Heart valves

Heart Procedures and Hemophilia

With the proper precautions, cardiac procedures can be successful in men with hemophilia
Author: Stephanie Watson

William “Pete” Johnson is a self-professed workaholic. Throughout his sales and management career in Philadelphia, he would easily clock 60 to 70 hours a week on the job. Turning 65 didn’t slow him down a bit.

Even recurrent chest pains and shortness of breath, which his doctors determined to be the result of a heart valve problem, didn’t keep him from work. But then one weekend, while visiting with family, his symptoms worsened. “I felt like a bolt of lightning struck my chest,” Johnson says. He knew immediately that he was having a heart attack.

After he was transported to the nearest healthcare facility, Frankford Hospital in Philadelphia, doctors confirmed Johnson’s diagnosis. His treatment was more complicated than that of the typical heart attack patient, though, because the hospital was ill-equipped to deal with a person with a bleeding disorder—and one who also had HIV.

Johnson was moved to Thomas Jefferson University Hospital in Philadelphia, where he remained for about a week. His doctors determined that the damage to his heart was severe enough to require open-heart surgery—a daunting prospect because of his severe hemophilia A. Even though the doctors assured him that he was in the best possible hands, Johnson says his surgeon had never before operated on someone with a bleeding disorder. “The only thought I had was, ‘Would I live?’” he says.

Performing open-heart surgery on someone with hemophilia is no easy task. It involves significant collaboration between the hematologist and cardiologist before, during and after the surgery. People with hemophilia typically need to receive continuous clotting factor infusion throughout the operation to prevent uncontrolled bleeding. They also need to be carefully monitored for bleeding issues after their procedure.

Johnson survived the six-hour surgery, but there were complications. He was accidentally given an antiplatelet drug, but was taken off it before any serious health repercussions could occur. He had fluid in his lungs, which needed to be drained. He also had some bleeding about 10 days after surgery, which might have been a result of the clopidogrel he was taking to keep open a blocked artery that could not be surgically bypassed. Johnson remained at the hospital for two months while he recovered.

Although the American Heart Association recommends aspirin therapy to prevent heart attacks, it should be avoided by people with bleeding disorders due to its blood-thinning effects. After Johnson’s surgery, his doctors decided not to put him on aspirin therapy, but they had no standards of care or guidelines to use in making their decision. “There’s no good guideline. We don’t know what to do for people with hemophilia,” says Jamie Siegel, MD, Johnson’s hematologist and director of the Cardeza Foundation Hemophilia Center at Jefferson Hospital. This lack of clear-cut guidance for heart patients with hemophilia underscores the need to educate people about their heart disease risk early on, she says.

Silent Jolt

For Richard Johnson (no relation to Pete), 56, of Fort Mill, South Carolina, there was no bolt of lightning. He found out about his heart problems while undergoing dialysis for kidney failure. A blood test revealed an abnormality that convinced his doctor to send him to a cardiologist.

At Thomas Jefferson University Hospital, Johnson’s cardiologist delivered some unexpected news. “They said I’d had three heart attacks,” he says. “But I didn’t feel a thing.” Although his doctors were reluctant to operate, several blockages in his arteries made open-heart surgery a life-or-death proposition.

Johnson, who has hemophilia A, underwent surgery shortly before his 50th birthday in 2001. His doctors infused him with factor to reduce the risk of severe bleeding during the operation. Nonetheless, the experience was frightening for him. “I was so nervous,” Johnson recalls. “My wife was there, and so were my aunt and my father and brothers. We were all concerned.”

The surgery went off without a hitch. But immediately afterward, Johnson had bleeding in his lungs. He was rushed into another operating room, where doctors ­performed a procedure to stop the bleeding. It took a few days for his lungs to be fully drained. In the end, Johnson was sore from his surgery and emotionally spent, but alive.

Facing Angioplasty

Wayne Cook, 46, was working as a welder at General Electric in upstate New York when he had his first heart attack in 1999. “I started to sweat and I turned gray,” he remembers. Cook was rushed to Ellis Hospital in Schenectady, New York, where doctors confirmed his heart attack with an echocardiogram, but were unfamiliar with treating patients with hemophilia B. He was referred to a second hospital, Albany Medical Center, where doctors evaluated their options.

The possibility of undergoing surgery is daunting for anyone, but it’s an especially difficult decision for people with hemophilia, according to Harry Dunn, MD, Cook’s hematologist at New York Oncology Hematology, P.C. “If you have hemophilia, the interventions are with medications that make you more prone to bleed. If we want to put a catheter into the heart, the first thing the cardiologist is going to ask is, ‘Can we do this?’” Dunn says. Ultimately, Cook’s doctors decided that he needed a procedure called angioplasty, which uses a balloon catheter to open blocked arteries. Small metal mesh tubes called stents were placed in Cook’s arteries to keep them open. Though it isn’t open surgery, angioplasty did carry risks for Cook because he had to be given blood thinners to prevent thrombosis, or blood clot formation.

“They told me there was a high risk of complications with the bleeding, and a high risk of dying,” Cook remembers. “I was awake for the whole thing, and I can remember them telling me, ‘Hang on tight; everything is going to be all right.’” The doctors kept his factor at 100% to avoid bleeding complications. As soon as the procedure was over, Cook could begin to feel the pressure lift from his chest.

The angioplasty went well, but then a difficult decision ensued: whether to put Cook on medication to prevent blood clots. Antiplatelet medications and aspirin help the stents stay open and prevent thrombosis. The downside is that these drugs can exacerbate existing bleeding issues in those with hemophilia. After carefully weighing the risks versus the benefits, Cook’s doctors started him on clopidogrel immediately after surgery. After a couple of weeks, they switched him to a full-strength daily aspirin regimen; after six months, the dose was cut down to a daily baby aspirin. “In someone with hemophilia, there is increased risk [of bleeding], but then again, heart attacks kill people,” says Mark Nelson, MD, MPH, FACC, Cook’s cardiologist. He is a specialist in the prevention and treatment of cardiovascular disease at Capital Cardiology Associates in Albany, New York. “We could have decided not to proceed as we did with Wayne. There’s a good chance he wouldn’t be alive today.”

To prevent future bleeds caused by the antiplatelet therapy, Cook was put on a prophylactic regimen of factor IX immediately after his angioplasty. He received 100% factor twice a week until he stopped taking clopidogrel, after which his dose was cut down to 50%. With his heart and bleeding problems under control, Cook resumed his normal lifestyle. Everything went well until April 2007. He had been working long hours and traveling often; “I was burning the candle at both ends,” he says. While getting ready to leave for a business trip, Cook experienced chest pains again. “I didn’t think much of them,” he says. “I thought they were anxiety pains because I was traveling an awful lot.”

A couple of days later, his condition worsened. Cook’s wife insisted he go to the hospital. The staff discovered that he’d had a second heart attack. Angioplasty was performed to insert another stent, and he was put on clopidogrel for a month while taking extra factor to keep his blood stabilized. “Every time I infused, I was like a fountain,” Cook remembers. “My veins were starting to collapse a little bit. It was very scary.” He’s since been taken off the blood thinners, but continues to take a baby aspirin every day. He infuses factor product twice a week and takes a regimen of heart medications, a cholesterol-lowering drug and high blood pressure medications to control his heart disease risks. Although Cook knows there is a risk with taking aspirin, he’s willing to take it.

After Surgery

Today, Pete Johnson is mindful of his heart and more careful about his health habits. Though he grumbles about his restrictive salt-free diet and still needs to receive oxygen to breathe fully, he feels better than he did before his heart surgery. From now on, he says, he’ll take it easier. “I’m anxious to get back to work, but I don’t have the strength to work 60 to 70 hours a week anymore,” he says. “I might have to slow down or work via telephone.”

When Cook’s doctors warned him that a third heart attack would probably be his last, he made some drastic lifestyle decisions. He changed his diet, stopped smoking, dropped about 35 pounds and started to control the stress in his life by prioritizing his responsibilities and cutting out any nonessential activities. “I am in great shape,” he says. “As far as my heart goes, I feel like a million bucks.”

For Richard Johnson, recovery from open-heart surgery was surprisingly quick. “A month later, I felt like Superman,” he says. “I felt a lot better, a lot younger.” Johnson stopped smoking and lost weight on his doctor’s orders. Despite having kidney failure, HIV, heart problems and hepatitis C, “I’m still here,” he says.

Today, as men with hemophilia are living longer, thanks to improved treatments, their doctors have to help them contend with the risks of middle age, including heart disease. But, as these three men have shown, it is possible for people with hemophilia to be successfully treated for cardiac conditions. “None of them expected to live this long,” says Siegel, Pete Johnson’s hematologist. “It’s amazing how well these men have done.”