Hypertrophic Cardiomyopathy (HCM) Treatment: What are the Available Options?

Once a diagnosis is established, there are much different treatment approaches your healthcare team may recommend based on the severity of your condition, these may include the following:

• Lifestyle adjustments
• Medications (ex. beta-blockers)
• Surgery
• Pacemaker (a small device implanted in the patient’s chest to control heartbeats)

Dr. Aeshita Dwivedi, a cardiologist at Lenox Hill Hospital/Northwell Health, points out "It’s also important that you remain active, which means moderate activity. A lot of patients with hypertrophic cardiomyopathy are restricted from doing strenuous activity because it can actually make their condition worse, but it’s important to still say active and can consider a supervised exercise program to remain active as well."

For some people, lifestyle adjustments may be enough to get their symptoms under control and prolong life. For those with advanced HCM, your doctor may suggest certain medication regimens, a surgical approach, or even a combination of different methods. The treatment route will depend largely on the severity of the disease as well as the patient's overall health status.

Dr. Dwivedi adds "The other important thing is to have healthy habits, excessive alcohol intake, or excessive stress on the body in terms of physical or emotional stress can also worsen the condition. So it’s important to maintain an overall healthy lifestyle, including a healthy diet and remain moderately active."

Treatment of HCM with Medication

"In some cases, one can use lifestyle modification [in] those that are asymptomatic (without symptoms) or less symptomatic," Dr. Philip Weintraub, a cardiologist at NYU Langone Health, tells SurvivorNet.

"In the more severe cases, one needs to bring in an arsenal of medications. In the past, we have used beta blockers, [which] are drugs that block adrenaline from the body, with the thought process being that if you could decrease the contractility of the left ventricle, you will decrease the amount of obstruction that occurs when the blood is being pumped out of the heart."

Dr. Weintraub also pointed to another type of drug called negative inotropes, which can be used to decrease the workload of the heart by decreasing the strength of the heart's contraction. "The theory behind them is that if you could make the heart function at a decreased rate, then you're going to wind up with less obstruction," he explains.

Treatment of HCM with Surgery

Hypertrophic cardiomyopathy may be treated with a surgical option called a septal myectomy, which is meant to thin out the wall between the left and right sides of the heart.

"You would have to surgically enter the left ventricle and 'carve out the area of the ventricle that is causing this particular problem," Dr. Weintraub explains. Because this is considered an open-heart surgery, it is extremely important to discuss the risks vs. benefits of undergoing such procedure beforehand with your healthcare team.

There are also minor procedures available, which may not require a specialized surgeon, that can treat the disease. One of those options is implanting something called a pacemaker, which is a device that can help the heart pump blood more efficiently.

Your doctor may also recommend something called alcohol septal ablation, which can be used to thin the heart's thickened and enlarged septum. "The belief is that the alcohol will cause the heart muscle to die," Dr. Weintraub adds. "So, it will not be allowed to express itself in the outflow tract."

Newer Treatment for Hypertrophic Cardiomyopathy

In April 2022, the Food and Drug Administration (FDA) approved a new drug treatment called Camzyos (mavacamten) for people with certain types of obstructive HCM.

"Camzyos (mavacamten) is an agent that will reprogram the muscle fibers of the heart," according to Dr. Weintraub. "These are the muscle fibers, and they will downscale them to the point where they do not produce as much muscle, which is contrary to what is necessary and what develops in hypertrophic cardiomyopathy."

"This drug has been studied and it appears to be quite effective in improving the quality of life where people who experience heart failure generally can move to a lower class in terms of how symptomatic they are."

Treatment Plans – Sometimes it Takes Trial & Error

The FDA approval of Camzyos (mavacamten) is for people with symptomatic New York Heart Association (NYHA) class II-III obstructive hypertrophic cardiomyopathy.

The NYHA class system provides a simple way to classify the extent of heart failure by breaking down severity into four separate categories:

• Category I
• Category II
• Category III
• Category IV

Category I. is for patients who have very little or no symptoms, while Category IV. is for people who experience extreme symptoms, limiting their everyday functioning and physical abilities.

Dr. Weintraub explains that research on this new drug shows that it is generally well tolerated, but HCM patients need to be mindful of associated side effects as well as drug interactions.

"It has been fairly well tolerated, but one must be careful to realize concomitant diseases in the body and also to protect against drug interactions," he says. "You do not want to take one thing, make your condition better, and at the same time, take something else and make it worse, so a lot of it is through trial and error as well as titrating doses up to an acceptable level."

Be an Active Advocate for your HCM

It is important to be an advocate for your health and wellness, especially if you have been diagnosed with HCM. Whether you suffer from symptoms or remain asymptomatic, it is beneficial to know all the current treatment options that may be available to you.

Be sure to have continuous communication with your healthcare team to ensure your condition is under control and not worsening. Sometimes, treatment plans change or new medications may come to market that has shown promising results in clinical trials. Staying educated on your condition as well as novel treatment options will help you be informed and improve your quality of life.

There are support groups available that can also help you stay educated and support you during your journey of living with HCM. Wellness programs, genetic testing resources, finding care, and counseling are a select few of the many offerings of nonprofit organizations, like the Hypertrophic Cardiomyopathy Association.

No one should have to cope with their condition alone. Take advantage of the resources available to you as they will help you understand the disease and keep you up to date on any treatment guidelines changes.

Moving Forward – Questions to Ask Your Doctor

• Does my HCM require treatment?
• What kind of lifestyle adjustments should I make?
• How is my disease classified?
• Should I consider medication or surgery?
• Am I a good candidate for Camzyos (mavacamten)?
• Are there any clinical trials available to participate in for novel treatments?

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