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Hypertrophic Cardiomyopathies

Hypertrophic cardiomyopathy (HCM) is a disease in which the heart muscle becomes thick. The thicker wall may block blood flow out of the heart.

If there's no significant blocking of blood flow, the heart's main pumping chamber (left ventricle) may still become stiff. This makes it hard for the heart to relax and reduces the amount of blood the ventricle can hold and send to the body.

People with HCM also have an abnormal arrangement of heart muscle cells (myofiber disarray). This can trigger abnormal heartbeats (arrhythmias) in some people.

What happens once you have hypertrophic cardiomyopathies?

People with HCM often don't have any symptoms and can lead normal lives. However, in a small number of people, HCM can lead to life-threatening abnormal heart rhythms (arrhythmias).

Symptoms of HCM may include:

  • Chest pain, especially during exercise.
  • Fainting, especially during or after exercise.
  • Shortness of breath, especially during exercise.
  • The sensation of rapid, fluttering, or pounding heartbeats (palpitations).
  • An abnormal sound from your heart, which a doctor might hear while listening to your heartbeat.

It's important to get diagnosed and appropriate care. See your doctor if anyone in your family has HCM or any of these symptoms.

Call 911 or your local emergency number if you have any of the following symptoms for more than a few minutes:

  • Chest pain.
  • Difficulty breathing.
  • Rapid or irregular heartbeat.

What causes hypertrophic cardiomyopathies?

HCM is usually passed down through families (inherited). If you have a parent with HCM, you have a 50% chance of having it. Parents, children, or sisters and brothers of a person with HCM should ask their doctors about screening for the disease, even if they don't feel symptoms.

What types of tests are used to diagnose hypertrophic cardiomyopathies?

Your doctor might order tests to diagnose HCM or rule out other conditions that can cause similar symptoms. Common tests include:

  • Echocardiogram: An echocardiogram uses sound waves (ultrasound) to see if your heart's muscle is abnormally thick. It also shows how well your heart is pumping blood.

  • Stress test: Sometimes, an echocardiogram is done while you exercise, usually on a treadmill.

  • Electrocardiogram (EKG): Sensors (electrodes) attached to adhesive pads are placed on your chest and sometimes legs. They measure electrical signals from your heart. An EKG can show abnormal heart rhythms and signs of heart thickening. In some cases, a portable EKG, called a Holter monitor, is needed. This device records your heart's activity over one to two days.

  • Cardiac MRI: A cardiac MRI uses powerful magnets and radio waves to create images of your heart. It shows your heart muscle and how your heart and heart valves work.

What types of treatments and procedures are used to treat hypertrophic cardiomyopathies?

The goal of HCM treatment is to relieve symptoms and prevent sudden death. Your treatment depends on how bad are your symptoms. You and your doctor will decide on the most appropriate treatment.

Medication: Medications can help reduce how strong the heart muscle squeezes and slow the heart rate so that the heart can pump blood better. Medications may include:

  • Beta-blockers.
  • Heart rhythm drugs.
  • Calcium channel blockers.
  • Blood thinners to prevent blood clots if you have atrial fibrillation.

Surgery: Different surgeries and procedures are available to treat HCM. They range from open-heart surgery to put in a device, a little machine, to control your heart rhythm. They include:

  • Septal myectomy: This open-heart surgery may be recommended if medications don't work. It involves removing part of the thick wall of muscle (septum) between the heart chambers. This improves blood flow and reduces the backward flow of blood (mitral regurgitation).

  • Septal ablation: This procedure destroys the thickened heart muscle with alcohol. The alcohol is injected into the artery supplying blood to that area. Possible complications include disrupting the heart's electrical system. That requires the implantation of a pacemaker.

  • Implantable cardioverter-defibrillator (ICD): An ICD is a small device put into your chest that monitors your heartbeat. It delivers electrical shocks to restore a normal heart rhythm.

What can I do to support my health when I have hypertrophic cardiomyopathies?

Since hypertrophic cardiomyopathy can range from a condition that never affects health to one that requires heart transplantation, there's no single way to live with it. The good news is that most people have no or minimal symptoms and can lead a normal life with treatment and follow-up.

Three keys to living well with it are knowledge, vigilance, and planning. Here are a few general guidelines:

  • Know the warning signs: Fainting, feeling dizzy, finding yourself unexpectedly short of breath, feeling your heart race, or chest pain that doesn't go away may be signs of trouble.

  • Make a plan: Talk with your doctor about whom to call if you are experiencing warning signs. Put your emergency numbers on the refrigerator or by the telephone. Assemble your medical records and put them where they can be grabbed easily.

  • Be healthy: Reduce salt, saturated fat, refined grains, sugary beverages, and alcohol from your diet. Light to moderate exercise is perfectly fine, even healthy. People with HCM should not participate in competitive sports or intense activities that strain the heart, such as weight lifting. If you're a competitive athlete with HCM, you should be screened once a year. Adults who don't compete in athletics can be screened every five years.

Why choose Presbyterian for hypertrophic cardiomyopathy treatment?

Presbyterian’s Pediatric and Congenital Cardiology team has many different options to help you manage your or your child’s heart condition. The team performs various diagnostic tests and procedures to help form an accurate diagnosis and create individualized treatment plans. Depending on the type of heart condition your child has and its underlying cause, the team can recommend a wide variety of treatment options. Our pediatric cardiologists, pediatric interventional cardiologists, and pediatric cardiovascular surgeons work closely together for cases in which cardiac repair or surgery is the best treatment option.