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Johns Hopkins Health - Take Heart

Winter 2011
Issue No. 11

Take Heart

Date: January 20, 2011

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Thirty-year-old John Campanella, who’d been happily married for a mere three months, felt as though he’d been handed a death sentence.

“I was in shock,” he recalls of the day in November 1997 when his doctor broke the news that he had arrhythmogenic right ventricular dysplasia, or ARVD for short. This sneaky heart condition had struck down his father, Joe, a former lineman for the Baltimore Colts who later became the team’s general manager, in his mid-30s during a vigorous game of handball. The family blamed his father’s death on a massive heart attack until Campanella’s sister, Carrie Campanella Becker, died just as young, at age 36, and just as suddenly, after riding a horse on the family farm.

Now Campanella, a former college lacrosse player, knew that ARVD stalked members of his athletic family, causing sudden cardiac death in the prime of their lives. He couldn’t help but wonder, will I be next?

A Stealthy Stalker
Each year, sudden cardiac death, also known as sudden cardiac arrest (SCA), hits more than 200,000 Americans like a lightning bolt. Many never suspect they have hidden heart disease—then die within minutes of the first symptoms.

“It’s not uncommon for sudden cardiac death to occur in someone who is apparently healthy—a 50- or 55-year-old vigorous man or woman who has sudden cardiac death with little or no warning,” says cardiologist Hugh Calkins, M.D., director of the arrhythmia service for Johns Hopkins Medicine.

Although SCA victims appear perfectly healthy, looks deceive. Ninety percent of adult victims had two or more major coronary arteries narrowed by fatty buildup, while scarring from a prior heart attack is found in two-thirds of its sufferers, according to the American Heart Association.

“While a heart attack is different from sudden cardiac death, the typical risk factors for a heart attack, like high blood pressure, smoking, sedentary lifestyle, elevations in blood lipid levels, diabetes and obesity, are all relevant to sudden cardiac death,” says Gordon Tomaselli, M.D., director of cardiology at The Johns Hopkins Hospital.

In those startling news accounts of star high school or college athletes who suddenly drop dead, a heart abnormality is the likely culprit. Adrenaline courses through the body during intense physical or athletic activity and triggers sudden cardiac death in an abnormal heart. During most sudden cardiac arrests, the electrical impulses in the diseased heart become rapid (ventricular tachycardia) or chaotic, or both (ventricular fibrillation). This irregular heart rhythm, also known as an arrhythmia, causes the heart to suddenly stop beating effectively.

Subtle Symptoms Never to Ignore
In 1967, no one detected warning signs that John Campanella’s father was seriously ill. On a brisk winter’s day, Joe ricocheted like a pinball across the handball court during a fiercely competitive game with Don Shula, then coach of the Baltimore Colts, and two other friends. All of a sudden, Joe staggered a few steps and muttered, “I’ve got to stop.” That’s when he collapsed; immediate attempts to revive him failed. Nearly 30 years later, Campanella’s sister, Carrie, didn’t suspect a connection when she started seeing a local doctor after experiencing fainting spells. After all, she suffered from a usually benign condition that caused her blood pressure to drop when she stood.

But fainting spells, or syncope, while often benign, may be one of the warning signs of sudden cardiac death. “Any fainting spell, particularly one that seems unexplained, should be investigated by a physician,” says Joseph Marine, M.D., director of electrophysiology at Johns Hopkins Bayview Medical Center. Although even healthy people can feel lightheaded or weak after standing quickly or for long periods, Marine warns that blackouts that occur suddenly or during exertion or exercise are red flags, especially when accompanied by a racing heart, shortness of breath, or chest tightness or pain.

Marine recommends also being on the lookout for heart attack warning signs, including chest discomfort—squeezing or tightness under the breastbone or in the upper abdomen that may radiate down the left arm and up into the neck—or feeling especially winded or fatigued after normal exertion.

“Even if they’re fairly mild, these are potentially serious symptoms that may signal a problem with blood flow to the heart,” Marine says. “And a problem with blood flow to the heart is the leading cause of sudden cardiac arrest in adults.”

Screen Savers
Meanwhile, it pays to delve into your family history, because people can inherit conditions that make them prone to arrhythmias, placing them at increased risk for sudden cardiac death. Examples of inherited disorders include ARVD, a progressive condition in which the muscle of the right ventricle is replaced by fat and fibrosis, which causes abnormal heart rhythms; and long QT syndrome, or LQTS. Because of problems with tiny electrical pores on the surface of the heart muscle cells, LQTS can cause sudden, uncontrollable, dangerous heart rhythms.

Thanks to advances in screenings available at Johns Hopkins, it is possible to buck a genetic predisposition and halt the progress of cardiovascular disease. Tomaselli says screenings include a detailed family and personal history, a physical examination and diagnostic tests such as:

  • An ECG, or electrocardiogram. This simple, painless test records the heart’s electrical activity.
  • An echocardiogram. During this gentle test, sound waves create pictures of your heart, giving your doctor information about the size and shape of your heart and how well its chambers and valves are working.
  • An electrophysiology study. Doctors use cardiac catheterization to record how your heart’s electrical system responds to certain medicines and electrical stimulation.
  • A CT (computed tomography) angiogram. This enables doctors to evaluate blood vessels and look for signs of atherosclerosis.
  • Genetic screening. “We have an excellent genetic counseling program in our Center for Inherited Heart Disease that we recommend as part of genetic screening,” Marine says.

“We’re doing some of the leading research in the United States related to genetic and imaging predictors of sudden death,” says Robert Weiss, M.D., director of the Donald W. Reynolds Cardiovascular Clinical Research Center at Johns Hopkins. “One of the new things we’re looking at is the use of MRI [magnetic resonance imaging] to identify scar tissue that’s interspersed with normal heart tissue. That may be a very strong predictor for sudden death.”

The Beat Goes On
Once tests showed that John Campanella had ARVD, he had surgery to help control his heart rhythms.

Now, at age 43, he enjoys a full life as a senior managing director of a commercial real estate firm in Washington, D.C., and as a husband and father of 11-year-old Caitlyn and 8-year-old Jack. He has given up competitive games of basketball and high-impact running in favor of milder forms of exercise, such as biking, golf and yoga. But that’s a small price to pay for the joy of watching his children grow up.

“The happiest day in my life was when their genetic tests for ARVD came back negative,” Campanella says. “I knew I wouldn’t have to be watching and wondering every day, will they be running down the stairs or bouncing on the trampoline and then have an arrhythmia and die?”

Instead, Campanella and his wife, Kathy, exuberantly cheer for Caitlyn and Jack as they play lacrosse and basketball for their school teams. And he can’t help but notice how his father’s legendary athleticism and competitive spirit live on.

It’s Not Just a Man’s Disease
If you’re a woman who thinks that heart disease is more likely to endanger the men in your life, think again. The American Heart Association reports that more women die each year of heart disease than men.

As women age, their risk of heart disease rises, sparking controversy over estrogen’s exact role (if any) in protecting the heart. Until that controversy is ironed out, cardiologist Pamela Ouyang, M.B.B.S., director of the Johns Hopkins Women’s Cardiovascular Health Center, lists four action steps women need to take today to protect their hearts:

  • Stop smoking. “For women who smoke, their life expectancy is 14 years less,” Ouyang warns.
  • Focus on fitness. “Across our population, women do much less physical activity than men,” she says.
  • Know your numbers. Don’t assume that your blood pressure and cholesterol levels are good—ask the next time you’re in the doctor’s office.
  • Call 911 immediately if you think you’re having a heart attack. Don’t wait for someone to give you a ride to the hospital.

Turning Tragedy into Triumph
When John Campanella was diagnosed with a potentially fatal heart condition—arrhythmogenic right ventricular dysplasia, or ARVD—in November 1997, he and his wife, Kathy, became the engine behind Johns Hopkins School of Medicine’s mission to unravel the mysteries surrounding a disorder that strikes young athletes. The couple have raised millions of dollars for research and established a Web site that has drawn patients from all around the world to Johns Hopkins for evaluation and treatment. Most exciting of all, researchers have pinpointed the gene responsible for ARVD.

To find out more, visit

Be Heart Healthy
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