electrical
impulses traveling through the heart and result in abnormal heartbeats, called
arrhythmias. Pacemakers stimulate the heart muscle to beat regularly when the
electrical signals from the top of the heart are blocked.
An
implantable cardioverter-defibrillator (ICD) is
another small device that may be surgically placed in the chest. It is used to
lower the risk of sudden death from life-threatening irregular heart rhythms
(arrhythmias). An ICD continuously monitors your heart.
If it detects a life-threatening rapid heart rhythm, it sends an electric shock
to your heart to restore a normal rhythm. You may need an ICD if you have had a
serious episode of life-threatening irregular heart rhythm or are at high risk
for having one.
A
heart transplant is available to a small number of
people who have severe end-stage restrictive cardiomyopathy and who meet
specific criteria for transplantation. The diseased heart is removed and
replaced with a healthy heart donated by a person who has recently died. There
are limited donor hearts available.
Stem cell transplantation
may be used for amyloidosis
(a buildup of protein), although the long-term benefits are not known.
Home Treatment
While medical care is important in
treating
restrictive cardiomyopathy, the following self-care
recommendations are also critical.
- Limit alcohol. Drink moderately, which is 2
drinks a day or less for men or 1 drink a day or less for women. Long-term
overuse of alcohol may increase the risk of developing cardiomyopathy in some
people.
- Restrict salt (sodium) in your diet. The body attempts to
compensate for heart failure by retaining salt and water. This leads to fluid
buildup and swelling. For more information, see:
Heart failure: Eating less salt.- Low-salt diets: Eating out.
- Limit fluids if your doctor tells you to. Talk to
your doctor about how much fluid is safe for your specific condition. For more
information, see:
- Heart failure: Watching your fluids.
- Weigh yourself daily. If fluid begins to build up rapidly, you
will notice a sudden weight gain. Your doctor may tell you how much weight to
watch for. But in general, call your doctor if you gain
3 lb (1.4 kg) or more in 2 to 3
days. For more information, see:
- How do I check my weight?.
- Limit your physical activity. People with
restrictive cardiomyopathy may need to avoid overexertion because their hearts
are not able to increase blood flow during exercise. Talk to your doctor about
the best balance of rest and activity for your specific condition.
- Avoid triggers for sudden heart failure. For more information,
see:
- Heart failure: Avoiding triggers for sudden heart failure.
- Take your medicines as directed. If you don't,
your heart failure may get worse, or you may develop
sudden heart failure. For more information, see:
- Heart failure: Taking medicines properly.
- Be careful using nonprescription medicines. Some
medicines can make your heart failure worse. For more information see:
For more information on home treatment, see the topic
Heart Failure.
Other Places To Get Help
Organizations
|
American Heart Association (AHA)
|
| 7272 Greenville Avenue |
| Dallas, TX 75231 |
| Phone: |
1-800-AHA-USA1 (1-800-242-8721) |
| Web Address: |
www.americanheart.org |
| |
|
Call the American Heart Association (AHA) to find your
nearest local or state AHA group. AHA can provide brochures and information
about support groups and community programs, including Mended Hearts, a
nationwide organization whose members visit people with heart problems and
provide information and support. AHA's Web site also has information on
physical activity, diet, and various heart-related conditions.
|
|
|
National Heart, Lung, and Blood Institute
(NHLBI)
|
| P.O. Box 30105 |
| Bethesda, MD 20824-0105 |
| Phone: |
(301) 592-8573 |
| Fax: |
(240) 629-3246 |
| TDD: |
(240) 629-3255 |
| E-mail: |
nhlbiinfo@nhlbi.nih.gov |
| Web Address: |
www.nhlbi.nih.gov |
| |
|
The U.S. National Heart, Lung, and Blood Institute
(NHLBI) information center offers information and publications about preventing
and treating:
- Diseases affecting the heart and circulation, such as heart
attacks, high cholesterol, high blood pressure, peripheral artery disease, and
heart problems present at birth (congenital heart diseases).
- Diseases that affect the lungs, such as asthma, chronic
obstructive pulmonary disease (COPD), emphysema, sleep apnea, and
pneumonia.
- Diseases that affect the blood, such as anemia,
hemochromatosis, hemophilia, thalassemia, and Von Willebrand disease.
|
|
|
Texas Heart Institute
|
| P.O. Box 20345 |
| Houston, TX 77225-0345 |
| Phone: |
1-800-292-2221 (Heart Information Service hotline)
(832) 355-4011 (general line) |
| E-mail: |
his@heart.thi.tmc.edu (Heart Information Services) |
| Web Address: |
www.texasheartinstitute.org |
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The Texas Heart Institute's national telephone hotline is staffed
by medical professionals who can answer heart-related health questions. The Web
site provides information on a wide range of heart topics, including common
disorders and prevention programs.
|
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References
Other Works Consulted
- Hare JM (2008). The dilated, restrictive, and
infiltrative cardiomyopathies. In P Libby, ed., Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed., vol. 2,
pp. 1739–1761. Philadelphia: Saunders Elsevier.
- Hoit BD, Gupta S (2008). Restrictive, obliterative,
and infiltrative cardiomyopathies. In V Fuster et al., eds., Hurst's The Heart, 12th ed., pp. 851–862. New York:
McGraw-Hill Medical.
- Hunt SA, et al. (2009). 2009 focused update
incorporated into the ACC/AHA 2005 guidelines for the diagnosis and management
of heart failure in adults. A report of the American College of Cardiology
Foundation/American Heart Association Task Force on Practice Guidelines.
Circulation, 119(14): e391–e479.
Credits
|
Author
|
Robin Parks, MS |
|
Editor
|
Kathleen M. Ariss, MS |
|
Associate Editor
|
Pat Truman, MATC |
|
Primary Medical Reviewer
|
Caroline S. Rhoads, MD - Internal Medicine |
|
Specialist Medical Reviewer
|
Stephen Fort, MD, MRCP, FRCPC - Interventional Cardiology |
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Last Updated
|
August 26, 2008 |
