Topic Overview
What is hypertrophic cardiomyopathy?
Hypertrophic
cardiomyopathy (say "hy-per-TROH-fik kar-dee-oh-my-AWP-uh-thee") happens when
the heart muscle grows too thick, so the heart gets bigger and its
chambers get smaller. This may result in:
- No symptoms or few symptoms. Many people
have no symptoms and live a normal life with few problems.
- The
heart not getting enough blood and oxygen, which can cause chest pain.
- A fast, slow, or uneven heartbeat (arrhythmia). In
rare cases, this can cause sudden death.
- The heart not pumping
blood well or not relaxing between beats as it normally does. In rare cases,
this can lead to
heart failure.
See a picture of a normal heart and a heart with
hypertrophic cardiomyopathy.
Hypertrophic
cardiomyopathy is the most common
genetic heart disease. This means it runs in families. About 1 out of 500 adults have this condition. It
often starts early in life, from the teens through the mid-30s.1
It
cannot be cured, but you can treat the symptoms.
What causes hypertrophic cardiomyopathy?
Certain
genes cause the heart to grow more than it should. If
you have family members with the disease, you are more likely to get it.
What are the symptoms?
If you have hypertrophic
cardiomyopathy, you may:
- Have no symptoms.
- Feel tired and
short of breath when you are active.
- Feel dizzy or faint, often
after you have been active.
- Feel like your heart is pounding,
racing, or beating unevenly (palpitations).
- Have chest
pain (angina). You may have a heavy, tight feeling in your
chest. Chest pain is often brought on by exercise, when the heart has to work
harder.
Call your doctor if:
- You have a rapid or irregular heartbeat or
fainting spells. You may have an arrhythmia, which makes sudden death more
likely. People with hypertrophic cardiomyopathy are at a higher risk for sudden
death than other people and can die at a young age.
- You have
symptoms that might be caused by heart failure, such as shortness of breath,
being very tired, or swelling in your legs or ankles.
How is hypertrophic cardiomyopathy diagnosed?
Your doctor will ask you about any health problems you've had and about
any family history of heart disease or early and sudden death. Your doctor will
do a physical exam. You may need tests such as an
electrocardiogram (ECG or EKG), chest X-ray, or
echocardiogram.
Your doctor may refer
you to a doctor who specializes in heart problems (cardiologist). Based on your
symptoms, past health, and family history, the specialist can assess your risk
for sudden death. People who are at high risk will need regular checkups.
If your parents or any brothers or sisters have the disease or
died suddenly at a young age, you are at risk. Talk to your doctor about
getting tests to check for the disease.
How is it treated?
Many people with hypertrophic
cardiomyopathy don't need treatment. Treatment depends on your symptoms and
whether you have developed heart failure or abnormal heart rhythms.
- You may take medicines to treat symptoms such
as shortness of breath and chest pain.
- An arrhythmia such as
atrial fibrillation is treated with medicines to
control the heart rate and rhythm and to prevent blood clots. Or you may get
cardioversion, an electrical shock to return the heart
to its normal rhythm.
- Heart failure is treated with medicines and
lifestyle changes, such as eating less salt. Surgery also can be an option.
If your doctor feels you are at high risk for sudden death
from an arrhythmia, you may need an
implantable cardioverter-defibrillator (ICD). An ICD
is a small device like a pacemaker. It treats dangerous heart rhythms.
What else can you do for hypertrophic cardiomyopathy?
Many adults with this disease have full and long lives. You can help
yourself by not smoking and by eating healthy foods.
Avoid strenuous activity and intense exercise, because they could lead to
sudden death. Talk with your doctor about activity levels that are right for
you.
Also talk to your doctor about how often you need checkups.
Frequently Asked Questions
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Symptoms
Many people with
hypertrophic cardiomyopathy do not have symptoms.
But if symptoms occur, at first they generally include:
- Shortness of breath (dyspnea).
- Chest pain or discomfort (angina).
- Fainting or near-fainting (syncope), especially with exertion.
- Heart
palpitations, an uncomfortable awareness of the heart
beating rapidly or irregularly.
Sudden death may occur from the onset of ventricular
tachycardia (a type of rapid heart rate) or other dangerous
arrhythmias. A genetic factor appears to influence
which people with hypertrophic cardiomyopathy are more prone to sudden death.
Other risk factors for sudden death include severe obstruction of the left
ventricle, multiple fainting (syncope) episodes, recurring episodes of
ventricular tachycardia, and an abnormal drop in blood pressure during
exercise.
Although it can occur in any age group, sudden death is
most shocking when it happens to young adults or athletes. While these tragic
deaths are often given prominence in the media, sudden death is rare (1% or
less each year in adults with hypertrophic cardiomyopathy).2
Complications of hypertrophic cardiomyopathy
Atrial fibrillation is a common complication of
hypertrophic cardiomyopathy. This abnormal heart rhythm interferes with the
normal pumping of the heart. It can cause blood clots to develop in the heart,
which can break off and travel through the bloodstream (systemic embolism).
This may cause a
stroke, heart attack, or blocked blood flow to an arm
or leg.
Heart failure
may develop if the disease progresses. In heart failure, the heart's lower
chambers are not able to pump blood effectively enough to meet the body's needs
for oxygen and nutrients. Common symptoms include fluid buildup (edema) in the
legs, ankles, and feet; shortness of breath while lying down or exercising; and
increased urination at night.
Athlete's heart syndrome
People who exercise
regularly and vigorously often develop changes in their heart muscle that can
be confused for hypertrophic cardiomyopathy. In such athletes, the heart muscle
grows to adapt to the extra demands from physical activities. This condition is
called athlete's heart syndrome. But unlike hypertrophic cardiomyopathy,
athlete's heart syndrome does not cause life-threatening heart rhythms and
sudden death. It is a benign, or harmless, condition. When an athlete stops
training, the heart returns to a normal size unlike those with hypertrophic
cardiomyopathy, in which the heart remains enlarged.
Exams and Tests
Hypertrophic cardiomyopathy can be difficult to diagnose, because it may not always
cause symptoms. The first step in diagnosing heart problems is a thorough
medical history and physical exam.
Your doctor will ask you about
any family history of hypertrophic cardiomyopathy or any heart disease. Your
doctor will also want to know if any relatives died an early and sudden death.
Sometimes when young people die of cardiac arrest from hypertrophic
cardiomyopathy, the disorder is not discovered because autopsies are not always
performed.
During the physical exam, your doctor will listen to
your heart with a stethoscope. If any extra or unusual heart sounds (gallops or murmurs) are heard, it may mean the
structure of the heart is abnormal.
You will usually have one or
more of the following tests to help your doctor diagnose and treat your
condition.
Electrocardiogram
An
electrocardiogram (ECG or EKG) measures the electrical
activity as it moves through the heart during contraction and relaxation. An
abnormal electrocardiogram may be the first sign of hypertrophic cardiomyopathy
in people who do not have any symptoms.
Echocardiogram
An
echocardiogram (echo) is a type of ultrasound exam
that uses high-pitched sound waves to create an image of the heart, which is
seen on a television screen. An echo is the main tool used to help doctors
diagnose hypertrophic cardiomyopathy and find out how bad it is.
Echocardiography can be used to:
- Estimate how well the lower left chamber of
the heart (left ventricle) is able to fill when the heart expands and measure
how much blood is pumped out as it contracts (ejection
fraction).
- Find out if the heart valves are functioning
normally.
- Measure overall heart size.
- Find out if the
heart muscle (myocardium), including the wall that separates the left and right
chambers of the heart, is unusually thick.
- Measure the degree that
blood flow is reduced during contraction (systole) if the wall that separates
the left and right chambers of the heart is abnormally thick.
Physical exam, electrocardiogram, and echocardiogram are
the best ways to diagnose hypertrophic cardiomyopathy. People with a family
history of sudden death, especially young athletes or those who are thinking about
starting an exercise program, should talk to their doctors about being tested
for hypertrophic cardiomyopathy. These tests may also help evaluate a person
who faints during strenuous physical activity.
Patients who are
considered to be low risk will not see their doctor as often as patients
considered at high risk, usually around every 3 years. An echocardiogram is
usually done again if your symptoms change or get worse.
Exercise test
Your doctor may have you run on a
treadmill or pedal a stationary bike while he or she looks at how well your
heart and lungs are working. An exercise test can give the doctor (and you) an
idea of how hard and how long you can exercise.
The results of an
exercise test may show that you have a higher risk for serious health problems
because of hypertrophic cardiomyopathy. Some doctors recommend an exercise test
before beginning any treatment for hypertrophic cardiomyopathy.
Chest X-ray
A
chest X-ray produces a photographic image of the heart
using rays of intense light energy that pass through the body and project an
image on a film. An X-ray can show information about the heart's size and
shape. In later stages of hypertrophic cardiomyopathy, an X-ray may reveal
signs of
pulmonary edema, which is fluid buildup in the
lungs.
Cardiac catheterization (cardiac cath) or coronary angiography
During a
cardiac catheterization or coronary angiogram, a long,
thin tube (catheter) is threaded through an artery or vein in the arm or groin
and into the heart to measure pressure in the heart chambers. Dye can be
injected through the catheter to see whether the coronary arteries are blocked,
how well the heart chambers are pumping, and whether heart valves are leaking.
Angiography may be done if the results of the echocardiogram are
inadequate. Angiography may also be used to check if surgery would be an option
for treatment of hypertrophic cardiomyopathy.
Genetic testing
Genetic testing can identify some
genes that are related to this condition. Genetic tests are not commonly used to diagnose hypertrophic cardiomyopathy.
Genetic testing might be done for close relatives of people who have hypertrophic cardiomyopathy. The testing can help see if they carry a genetic change that raises their risk for getting the disease. An echocardiogram or electrocardiogram are more commonly done to check their hearts.
Treatment Overview
Initial treatment
Many people with
hypertrophic cardiomyopathy do not need treatment. But
in some cases, having a thickened heart muscle can cause problems. If symptoms
develop, treatment is usually recommended. Medicines cannot cure hypertrophic
cardiomyopathy, but they may be used to treat complications, including
atrial fibrillation and
heart failure. These medicines include:
- Beta-blockers. Beta-blockers are often used to
treat people who develop symptoms such as shortness of breath or chest pain.
They lower blood pressure, slow heart rate, and improve blood flow, which helps
decrease symptoms and improves your ability to exercise. They may even prevent
or delay the progression of heart failure related to hypertrophic
cardiomyopathy.
- Calcium channel blockers. Calcium channel blockers are used to help relieve symptoms, especially chest pain. These
medicines also slow heart rate and lower blood pressure.
- Antiarrhythmic medicines. These medicines are used to control the heart rhythm. One example is disopyramide (Norpace). An antiarrhythmic medicine might be used if you also have atrial fibrillation.
Atrial fibrillation occurs in about 1 out of 4 people
who have hypertrophic cardiomyopathy. In
atrial fibrillation, abnormal electrical impulses
cause the upper chambers of the heart (atria) to fibrillate, or quiver,
resulting in irregular and rapid beating of the ventricles, the heart's main
pump. For most people, this aspect of atrial fibrillation in itself is usually
not life-threatening. But for people who have hypertrophic cardiomyopathy,
atrial fibrillation can increase your risk for other abnormal heart rhythms
that can be life-threatening. It also increases your risk for heart failure and
stroke. For these reasons, most doctors aggressively treat atrial fibrillation
in people who have hypertrophic cardiomyopathy. Aggressive treatment may
include medicines to control the heart rate or rhythm,
electrical cardioversion to return the heart to its
normal rhythm, or catheter ablation or surgery to destroy heart tissue that is
causing atrial fibrillation. For more information, see the topic
Atrial Fibrillation.
Anticoagulants often are prescribed for people who
have atrial fibrillation. Anticoagulants help protect against blood clots that
develop in the heart. Blood clots can be dangerous because they may break loose
and travel through the bloodstream (thromboembolism), which may cause a stroke,
heart attack, or blocked blood flow to an arm or leg.
Most people
with hypertrophic cardiomyopathy should be assessed by a cardiologist to
find out their risk for
ventricular tachycardia, an abnormally fast heart rate
that can result in sudden death. For those in a
high-risk category, an
implantable cardioverter-defibrillator (ICD) appears
to be the most effective treatment for preventing sudden death.
Because of the risk of sudden death, it is important for people with
hypertrophic cardiomyopathy to avoid too much
strenuous activity and intense exercise. Talk to your doctor about what level
of exercise and what kinds of activities are safe. Prolonged activity in hot
weather is not recommended, because dehydration can also make symptoms in people with hypertrophic cardiomyopathy worse.
Ongoing treatment
It is important for people with
high-risk
hypertrophic cardiomyopathy to have frequent check-ups
with their doctors. People who are low-risk may not see their doctors as often.
But you may see the doctor more often if you have a change in your symptoms or
your overall health. When symptoms appear or start to get worse, a check-up
might include an
echocardiogram (echo),
electrocardiogram (ECG, EKG), or exercise test. Your
doctor will talk about your symptoms and your health history. You may also talk
about the health history of people in your family. These regular visits will
help your doctor identify things that may put you at risk for sudden cardiac
death and other serious medical conditions.
If symptoms develop,
treatment is usually recommended. Medicines cannot cure hypertrophic
cardiomyopathy, but they may be used to treat complications, including
atrial fibrillation and
heart failure. After medicines are started, most
people need to take them for the rest of their lives.
Treatment if the condition gets worse
If you
have serious heart rhythm problems or are at high risk for sudden death,
your doctor might recommend an
implantable cardioverter-defibrillator (ICD).
Medicines for heart failure may be used if
hypertrophic cardiomyopathy progresses to that
advanced state. For more information, see the topic
Heart Failure.
A surgery called a
myectomy or myomectomy may be advised for some people when medicines do not
help relieve severe symptoms of heart failure (NYHA class III and IV) due to hypertrophic cardiomyopathy. In this surgery, a portion of
overgrown heart muscle is removed. Often the excess muscle tissue is found in
the septum, which divides the left and right lower heart chambers (ventricles).
An overgrown septum can interfere with the function of the left ventricle and
limit blood flow out of the heart. Most people who have this surgery recover
well and end up with fewer symptoms. After surgery, physical activity is easier
too.
Another option for people with hypertrophic cardiomyopathy
is nonsurgical septal reduction, also called alcohol septal ablation. When the area of the heart muscle that divides
the right and left chambers (septum) becomes too thick, the lower left heart
chamber (left ventricle) becomes obstructed, which hinders its ability to pump
normally. The thickened septum is reduced in size by injecting alcohol into the
coronary artery that supplies this area of the heart with blood. The alcohol
destroys some of the heart muscle in the thickened septum. This reduces the
obstruction and improves the left ventricle's pumping ability. Advantages of
this procedure are that major surgery and lengthy recovery are avoided because
the alcohol can be given through a catheter during a
cardiac catheterization procedure. This procedure can help relieve symptoms. But it is a newer procedure, so the long-term effects are not yet known. Experts recommend that this complex procedure be performed in a large
medical center where the staff has substantial experience with it.
Home Treatment
Because of the risk of sudden death, it
is important for people with
hypertrophic cardiomyopathy to avoid too much
strenuous activity and intense exercise. Sudden death in people who have
hypertrophic cardiomyopathy can occur during or just after strenuous physical
activity. Talk to your doctor about what level of exercise and what kinds of
activities are safe. Because dehydration can also make symptoms in people
with hypertrophic cardiomyopathy worse, prolonged activity in hot weather is not
recommended.
The following self-care recommendations are also
important.
- Be active at low aerobic levels to help keep your heart and body healthy. Ask your doctor about activities that are safe for you.
- If you smoke, try to quit. Medicines and counseling can help you quit for good.
- Stay well hydrated
(unless you have heart failure or another medical condition and need to limit
your fluid intake).
Follow your doctor's recommendations for regular exams to
monitor your condition.
Because some people with hypertrophic
cardiomyopathy may be at increased risk for sudden death, it is a good idea for
family members to learn CPR (cardiopulmonary resuscitation).
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.heart.org |
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Visit the American Heart Association (AHA) website for information on
physical activity, diet, and various heart-related conditions. You can search for information on heart disease and stroke, share information with friends and family, and use tools to help you make heart-healthy goals and plans. Contact the AHA to find your
nearest local or state AHA group. The AHA provides 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.
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| 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 |
| Email: |
nhlbiinfo@nhlbi.nih.gov |
| Web Address: |
www.nhlbi.nih.gov |
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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.
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References
Citations
- Ommen SR, et al. (2008). Hypertrophic cardiomyopathy.
In V Fuster et al., eds., Hurst's the Heart, 12th ed.,
pp. 822–850. New York: McGraw-Hill Medical.
- McKenna WJ, Elliott PM (2007). Hypertrophic
cardiomyopathy. In EJ Topol, ed., Textbook of Cardiovascular Medicine, 3rd ed., pp. 482–501. Philadelphia: Lippincott Williams and
Wilkins.
Other Works Consulted
- Fifer MA, Vlahakes GJ (2008). Management of symptoms
in hypertrophic cardiomyopathy. Circulation, 117(3):
429–439.
- Gersh BJ, et al. (2011). 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation, 124(24): e783–e831.
- Maron BJ (2008). Hypertrophic cardiomyopathy. In P
Libby et al., eds., Braunwald's Heart Disease, 8th ed.,
pp. 1763–1774. Philadelphia: Saunders Elsevier.
Credits
| By |
Healthwise Staff |
| Primary Medical Reviewer |
E. Gregory Thompson, MD - Internal Medicine |
| Specialist Medical Reviewer |
Stephen Fort, MD, MRCP, FRCPC - Interventional Cardiology |
| Last Revised |
February 3, 2012 |
