Monday, October 10, 2011

Some Clues to Detecting Potential for Sudden Death in Youth

Clinton High School Girls’ Tennis Coach, Derek Moisan, is seen with one of the school’s cardiac defibrillators. (MATT WRIGHT)
The sudden death of a seemingly healthy 12-year-old boy at a Holden soccer camp this past summer shocked camp workers and traumatized fellow campers.

The article below is reprinted from:

Wednesday, September 28, 2011
Screening for danger
By Karen Nugent TELEGRAM & GAZETTE STAFF Worcester, Massachusetts, USA

The youth, Joshua D. Thibodeau of Holden, had no obvious pre-existing health condition and his family had completed a medical waiver and everything else required to clear their son for participation in the private program. Tragically, he collapsed and died July 18 during a low-impact drill with 15 other campers.

Joshua’s cause of death, according to the state medical examiner’s office in Boston, was cardiac dysrhythmia complicating hypertrophic cardiomyopathy, an inherited condition that affects approximately 1 in 500 people. Every year, there are similar horrific stories of young people suddenly collapsing and dying during sporting events.

According to the American Heart Association, hypertrophic cardiomyopathy, or HCM, is the most common cause of death in athletes younger than 35, responsible for about one-third of deaths. The heart muscle fibers of those afflicted multiply rapidly, especially during adolescence, leading to an enlarged heart that could be double or triple the normal size.

Cardiac dysrhythmia is a term encompassing various types of irregular heartbeats — from annoying to life threatening. If such a beat happens while a person with HCM is exercising, the electrical system in the heart suddenly fails and a heart attack occurs. Usually those with HCM have no idea they have the condition until disaster strikes.

Can the condition be discovered beforehand via an electrocardiogram as part of a routine physical?

Some say yes, some disagree.

The American Heart Association’s current 12-item guidelines for athletes include a physical exam checking for heart murmurs, taking pulse rates, and blood pressure; and obtaining a clinical history on symptoms of heart disease such as chest pain, fatigue associated with exercise, lightheadedness, and high blood pressure. A family history checking for heart disease and premature deaths from heart problems is also required.

Although recommended by many doctors, the association does not require EKG screening because it is considered impractical, would be costly, and could lead to false positives that might disqualify children from joining sports programs, according to a 2007 updated article in the Journal of the American Heart Association on pre-participation screening for cardiovascular abnormalities in competitive athletes.

A statement last week from the American Heart Association says: “We have a carefully designed tool for recognizing at-risk athletes … if any of the 12 screening elements has a ‘yes’ answer, the participant would be referred for further cardiovascular examination, which may include an EKG. There’s no evidence that adding an EKG to the first line of athletic pre-participation screening would decrease death rates from SCD (sudden cardiac deaths) in young athletes. To determine whether adding an EKG to athletic pre-participation is reasonable, we need more data. A national registry for these events would go a long way to help us understand the true magnitude of the problem, and whether we need a different approach to prevention.”

The European Society of Cardiology, however, and the International Olympic Committee, several years ago changed their recommendations to include EKGs.

But a recent study at Stanford University study reported in the Journal of Pediatrics found EKG screening of young athletes to be ineffective.

The researchers selected 18 EKGs, including eight from patients with normal hearts and 10 from patients with any of six common heart abnormalities related to sudden cardiac death, including HCM. They were shown to 53 experienced pediatric cardiologists to see if they would make the correct diagnoses, and properly restrict or allow sports activities.

The cardiologists correctly identified 68 percent of the abnormal cases, but 32 percent of teenagers with abnormal EKGs were not detected. And of the cases identified as abnormal, 30 percent were normal. Also, 19 percent of patients who should have had exercise restrictions were not identified; and 26 percent who should have been allowed to exercise were restricted.

Dr. Darshak M. Sanghavi, chief of pediatric cardiology at the University of Massachusetts Medical School in Worcester, questions the Stanford study because of the small number of patients, and a lack of information in the published article. The EKGs themselves were not available for readers to look at, he pointed out.

“I was perplexed. And I am not exactly sure what happened there — maybe they were poorly trained cardiologists — because the preponderance of data shows that EKG screening does make a difference,” he said.

Dr. Sanghavi, while not speaking about Joshua Thibodeau’s case specifically, said an EKG should reveal hypertrophic cardiomyopathy, along with several other heart abnormalities. Using EKGs as a screening tool for young athletes, he said, “is a reasonable thing to do.”

“The way we do it now misses those problems,” he said.

“It’s a complicated issue because of costs and other things. And you don’t want to make it so people have to jump through hoops to participate. You don’t want to prevent them from joining,” he said. “But we just can’t sit back.”

Dr. Sanghavi said having access to automatic electronic defibrillators, to shock a heart back to its normal rhythm, would save lives. They cost approximately $1,000 and are relatively easy to use. At the least, he said, they should be available at high school sporting events.

Clinton High School Principal James S. Hastings said while cost resulted in canceling the former policy of having an EMT and ambulance at games, there are defibrillators available.

Joshua’s death, he said, “forced us to do some self-analysis.”

Dr. David A. Kane, an assistant professor of pediatrics at UMass Medical School, and a specialist in pediatric cardiology, acknowledged controversy about whether to include EKGs in pre-athletic physicals.

“These sudden deaths are tragic events that shake the core of a community, and you always want to find out why they happened,” he said.

Dr. Kane said while EKGs can find many forms of pediatric heart disease, they are not foolproof.

“It’s not a perfect test,” he said.

A thorough clinical and family history — with a parent present — during the physical exam is crucial, he said.

“This small (Stanford) study demonstrated that even the most well trained physicians can have a difficult time interpreting a pediatric EKG. This has been one of the concerns that the American Heart Association has raised in its decision to not recommend routine EKG screening in young athletes in the U.S.,” Dr. Kane said.

Dr. Sanghavi expressed frustration about the way medical examiners report causes of death of young people who die suddenly. He said they lack molecular information, such as DNA testing that could determine if inherited conditions such as hypertrophic myopathy are present in family genes.

“There could be siblings. Wouldn’t you want to know if your child had that gene?” he said.

(End of newspaper article) ________________________

NOTE: It is to be remembered that Daniel had no such cardiac enlargement or any detectable abnormalities following sudden death. Still, cardiac sudden death is likely to be multi-causational and if we can detect some of the cases, we can help to decrease the number of pre-teens and teens to whom this occurs.

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