Mavacamten (Camzyos) for Obstructive Hypertrophic Cardiomyopathy (HCM)

How Mavacamten (Camzyos) Treats Obstructive Hypertrophic Cardiomyopathy (HCM)

How Mavacamten (Camzyos) Treats Obstructive Hypertrophic Cardiomyopathy (HCM)
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If your current medications aren’t doing enough to manage your obstructive hypertrophic cardiomyopathy (HCM), your cardiologist may recommend mavacamten (Camzyos). The drug helps your heart relax, reducing symptoms such as fatigue and shortness of breath.

Mavacamten works differently from other medications for this genetic condition. Talk to your doctor about whether it can make a difference for your HCM symptoms.

How Mavacamten (Camzyos) Works

Mavacamten is a cardiac myosin inhibitor that helps the heart squeeze less forcefully, addressing a root cause of symptoms of HCM, in which the heart muscles are thickened and have difficulty pumping blood effectively. The medication reduces the interaction between actin and myosin, two proteins that cause the heart to contract.

“It lowers heart muscle contraction at the molecular level, allowing the heart to pump more efficiently and relax more effectively, which can relieve obstruction and improve symptoms,” says Mohammed Makkiya, MD, the director of the HCM clinic at VCU Health Pauley Heart Center in Richmond, Virginia.

Mavacamten comes in an oral capsule that you take once a day. The daily dosage usually starts at 5 milligrams (mg) and gradually increases every four weeks until you reach 15 mg per day.

Who Is Mavacamten For?

The U.S. Food and Drug Administration (FDA) has approved mavacamten for adults with obstructive HCM, in which the thickened heart muscle blocks or limits blood flow from the heart to the rest of the body. It hasn’t been shown to be effective for non-obstructive HCM.

Your doctor may recommend mavacamten if medications such as beta-blockers or calcium channel blockers haven’t done enough to manage your symptoms.

“These are often patients who are experiencing shortness of breath, chest pain, or exercise intolerance but who are not yet ready for or who may want to avoid an invasive procedure,” like surgery, Dr. Makkiya says.

Mavacamten is not for people who are pregnant or planning to become pregnant, though. It can cause serious birth defects for up to four months after the final dose.

Let your doctor know if you’re breastfeeding or if you have an existing medical problem such as an irregular heartbeat or a severe infection.

Benefits

Mavacamten can improve obstructive HCM symptoms such as shortness of breath and chest discomfort.

 That can give you more stamina during exercise and your everyday activities, improving your quality of life.

“Patient will say things like, ‘I used to have to stop at the top of a flight of stairs because of shortness of breath, but now I can walk three flights of stairs without stopping,’ or ‘I get less chest pressure or palpitations when walking around,’” says Sandeep Jani, MD, MPH, the medical director of advanced heart failure for the Baltimore region for MedStar Health.

These benefits usually build over time, Dr. Jani says, though your heart may start working more efficiently in just a week after starting mavacamten.

 Two-thirds of people who have taken mavacamten report no or minimal symptoms after 3.5 years on the medication.

Heart Failure Risk and the REMS Program

There is one notable risk associated with this drug: Mavacamten can increase your risk of heart failure.

“Because mavacamten decreases heart muscle contraction, there is a possibility that the heart muscle may overreact to it, increasing the risk of the heart becoming too weak,” Makkiya says.

Mavacamten is only available as part of a risk evaluation and mitigation strategy (REMS) program, which requires ongoing monitoring to ensure you aren’t developing heart failure. This makes getting a prescription more complex, as it requires you and your cardiologist to be enrolled in the REMS program.

Your cardiologist will:

  • Evaluate your heart failure risk with an echocardiogram 
  • Assess your existing medications to rule out interactions with mavacamten. Taking diltiazem, disopyramide (Norpace), ranolazine, rifampin, or verapamil with a beta-blocker can increase your risk of heart failure or heart-rhythm problems, for example.

  • Check your heart function with echocardiograms regularly. These are required to continue or increase mavacamten dosages.
Only 1 percent of mavacamten users in the REMS program have experienced heart failure.

“When prescribed appropriately within [REMS], mavacamten is very safe, and dose adjustments can prevent long-term problems,” Makkiya says.

Other Side Effects

Mavacamten’s side effects can vary. They include:

  • Dizziness (most common)
  • Fainting (most common)
  • Breathing issues
  • Chest pain or tightness
  • Dilation of neck veins
  • Fatigue and weakness
  • Irregular heartbeat
  • Reduced urine volume
  • Swelling in your fingers, face, feet, or lower legs
  • Weight gain

Warnings

Mavacamten isn’t for people who are pregnant or taking certain medications, so it’s important to discuss your risk factors with your cardiologist. Do not smoke or consume alcohol while taking mavacamten, as interactions may occur.

You should also avoid drinking grapefruit juice while taking mavacamten, as it can lead to too much of the medication in your blood, increasing the risk of heart failure.

The Takeaway

  • The medication mavacamten (Camzyos) can improve or eliminate symptoms of the heart condition obstructive hypertrophic cardiomyopathy (HCM), such as shortness of breath or chest pain, but the FDA has not approved the drug for non-obstructive HCM.
  • Mavacamten reduces the protein interaction that makes your heart contract with too much force, helping it work more efficiently.
  • Because taking mavacamten could increase the risk of heart failure, qualifying for a prescription requires regimented, regular checkups and an assessment of potential medication interactions.
  • Dizziness and fainting are mavacamten’s most common side effects.

Resources We Trust

EDITORIAL SOURCES
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chung-yoon-bio

Chung Yoon, MD

Medical Reviewer
Chung Yoon, MD, is a noninvasive cardiologist with a passion for diagnosis, prevention, intervention, and treatment of a wide range of heart and cardiovascular disorders. He enjoys clinical decision-making and providing patient care in both hospital and outpatient settings. He excels at analytical and decision-making skills and building connection and trust with patients and their families.
Marygrace Taylor

Marygrace Taylor

Author

Marygrace Taylor is an award-winning freelance health and wellness writer with more than 15 years of experience covering topics including women’s health, nutrition, chronic conditions, and preventive medicine. Her work has appeared in top national outlets like Prevention, Parade, Women’s Health, and O, The Oprah Magazine.

She's also the coauthor of three books: Eat Clean, Stay Lean: The Diet, Prevention Mediterranean Table, and Allergy-Friendly Food for Families. She lives in Philadelphia.