IN THE RIGHT PLACE AT THE RIGHT TIME

By LISA PEARSON , Staff Writer, BRISTOL PRESS 02/01/2004

BRISTOL, CN -- Robert Britton of Plainville headed down to Florida last year on vacation without a care in the world. Little did he know that by the end of his trip he would have had his heart jump-started twice and be diagnosed with a unfamiliar genetic heart disease.

"I was in perfect health," said the 52-year-old Britton, owner of an explosives company. "I had never had any problems except occasional high blood pressure because of stress-related problems with work."

Britton remembers the Saturday morning that his life changed. He and his wife had gone out to an antique car auction when he began developing chest pains. "I had never had chest pains before," he added.

But Britton got medical attention anyway, and felt better within about 10 minutes. On the way to the hospital in the ambulance, his heart stopped, and then it restarted on its own.

The doctor in Tampa told him that Britton did not have a heart attack and that his arteries were crystal clear. "It was strange," said Britton.

The Tampa doctor then spoke with a colleague across the country, suspecting that what the electrocardiogram was showing was a newly discovered disease called Brugada syndrome.

The doctor ordered an EP test, which checks the heart's electrical activity and whether there is any type of malfunction. This procedure is performed by going through a vein in the leg and into the lower chambers of the heart. With this procedure, doctors were able to recreate the same scenario once again, by stopping, then re-starting Britton's heart.

"There wasn't any pain. I just remember waking up and asking everyone what the hell had just happened," said Britton.

The doctors informed him that he did in fact have Brugada syndrome, and that he would need to have a defibrillator installed in his chest, which he would need for the rest of his life.

Brugada syndrome was discovered in 1992 by Pedro and Josep Brugada, who identified the disease, which prevents the sodium channels in the heart's electrical impulses to pass correctly.

Dr. Ramon Brugada, director of the Molecular Genetics Program at the Masonic Medical Research Laboratory in Utica, New York, and brother of Josep and Pedro, has been researching this disease since 1995, and believes it is possible that one in 5,000 Americans may have the syndrome, and that an estimated 20 percent of all sudden cardiac death in America may be cause by these genetic abnormalities in the heart.

"Don't ever think it's normal to have an unexpected, sudden death, like that of a young child or a healthy 40-year-old. It's never normal, unless the condition may be related to a very elderly person," said Brugada, who is familiar with Britton's case.

"When we got into the history of his family, we found that he had a couple cousins that had died of Sudden Infant Death Syndrome. It's just not normal for an infant to die suddenly."

Brugada syndrome is believed to be so close to SIDS that in Southeast Asia, the adult disease is also known as SUDS, or Sudden Unexpected Death Syndrome. Researchers at MMRL claim that Brugada syndrome is the second only to car accidents as the cause of death for young men -- accounting for approximately 1 in every 2,000 deaths.

Dr. Henry Ward, Britton's regular cardiologist, said that although he has been informed that the disease is prevalent in Asia, he doesn't necessarily agree with the numbers, and that he believes Brugada syndrome is one of the rarer diseases.

"These numbers are estimations. I don't know if you can pinpoint it," he said.

A number of area health professionals contacted for this article had never even heard of the disease, which is why Brugada is trying to get as much information to the public as possible.

"It's important to have your blood screened for genetic abnormalities, especially when a healthy member of your family dies suddenly," he said. "Wen need to get awareness of this disease out there."

Although there is no medicinal cure for this disease, sufferers can live a healthy life with an implanted defibrillator to jump-start the heart every time it stops. The device is checked regularly with software every three months.

Britton said the defibrillator doesn't bother him, although he does always know it's there. He claims that his life hasn't changed much, except in a few different ways.

"I can't use remote-controlled airplanes or cars, and I can't go through metal detectors. Oh, and I can't jump-start a car." If he does any of these, he risks sending a false trigger that will give him a sudden shock, which could then cause cardiac arrest.

"All I know, is that I was in the right place at the right time when all of this happened," added Britton, who also said that he is not sure if the same diagnosis would have been made had he been in Connecticut instead of Florida. "I'm glad I'm still healthy."

Brugada recommends that people with any history of sudden death in their family, or symptoms of frequent fainting, should seek the assistance of a physician immediately.

For more information on this disease, visit www.brugada.org, or www.mmrl.edu.

©The Bristol Press 2004