Hypertropic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is a condition in which the heart muscle becomes thick. Often, only one part of the heart is thicker than the other parts. The thickening can make it harder for blood to leave the heart, forcing the heart to work harder to pump blood. It also can make it harder for the heart to relax and fill with blood.

Causes

Hypertrophic cardiomyopathy is a condition that is most often passed down through families (inherited). It is thought to result from defects in the genes that control heart muscle growth. Younger people are likely to have a more severe form of hypertrophic cardiomyopathy. However, the condition is seen in people of all ages.

Symptoms

Some people with the condition may have no symptoms. They may first find out they have the problem during a routine medical exam. In many young adults, the first symptom of hypertrophic cardiomyopathy is sudden collapse and possible death. This can be caused by highly abnormal heart rhythms (arrhythmias). It may also be due to a blockage that prevents the outflow of blood from the heart to the rest of the body.

• Chest pain

• Dizziness

• Fainting, especially during exercise

• Fatigue

• Light-headedness, especially with or after activity or exercise

• Sensation of feeling the heart beat (palpitations)

• Shortness of breath with activity or after lying down (or being asleep for a while)

Exams and Tests

The health care provider will perform a physical exam and listen to the heart and lungs with a stethoscope. The pulse in your arms and neck will also be checked. The doctor may feel an abnormal heartbeat in the chest. Tests used to diagnose heart muscle thickness, problems with blood flow, or leaky heart valves (mitral valve regurgitation) may include:

• Echocardiography

• ECG

• 24-hour Holter monitor (heart rhythm monitor)

• Cardiac catheterization

• Chest x-ray

• MRI of the heart

• Transesophageal echocardiogram (TEE)

• Blood tests may be done to rule out other diseases.

• Close family members of people with hypertrophic cardiomyopathy may be screened for the condition.

Treatment

Always follow your doctor's advice about exercise if you have hypertrophic cardiomyopathy. You may be told to avoid strenuous exercise. Also, see your doctor for regularly scheduled checkups. If you have symptoms, you may need medicines such as beta-blockers and calcium channel blockers to help the heart contract and relax correctly. These drugs may relieve chest pain or shortness of breath when exercising.

People with arrhythmias may need treatment, such as:

• Medicines to treat the abnormal rhythm

• Blood thinners to reduce the risk of blood clots (if the arrhythmia is due to atrial fibrillation)

• A permanent pacemaker to control the heartbeat

• An implanted defibrillator that recognizes life-threatening heart rhythms and sends an electrical pulse to stop them. Sometimes a defibrillator is placed, even if the patient has not had an arrhythmia but is at high risk for a deadly arrhythmia (for example, if the heart muscle is very sick or the patient has a relative who has died suddenly).

When blood flow out of the heart is severely blocked, symptoms can become severe. An operation called surgical myectomy may be done. In some cases, patients may be given an injection of alcohol into the arteries that feed the thickened part of the heart (alcohol septal ablation). People who have this procedure often show much improvement. You may need surgery to repair the heart's mitral valve if it is leaking.

Prognosis

Some people with hypertrophic cardiomyopathy may not have symptoms and will have normal lifespan. Others may get worse slowly or quickly. In some cases, the condition may develop into dilated cardiomyopathy. People with hypertrophic cardiomyopathy are at higher risk for sudden death than people without the condition. Sudden death can occur at a young age. There are different types of hypertrophic cardiomyopathy, which have different prognoses. The outlook may be better when the disease occurs in elderly individuals or when there is a particular pattern of thickness in the heart muscle.