At age 17, John B. learned that he had a heart murmur. It didn’t cause him much grief for three decades. But as he approached age 50, his routine stress tests began showing some irregular results.
“I started feeling like I was out of breath a bit more often,” he says. “I’m a medicinal chemist, and I stand at a bench working all day. I thought that maybe I needed to get out and do more exercising.”
Over the course of the next decade, John’s symptoms kept getting worse. “I have two beautiful grandkids,” says John, now 63. “I used to roll on the floor and wrestle with them. Then it got to the point that playing with them got me tired right away.”
Ted Gutkowski, MD, John’s long-time cardiologist at CentraState Healthcare System, an Atlantic Health System partner, suspected that John was developing hypertrophic cardiomyopathy (HCM). It’s a congenital disease that causes the heart muscle to thicken and impede blood flow from the heart. In John’s case, his septum, the wall between the two lower chambers of the heart, was enlarged.
John needed care from a specialist, and Dr. Gutkowski referred him to sports cardiologist Matthew Martinez, MD, a nationally recognized HCM expert with Morristown Medical Center. “Dr. Gutkowski told me that they take care of the New York Jets, so they’re pretty good,” John said.
Exploring a brand-new treatment option
Dr. Martinez confirmed that John had a subtype of HCM known as obstructive HCM. “About two-thirds of all patients with HCM have obstructive HCM,” Dr. Martinez says. “That means that the increased muscle thickness narrows the tunnel where blood tries to leave the heart.”
In obstructive HCM, blood flow gets worse with exertion. “That leads to a faster heart rate, breathlessness, dizziness, chest pressure, and a feeling like you’re going to pass out,” Dr. Martinez says.
In the past, patients like John with obstructive HCM had relatively few treatment options. They could get open-heart surgery (called septal myectomy) to remove excess heart muscle. Or they could undergo a minimally invasive procedure (called alcohol septal ablation) that shrinks heart tissue using a catheter. John, who has underlying health issues, didn’t qualify for surgery but did consider ablation.
However, Dr. Martinez gave John a third, brand-new treatment option: mavacamten (Camzyos™), the first medication ever developed specifically for HCM. At the time, the medication was coming off a clinical trial and was pending U.S. Food and Drug Administration (FDA) approval. Morristown Medical Center was among the clinical trial participants.
“As a medicinal chemist, I was very much intrigued by this,” John says. “I said, I have to get this once it’s approved.”
In May 2022, mavacamten received FDA approval. The next month, John met with Dr. Martinez. “Guess what,” Dr. Martinez told him. “You’re going to be my first patient on mavacamten.”
Taken orally once a day, mavacamten gives patients a nonsurgical option for treating obstructive HCM. It works by reducing the amount of muscle contraction inside the heart.
“The way I explain it to my patients is to think of a whole group of rowers on a boat,” Dr. Martinez says. “Each time a rower rows, the heart muscle contracts. A normal heart has five rowers. An HCM heart has 10 rowers. Mavacamten tells some of those ‘extra’ rowers to stop rowing so the heart can squeeze less vigorously.”
Clinical trial data showed that mavacamten may help people with obstructive HCM delay surgery or avoid it altogether.
The Chanin T. Mast Center for HCM at Morristown Medical Center is the only site in New Jersey currently authorized to prescribe mavacamten.
Feeling better ‘after a couple of days’
John began his mavacamten treatment with a 5 milligram dose. “I felt better after a couple of days,” he says. “The symptoms I had—not being able to get a full breath—reduced significantly.” After four weeks of treatment, John learned that his pressure gradient—a measurement of muscle contraction inside the heart—had fallen from a high of 114 – 115 millimeters of mercury (mm HG) to 40 mm HG (lower is better).
As of October 2022, John was one of about two dozen patients undergoing mavacamten therapy at Morristown Medical Center. Those patients visit the Mavacamten Clinic at the HCM Center, where they receive echocardiograms on a routine basis and review results with their clinicians. John started with monthly visits and now visits the clinic once every three months.
“The clinic is fantastic,” John says. “I get my echo, Aleksandra (Novik), my nurse practitioner, comes in and reviews the results with me. Dr. Martinez always stops by as well. Then I leave with my next appointments already in hand. You don’t normally see that level of support in a doctor’s office.”
Today, life for John is better. He currently takes 10 mg of mavacamten once each day. His shortness of breath is significantly reduced. He can work in the yard of his Millstone Township house again. And a couple weeks ago, he had an impromptu dance competition with his grandchildren. “I could dance with them again!” he says.
“To me, mavacamten is a game-changer,” Dr. Martinez says. “Patients like John are reporting life-altering improvements in their exercise tolerance with no side effects. As a physician, I don’t think I’ve had anything as rewarding as this medication has been.”