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University of Minnesota
University of Minnesota

Surgery on the cutting edge

October 5, 2011

Eddie and Daniel Gruenstein.

Eddie Kunze shows off the accuracy of his left arm in front of his doctor, Daniel Gruenstein, director of interventional cardiology in the U’s Department of Pediatrics.

Photos: Patrick O'Leary

A new hybrid procedure at the U for children with critical heart defect

By Rick Moore

Eddie Kunze came into this world bright and early on the last day of autumn in 2007. By all outward appearances he was healthy and normal.

“He was born at 5 in the morning and that whole first day he looked great,” says Christina Kunze, Eddie’s mother.

But that evening, things took a quick and dramatic turn for the worse. Nurses noticed a murmur in Eddie’s heart and he was rushed to neonatal intensive care. The following morning he was given an electrocardiogram and, says Christine, “they discovered he had a huge heart defect. … He looked just fine. It was hard to believe.”

Eddie was diagnosed with Hypoplastic Left Heart Syndrome (HLHS), a condition where the left side of the heart—the part responsible for circulating oxygenated blood to the body—is extremely underdeveloped, according to Dr. Daniel Gruenstein, director of interventional cardiology in the U’s Department of Pediatrics. 

Two to three decades ago, this was a uniformly and quickly fatal disease, and still is in many parts of the world. But Eddie received a series of surgeries at the University of Minnesota that now have him back at home in Buffalo, Minnesota, chasing after his four siblings.

A hybrid surgery

The initial surgical procedure that gave new hope to children with HLHS was first performed by William Norwood and bears his name. It’s a staged series of operations that allow the heart to slowly only accept the high-oxygenated blood and pump that blood to the body, while the low-oxygenated blood is rerouted passively to the lungs, says Gruenstein, one of Eddie’s doctors.

But there are downsides to the Norwood procedure. Its first stage is a long, complex operation that requires use of a heart-lung bypass machine and necessitates complete cessation of blood flow to the brain for a time, putting patients at a risk for brain damage.

The hybrid first-stage operation, which Eddie received, combines surgical techniques by teams of pediatric cardiothoracic surgeons and interventional cardiologists like Gruenstein.

Instead of placing the child on a heart-lung bypass machine in the first surgery, the entire procedure is performed while the child is circulating his own blood, so blood flow is never blocked to the brain. Surgical bands are inserted to regulate the flow of blood to the lungs, and a stent is inserted through a catheter into the PDA, a small artery that typically closes in the first few days of the life. In patients with HLHS, the PDA provides the only stable source of blood flow to the aorta, the brain, and the heart muscle.

The child is then able to return home and have six months of growth prior the big surgery involving the heart-lung bypass machine. Gruenstein says that a child whose brain has developed for six months is thought to be less vulnerable to injury from the procedure.

“Eddie was the third patient we did this procedure on,” Gruenstein notes. “He was not an ideal candidate for having the hybrid surgery because of his aorta anatomy. But by the time he got to us, he had very, very poor circulation, the amount of acid in his body was dangerously high, and he was actually having seizures and having poor oxygen delivery to his brain. … His heart function was extremely poor.”

At the leading edge of medicine

According to Gruenstein, the University’s is the first program in the Upper Midwest to perform the hybrid procedure.

“It’s always challenging to start doing something that’s brand new,” Gruenstein says. “There’s no data to point to in deciding whether this is a better procedure than preexisting procedures. Someone has to create that data for everyone else to look at.

“We have a long tradition at the University of Minnesota—at the Medical Center and here at the Amplatz Children’s Hospital—of being the ones on that front edge of technology to start providing the data for the rest of the country.”

But it requires significant buy-in.

“There has to be some leap of faith, not only by the teams involved in performing the procedure, but in the referring cardiologists who are asking to having something new performed on their patients,” Gruenstein says. “And then, most importantly, a leap of faith by the families of very vulnerable children. We’re asking them to try something brand new, typically at a time when they weren’t expecting to have any other challenge than making sure they had enough diapers at home and a room set up for their baby.

Back at home and settling in

Eddie received his last staged procedure on July 21, 2011, and needed an extended stay at the state-of-the-art Amplatz Children’s Hospital to overcome some excess fluid that was collecting in his lungs, a not-uncommon hurdle after this type of surgery. Christina Kunze took him home on Labor Day—September 5—to rejoin his four siblings, ages 9, 13, 15, and 18 at the time.

Watching Eddie go through multiple surgeries and their accompanying recoveries has been difficult for Christina, but her parents have been able to watch over the other children while Eddie was in the hospital.

“I look at it as God’s way of making sure that I kept it together for my family and for my kids,” she says. “My other four have dealt with it so amazingly. This is their brother and they take him for what he is.”

“He’s an old soul,” she adds. “He’s earned his age in his brief time he’s been here.”

And his pictures have earned a place in Gruenstein’s office. Each year at the end of October he’s received a picture of Eddie dressed up like a monkey for Halloween—as a newborn, a 1-year-old, and a 2-year-old. It’s a poignant “data point” of how the cutting-edge work at the U is dramatically improving lives of children.

“It’s wonderful any time anyone gets a role in making a kid feel better,” Gruenstein says. “Whether that’s being the person to put the stent in their heart or being the nurse to give him the pain medication afterward, or being the child family-life specialist to play games with him to help keep him distracted, we’re all really blessed to be in a position where our daily job is to make kids feel better.”

Sitting in the lobby of Amplatz, Gruenstein reflects for a moment on how those blessings have been passed on to an animated, energetic 3-year-old who always makes him laugh, regardless of either’s mood that day.

“In this case, it is more rewarding because we took a child who was really at death’s door and hopefully we’ve given him an entire life out of a situation where, even just a couple of years earlier, I don’t think he would have had a chance.

“And I’m looking forward to the end of October when I can expect my yearly Halloween card.”

Eddie Kunze and Christina Kunze.Following his final staged surgical procedure, Eddie had an extended stay at the University of Minnesota Amplatz Children's Hospital. His mother, Chris, was by his side every step of the way.

Tags: Academic Health Center

Related Links

Dr. Daniel Gruenstein

University of Minnesota Amplatz Children's Hospital

The Heart Center

Hypoplastic Left Heart Syndrome

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