The interventional cardiologist watched, transfixed, not quite believing what he was seeing but immediately grasping what it meant.
Christopher Cove had just completed a procedure designed to choke off the blood supply to a large tumor lodged in the heart of a 32-year-old nurse. Seconds after he finished the coil embolization, the tumor, like a monster in the movie “Alien,” had formed new blood vessels, vanquishing Cove’s efforts to starve it.
“I was very discouraged,” recalled Cove, assistant chief of the cardiac catheterization lab at the University of Rochester Medical Center in New York. “I wasn’t 100 percent convinced it would work, but I didn’t expect that.” Cove remembered what he had told his patient Jamie Arliss: “ ‘If it failed, we could try something else.’ Of course, I didn’t know what that was.”
For two years, a team of specialists had scrambled to figure out how to help Arliss, whose case was unlike anything they had seen. The first challenge, which took months, was identifying her tumor. The second was what to do about the growing, golf-ball-size mass. Doctors feared that removing the tumor could kill her.
Precedent was no guide: Arliss’s tumor was so rare that it had been reported only a handful of times in the medical literature, mostly after autopsies. She was desperate to avoid the only treatment that seemed viable — a heart transplant — and there was no guarantee a donor heart would become available in time.
Several weeks after the failed embolization last October, Cove’s chance interaction with a neurosurgeon resulted in a risky, last-ditch effort that the New York specialists say had not previously been reported.
“With most procedures you can quote a risk,” Cove said of the operation that he and Babak Jahromi performed in December. “I couldn’t quote any risk. But Jamie wanted to do it.” And time was clearly running out.
For four or five years beginning in her mid-20s, Arliss recalled, she had felt unaccountably tired. Doctors found nothing obvious and brushed it off as anxiety, she said. Arliss figured it was a consequence of working a lot of overtime as a licensed practical nurse and taking care of her husband and their young daughter.
In the summer of 2008, soon after she went back to school to get an RN degree, Arliss left her job in a Rochester area nursing home for a position in a doctor’s office. As part of her orientation, employees showed her how to use the EKG machine, which records the heart’s electrical activity, and they practiced taking readings on one another.
“Mine was most abnormal,” Arliss recalled. She showed the reading to the doctor for whom she was working, who thought it was probably the result of misplaced electrodes. But Arliss, a self-described worrywart, consulted her own doctor, who performed another EKG. After it revealed the same abnormality, she was sent for an echocardiogram, which inspects the heart using ultrasound. That test revealed a large mass that she was told might be an extremely rare cardiac cancer or a metastasis from a malignancy elsewhere in her body.
Arliss felt “freaked out,” she remembers, adding, “I just kind of did what they said.” After a full-body CT and bone scan were clear, doctors told Arliss they considered cancer unlikely but did not know what kind of tumor she had.
In February 2009, further tests at the University of Rochester’s Strong Memorial Hospital revealed a mass that appeared to be vascular — containing blood vessels — measuring 3.5 by 3.1 centimeters — about an inch tall and an inch wide. Cardiac surgeons expected it would turn out to be a common heart tumor that could be removed fairly easily.
A biopsy, performed a month later, revealed a stunning surprise: The mass was anything but ordinary, and its size and location rendered it inoperable. Arliss had an arteriovenous malformation tumor, which results from an abnormal tangle of blood vesssels; the tumor was being fed by the heart’s rich blood supply. Most AVMs occur in the brain and the lungs; they are virtually unknown in the heart and are usually far smaller than Arliss’s tumor, which was engulfing her left ventricle, the heart’s primary pumping chamber.
The cardiac surgeon who performed the biopsy “described it as a big rat’s nest of pulsating blood vessels,” Cove said.
The cause of AVMs, which are present at birth, is unknown, according to the National Institutes of Health. Many are found incidentally during CT scans or other imaging tests, often in people younger than 40.
After identifying Arliss’s tumor, doctors scoured the medical literature to see how best to treat it. The news was not good: There were no reported treatments, and the handful of cases cited were mostly autopsies.
‘A light went off’
Arliss was referred to a heart transplant surgeon while doctors monitored her, hoping to come up with a less invasive option before her condition became even more dire. In November 2009 surgeons published images of the rare tumor in the Journal of the American College of Cardiology, hoping they might generate clues about possible treatments from other doctors. None surfaced.
By the summer of 2010 Arliss was worse — so short of breath she could not walk more than three blocks without resting. The tumor was expanding, and surgeons worried it might be growing outside her heart, a fear later confirmed by an MRI. She was referred to Cove for a possible coil embolization.
A procedure developed in the 1990s to treat brain aneuryms, coil embolization involves threading small coils to a target spot through an artery in the groin. The coils are then released and spring into position, forming a blood clot designed to obstruct blood flow. The scar created after the clot forms is supposed to create a permanent seal.
Cove wasn’t sure it would work, but thought it was worth a try. “I did my own literature search and talked to doctors” around the country, who agreed that cutting off the main blood supply should kill the tumor. Arliss, he said, had a “gray, chronically ill appearance” and told him she was ready to try anything that might work.
Around the time the embolization failed, Cove met Jahromi, a staff neurosurgeon who was working temporarily in the cardiology suite during a renovation; the two discussed the case.
Cove knew that Jahromi used a new drug called Onyx, a substance referred to as medical superglue, as a form of liquid embolization to treat AVMs in the brain. “Basically a light went off, and I said, ‘I bet we can inject Onyx into the tumor,’ ” Cove recalled. Jahromi agreed, and both proposed the apparently unprecedented experimental treatment to Arliss.
“I said, ‘This has never been injected into a heart,’ ” Cove recalled telling Arliss. “She was very desperate to get something done, and she spurred us on.”
Arliss remembers feeling somewhat relieved that there was a plan to deal with “the time bomb in my body. It was very frustrating and hard knowing it was there and that doctors couldn’t do anything about it.”
On Dec. 9, heavily sedated but conscious, Arliss was wheeled into the cardiac cath lab for the painstaking procedure. Cove’s goal, he said, was to “fill the entire rat’s nest” with liquid Onyx, thereby depriving the tumor of its blood supply and hopefully killing it before it could sprout new blood vessels. “It’s like any alien,” Cove said. “If one little piece is left, it could grow.”
The theory was that the tumor and drug would gradually be reabsorbed. The hope was that the procedure would not damage Arliss’s heart — or kill her by triggering a massive heart attack.
Watching the progress using echocardiography, Cove and Jahromi needed to find the best access point to the center of the tumor, after determining which veins and arteries were feeding it. They discovered that the coil embolization had destroyed a major blood vessel, “so the Onyx had nowhere else to go,” Cove said. “Otherwise it could have gone in one side and out the other.”
After using the hospital’s entire supply of Onyx, which had to be injected slowly, they waited anxiously to see how Arliss would fare. Her vital signs were stable and her heart function was undamaged, but it would take weeks to see whether the treatment had worked or complications arose.
The first three days, Cove said, were anxiety-filled for the doctors. Arliss doesn’t remember much; Cove said she was in significant pain, probably as parts of the tumor died. Six weeks later, an echocardiogram showed shadows where the Onyx had been deposited. Since then the tumor has steadily shrunk and is one third smaller; Arliss said she is gradually feeling better and is back at work.
“Some days I still get very tired,” she said. “I would love to have this thing gone.”
Cove said he expects it will take a year for doctors to fully evaluate the effectiveness of the treatment and to see if the tumor disappears entirely. He and Jahromi, who presented the case at a recent scientific meeting, are collaborating on a paper about their novel treatment.
“I am very optimistic, actually,” he said several weeks ago, noting that he had seen Arliss for a follow-up appointment that day and was heartened by her improved appearance and energy level. “Everything in medicine is so mapped out and described. I’ve never done anything like this.”
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