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Heart

Hypertrophic cardiomyopathy affects young and old

Heart

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Hypertrophic cardiomyopathy is the most common genetic heart condition, but it’s not widely understood.

If you do the math, there are around 14,000 people in the Dallas-Fort Worth area who potentially have an enlarged heart muscle – called hypertrophic cardiomyopathy (HCM) – a potentially deadly condition.

Several prominent athletes have been affected by the condition over the years. Hank Gathers, a star college basketball player at Loyola Marymount University, collapsed during a game in 1990 and died from HCM. He was just 23 years old.

Although it is the most common genetic heart condition, affecting 1 in 500 people both young and old, HCM isn’t widely understood. Many people who have this condition don’t even realize it.

What is hypertrophic cardiomyopathy?

HCM is the abnormal thickening of the heart muscle. We usually see this thickening in the septum, the heart wall that separates the left chamber from the right chamber.

A normal septum is 1 centimeter wide, but for people with HCM the septum can be up to 3 centimeters wide.

Enlarged muscle in that area of the heart can cause many symptoms, including:

  • Shortness of breath
  • Lightheadedness
  • Dizziness
  • Chest pain

When the heart muscle thickens, it causes higher pressure inside the heart. Blood doesn’t flow into or out of the heart as it normally would and thus isn’t properly circulated throughout the body.

HCM can cause sudden heart problems in people in their 20s and 30s. These people may not experience any symptoms unless they exercise.

Older people with the condition, usually in their 50s or 60s, may develop symptoms over time. Other people with HCM never develop symptoms. There is still a lot we don’t know about the disease.

How is hypertrophic cardiomyopathy treated?

We can easily diagnose hypertrophic cardiomyopathy by taking a picture of the heart muscle using an echocardiogram. Once we’ve confirmed the condition, our first treatment option is medication. We assess the patient’s symptoms and prescribe drugs to treat them – usually beta blockers or calcium channel blockers.

UT Southwestern is one of several locations participating in a clinical trial aimed at identifying another kind of drug that could help patients with HCM.

If the patient can’t tolerate medication or it doesn’t alleviate the symptoms, our other options are surgery or a catheter-based procedure.

At UT Southwestern, we can offer patients an extended myectomy surgery if other treatment options don’t work.

During an extended myectomy, a heart surgeon accesses the trouble spot through the aortic valve in the heart and trims the muscle off the ventricle. Patients are connected to a heart-lung machine, which takes over the function of the heart while the surgeon operates. It’s a fairly complex procedure and takes about three hours.

Most patients who have surgery for HCM notice improved breathing right after the surgery. Just getting that blood circulating correctly can make a big difference. Usually these patients are in the hospital five to seven days and can go back to normal activity in four or five weeks.

UT Southwestern is the only medical center in North Texas – and one of just two in the entire state – equipped to perform extended myectomy surgery.

Advice for patients

HCM is not a rare condition, but it is also not very common. We know that people have a much higher risk of developing HCM if a relative has had it.

There are several things you can do to give yourself the best chance to avoid or manage this disease. Here are a few tips:

  • Don’t ignore symptoms.
  • Find out if anyone in your family has had HCM or its symptoms.
  • If you have symptoms or a family history, talk to your doctor about having an echocardiogram to see if there is a problem.
  • Consider genetic testing. A simple blood test will allow us to search for the gene mutation known to cause HCM.
  • If you have been diagnosed with HCM, ask your cardiologist if you should have surgery to manage the condition. Some patients are candidates for surgery and some are not.

The American Heart Association has encouraged patients who have HCM to be regularly monitored by a multidisciplinary program like we have here at UT Southwestern. Having that broad base of experience to rely on is in the patient’s best interest.

As a patient, it’s your choice where to receive medical care. If you have HCM symptoms, the most important step is to see a doctor. Depending on your treatment needs, you may want to ask your cardiologist about a referral.

Patients who come to UT Southwestern or are referred with HCM are given assessments of their personal risk annually. From there, we adjust treatments or consider different options.

We also evaluate the family’s risk for developing the condition. In about 50 percent of cases, we can confirm the disease is genetic because we’re able to identify with certainty a gene mutation. Our genetic testing program can provide valuable results to you and your loved ones.

For more information about our services, or to inquire about genetic testing, request an appointment or call 214-645-8300.