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Cardiovascular Report - The Lonely Truth About Kids and Long QT

Cardiovascular Report Spring 2010

The Lonely Truth About Kids and Long QT

Date: May 15, 2010

In tackling an arrhythmia known to fell young athletes, kid gloves rule.


Jane Crosson
Cardiologist Jane Crosson believes achieving compliance with young long QT patients relies on “good doctoring.”

Part of Jane Crosson’s job is telling young athletes that it’s time to pull into the slow lane, but one recent case really tested her mettle. After a Maryland father’s EKG showed evidence of long QT syndrome, Crosson soon confirmed that all three of his teen sons had inherited the same disorder. Worse, the boys were all competitive swimmers, an activity known to provoke the potentially fatal arrhythmias.

“We had to redirect their competitive activities,” says Crosson, a Johns Hopkins cardiologist who specializes in pediatric electrophysiology. “They’ve taken up golf. And they’ve all been great sports about it.”

Given the boys’ athletic zeal—evidenced by their hard-won spots on their school swim teams—Crosson says the transition called for special powers of persuasion. None of the boys had developed a symptom, and no family member was known to have died from long QT. “It’s hard for teens because they can’t see or feel it,” she says. “They can’t wrap their head around it.”

The trick to winning patient buy-in, Crosson believes, is a combination of support by adult family members and “good doctoring.” In this case, says Crosson, the consensus of multiple senior specialists helped. The group marshalled strong educational resources and assistance with emotional support.

With patient buy-in, says Crosson, comes the crucial compliance with the new regimens of medicine, sports cutbacks and attention to the value of having portable defibrillators nearby when needed. The prize can be a long and healthful life.

Such family cases raise the profile of the group’s genetic counseling service, says Crosson, and the field is making strides. Seven of the major genetic markers are now well-known, covering 74 percent of all known cases. Crosson says long QT experts are hot on the heels of the missing markers.

Drilling down, Crosson says her field is also getting better at defining three separate forms of the syndrome, all of which are characterized by the essential abnormality in refractory time. Cardiologists all know the disorder stems from an abnormality in the intracellular movement of sodium or potassium, which can set off the distinguishing torsade de pointes form of tachycardia, a “very nasty rhythm,” says Crosson, that can kill if it’s not disrupted.

What’s changing, she says, are the recommendations for the optimal therapies. For instance, it’s known that beta blockers are excellent for the first type of LQT, but they can worsen the risks for LQT3.

The good news, Crosson says, is that both genetic and therapeutic advances are moving the field forward: “The goal is to find better individual treatments for individual types of long QT. We’re closer on the medical treatment than we are on the gene treatment, but we are getting there.”

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