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Entries in Long QT Syndrome (1)

Thursday
May052011

Doctors Implant New Defibrillator in Family with Genetic Heart Condition

Creatas/Thinkstock(NEW YORK) -- Members of LaNay Miceli's family seemed to be dying, young and suddenly, all around her. There was her 24-year-old cousin who just didn't wake up one morning, her 55-year-old cousin who went into cardiac arrest while chatting on the telephone and another cousin who was only 40 when he had a heart attack. And that was just last year.

Doctors finally suggested that Miceli and her family be tested for Long QT syndrome, a rare genetic heart rhythm disorder that causes fast and chaotic heart beats. The rapid and out-of-sync beats can trigger fainting spells, seizures, and even sudden death.

"I have it, but my sister doesn't," said Miceli. "My two children had to be tested and they both have it, and my daughter's two boys were both diagnosed, as well." Miceli said her 22-year-old cousin was also tested and diagnosed with Long QT. No one in the family had ever had symptoms or indicators of heart arrhythmia.

Miceli, her children and cousin decided to have heart defibrillators implanted in their bodies to prevent cardiac arrest. Doctors said Miceli's grandchildren, aged 5 and 1, are too young to have the devices implanted, and instead were given beta blockers, medications they take three times a day.

The devices, called implantable cardioverter defibrillators save lives by detecting and correcting abnormal heart rhythms that can lead to cardiac arrest and death. An ICD includes a battery pack that is typically placed under the collar bone, with wires that run through the veins and into the heart, usually through the right ventricle. The device acts as an emergency response that signals and restores an irregular heartbeat if necessary.

"In young patients, the [older model] can be a real problem because the wires we have are imperfect and because the wires in the blood vessel are subjected to stress and trauma from the heart beating," said Dr. Kenneth Ellenbogen, chairman of the division of cardiology at Virginia Commonwealth University School of Medicine.

That's why Miceli and her family opted for a new model, which is still under review with the FDA. Researchers formally presented the new device on Wednesday at the Heart Rhythm Society's 32nd Annual Scientific Sessions. The defibrillator, known as S-ICD, is implanted just under the skin, on the chest wall, below the breast tissue. Similar to the old model, scientists said that it provides protection against abnormal heart rhythms, but it is less invasive because it does not require wires to touch the heart.

The device is already in use in Europe and it is currently available through clinical trials in the United States.

In the presented study, researchers in the Netherlands implanted the device in 98 patients and found that the S-ICD system was 100 percent effective in correcting artificially-induced heart arrhythmias. Sudden death did not occur in any patients during the nine-month follow-up period.

The researchers concluded that "in our experience, the S-ICD system is a viable alternative to conventional ICD systems for selected patients."

According to the American Heart Association, sudden cardiac arrest affects about 300,000 Americans each year. Brain death and permanent death occur within four to six minutes after cardiac arrest occurs so it is crucial to correct any sort of heart malfunction quickly.

Copyright 2011 ABC News Radio







ABC News Radio