Hypertrophic Cardiomyopathy (HCM): Not Just Athletes

Hypertrophic cardiomyopathy – or HCM – is when the heart muscle becomes unusually thickened and cannot pump blood as well as it should. HCM is the leading cause of sudden death in athletes, but it can happen to non-athletes too. We recently sat down with George Lolay, MD, a cardiologist at Franciscan Health Indianapolis, to learn more about HCM.

More Common Than Previously Thought

HCM occurs in about 1 in every 500 people. “However, recent literature says it may actually be much more common and happen in as many as 1 in 200,” Dr. Lolay said.

As the leading cause of sudden death in athletes – people considered much healthier than the general population – there’s a misconception that HCM occurs more often in athletes. However, that’s not the case.

Dr. Lolay explains that there aren’t any gender, race or occupational differences in people who develop HCM.

The Causes Of HCM

In most cases, a genetic mutation passed down in families causes HCM. But in a smaller number of people, HCM develops without any family history.

Signs and Symptoms Of HCM

In young people, the only symptom of HCM is most often death – either during sleep or physical exertion (such as high-intensity workouts or contact sports). If a person is older and develops the condition, they are less likely to experience sudden death as the initial symptom. Their symptoms may include the following:

• Atrial fibrillation (AFib)
• Chest pain – especially during physical exertion
• Dysrhythmias (abnormal heart rhythm)
• Heart palpitations
• Shortness of breath (due to heart failure)

Another condition that causes thickening of the heart muscle – stiff heart syndrome – can cause similar symptoms. However, stiff heart syndrome differs from HCM and occurs when the heart muscle thickens in response to high blood pressure. This can lead to shortness of breath and fluid buildup in the lungs and legs.

Screenings For HCM

If you have a family history of sudden unexplained death or a family history of the gene mutation that causes HCM, you should undergo an annual screening echocardiogram (heart ultrasound). You should also encourage your first-degree relatives (parents, siblings and children) to have genetic tests and annual screening echocardiograms, if recommended based on your genetic tests.

Dr. Lolay stresses that a screening echocardiogram each year is critical. If you carry the gene mutation, the condition can develop suddenly – even if your prior year’s screening echocardiogram showed no sign of the disease.

You Can’t Prevent Or Reduce Your Risk Of HCM

If you have the gene mutation that causes HCM, no preventive measures will prevent it. However, your annual screening echocardiogram can ensure the condition hasn’t progressed. If you have developed hypertrophic cardiomyopathy, your annual screenings allow your heart doctor to begin a treatment regimen as soon as possible.

Athletes With HCM Should Take Extra Care

Dr. Lolay explains that researchers have firm recommendations for athletes with HCM.

“Experts say that because it’s an unpredictable disease, athletes should not participate in competitive sport,” he said.

At a minimum, Dr. Lolay advises that anyone diagnosed with HCM should avoid intense cardio workouts and contact sports. They should also work with their cardiology care team on personalized recommendations if they want to participate in recreational sports.

Treatments For HCM

“The treatment for HCM depends on how the disease affects your heart,” explains Dr. Lolay.

Your HCM treatment plan will consider how the condition affects your body and what other health conditions you may have.

• If you have AFib, you’ll work with an electrophysiologist to manage that condition. Your specialist will talk to you about how to prevent strokes with AFib, and you may be on an anticoagulant (medicine to prevent blood clots). If your mitral valve (opening between the two left areas of the heart) is affected, you may need surgery to correct that.
• If you have heart failure – including shortness of breath – the standard treatment for HCM is a surgical procedure called septal myectomy. During this procedure, your surgeon removes a portion of the thickened heart muscle. After a septal myectomy, a person is not cured of HCM, but they can have a normal life expectancy.
  • If you aren’t a candidate for surgery, your cardiologist may recommend an ethanol ablation. During an ethanol ablation, your specialist induces a mild heart attack in a controlled setting such that the extra muscle on your heart doesn’t work.
• If you meet certain high-risk criteria, such as a family history of sudden death and heart muscle thickness over 30 millimeters, your cardiologist may recommend an implantable cardioverter-defibrillator (ICD) to help manage your HCM. An ICD sends small electrical shocks to your heart to restore a regular rhythm during episodes of lethal heart rhythms.

Dr. Lolay notes that there is a new medication called mavacamten (Camzyos) that the U.S. Food & Drug Administration (FDA) has recently approved for use. “Researchers continue to explore how this new medication fits into the HCM treatment plan before the care team explores surgical options for a patient,” he said.

Find A Franciscan Provider

If you have a family history of sudden, unexplained death or HCM, schedule an appointment with a member of our Heart Care team to get started on your annual screening regimen.