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Special Pacemaker Surgery is a Perfect Fit for Toddler at Johns Hopkins All Children’s Heart Institute

Posted on Oct 02, 2015

The sight of 2-year-old Giselle Thomas pulling a little red wagon up and down a Cardiovascular Intensive Care Unit corridor, giving her favorite stuffed animals a joy ride, was a moment worth savoring.

Less than three days earlier, Giselle lay on a table in a CVICU catheterization suite at the Johns Hopkins All Children’s Heart Institute. Her cardiologist, Jamie Decker, M.D., stood on one side of his patient to assist in the operation, while pediatric cardiac surgeon Tom Karl, M.D., worked deliberately on the other. As monitors beeped and nurses provided support throughout the room, Dr. Karl placed a defibrillator lead behind the toddler’s small heart – a relatively new approach ideally suited to small children.

“The approach that we took for Giselle allowed us to avoid an open surgical procedure which typically requires a large incision,” Karl explains. “In this case we could use a very limited incision just below the sternum, which will have a superior cosmetic as well as functional result. At the same time we can achieve effective protection from life-threatening electrical rhythm disturbances.”

The traditional method employed for teens and adults involves what is known as a “transvenous” system, in which the device sits up near the chest and the tiny leads are inserted through the veins inside the heart – through two incisions made in the shoulder area. This approach is usually impossible in small children, and an open procedure is required to implant the system.

At All Children’s, however, we are doing something different, something far better suited for youngsters. Making a single incision in the abdomen without having to cut open the breastbone, Karl utilizes the leads that are designed to go through the veins, but instead attaches them to the outside of the heart.

The result: a less invasive surgery that reduces the trauma on a toddler, and accelerates healing time – all part of the overarching quality and safety mission at All Children’s Hospital Johns Hopkins Medicine.

The first such surgery at the Johns Hopkins All Children’s Heart Institute had been performed by Karl one day earlier. The second patient to benefit from the new technique was little Giselle.

 “I first used this approach in Australia, after learning about it from colleagues in South Carolina,” he continues. “We modified it slightly to suit our smallest patients, and I am now certain that it is the best approach. Although the procedure is novel here, I can assure parents that my past experience gives me the confidence to recommend the operation for patients such as Giselle.” 

Proof of success was soon evident: The surgery took place late on a Thursday afternoon and by Sunday Giselle was all smiles – and ready to be discharged. It was the happy and hopeful culmination of a frightening experience for parents Alexis and Daniel Thomas over the past 10 months, including two episodes of their precious daughter passing out inexplicably.

“We’re so grateful to All Children’s,” says Alexis. “Dr. Decker came in multiple times before surgery and Dr. Karl did, too. We had plenty of opportunities to talk and ask questions and make sure we knew exactly what was going on.  When Giselle went into surgery, we felt completely confident – knowing this is what we had to do, and this is going to be best for her.”

The problems began in August of 2014, when Giselle passed out with no warning. She had become upset at having to go to bed one evening, held her breath and then fainted – resulting in CPR and a terrifying trip to the ER. After that, Giselle began seeing Heart Institute pediatric cardiologist Michelle Miller, M.D., at All Children’s Outpatient Care, Sarasota. Because her father had received a pacemaker six years earlier, he soon underwent tests to determine if he had a condition possibly related to his daughter’s.

That’s when it was determined Daniel had Brugada syndrome, a heart rhythm disorder that can be life-threatening, with a risk of abnormal rhythms in the heart’s lower chambers. Initial tests on Giselle did not reveal any evidence that she had Brugada syndrome. But then came her second episode in  May 2015, and an appointment was made for her to see Dr. Decker at the All Children’s Hospital Johns Hopkins Medicine main campus in St. Petersburg.

A week before that appointment, however, Giselle came down with a cold and fever, something that can make Brugada easier to detect if it exists. An ECG was immediately performed and showed a highly pronounced abnormality in her heart rhythm. Dr. Decker was informed of these results and immediately arranged for an All Children’s emergency transport ambulance to bring Giselle from Sarasota to St. Petersburg.

“We met Dr. Decker that Monday afternoon for the first time and he said there was no question on the ECG – Giselle had Type 1 Brugada, which is what my husband also has,” says Alexis. “He said she definitely needed the defibrillator.”
 ”This disease is challenging to diagnose because it is based on characteristic ECG findings that are typically intermittent,” Decker explains. “The decision to send the family for the ECG while she was ill and had a fever, when the Brugada pattern is more likely to be present, demonstrated the expertise of the members of the Johns Hopkins All Children’s Heart Institute and a large reason why I work here.  Being part of a team where we not only diagnose such conditions, but can tailor treatment plans to what is best for individual patients, which is a huge mission of our hospital, reaffirms why I became a doctor.  This could have been misdiagnosed as something more common and less serious and Giselle may not have woken up the next time she passed out.”

Because of Giselle’s cold, surgery had to wait a few more days. The stage was set for the minimally invasive procedure employed by Karl. One additional advantage to inserting the leads through the belly – and not the shoulder – is that it minimizes the chance a toddler might accidentally pull out the wires or break them.

“You can’t tell a 2-year-old not to wave their arms in the air or over their head,” Alexis says. “This way, she doesn’t have to be restricted nearly as long.”

And arms can be used for more enjoyable pursuits just days after surgery, like pulling a little red wagon with furry pals along for the ride.

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