New Defibrillators Keep 5 Family Members From Cardiac Arrest

LaNay Miceli and her family were diagnosed with Long QT Syndrome.

May 5, 2011 — -- 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.

In the past, three of Miceli's other cousins also died unexpectedly -- one at 35, another at 39 and the last at 52. Her mother's brother died of a heart attack at 36.

"I thought something was odd," said Miceli, 53, a sanitary worker for the city of Chicago. "Too many people kept passing away."

An explanation came only when Miceli's 22-year-old cousin underwent medical tests that revealed Long QT syndrome, a rare genetic heart rhythm disorder that causes fast and chaotic heartbeats. They can trigger fainting spells, seizures, and even sudden death.

Dr. Bradley Knight, director of cardiac electrophysiology at Northwestern Memorial Hospital in Chicago, reached Miceli, suggesting that she and her family be tested as well.

The results were sobering.

"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."

No one in the family had ever had symptoms or indicators of heart arrhythmia.

"It was a little overwhelming to hear [the diagnosis], but we kept it in perspective," said Miceli. "We don't have a death sentence because we had the device put in."

Miceli, her children and cousin decided to have heart defibrillators implanted in their bodies to prevent cardiac arrest.

"In general, the patients who are at high risk of sudden cardiac arrest are older patients with heart disease," Knight said. "However, a small number of these patients have primary electrical problems of the heart. These can even be pediatric patients."

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 -- ICDs for short -- 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. "Every time the heart beats, it's being stretched and pulled, and over a period of time, there is a risk that the wires will fracture or break."

To avoid this problem, Miceli and her family opted for a new model on which Knight is conducting human trials. It is 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.

Study Finds Less Invasive Defibrillator

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.

"We all decided on the device because it's a lot less complicated, and it was a simple procedure," said Miceli. "If I tell my kids and grandkids to get this done, then I was going to get it done, too. We're all in this together."

In January, Miceli's 22-year-old cousin, Matthew, was the first to have the device implanted. Her two children had the surgery soon after.

"I wanted to make sure they were taken care of and make sure they did it before I got it done," said Miceli, who had the S-ICD put in two weeks ago.

Knight said the device is currently in the experimental phase, though he is hopeful that it will win FDA approval in the future.

As for helping the Miceli family with the device, Knight said it was a "good feeling."

"There's the burden of taking care of four family members who all have the same experimental device," he said. But he added that he is "cautiously optimistic" and that he and his team will keep track of the family to see how they do with the cutting edge devices.

Miceli, for one, said she feels the experimental device was the right choice for her and her family.

"I love life, and I want to see my grandkids grow older, and I thought this was the best way to go," she said.

"Having Long QT and then getting this device is like a seatbelt in the car, it's feels like our safety net."