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Congenital Heart Defect: Matthew Williams Gets Second Chance

"He's like a little Energizer Bunny."

Matthew Williams was born with a complex heart problem, where he only had one chamber to pump blood through his body. He had surgery to re-route some of the blood flow at age 3 months, and he received a permanent pacemaker at age 3. Today, Matthew spends quality time with his brothers playing basketball and baseball.

"Everything Matthew does is running, running, running. He's like a little Energizer Bunny," said Lisa, Matthew's mom.

Life has not always been so energetic for 7-year-old Matthew Williams. Matthew was born with a congenital heart defect known as a double inlet single ventricle. In other words, instead of having two heart chambers to pump blood throughout his body, Matthew has only one. In addition, Matthew's heart is flipped over and in the center of his chest.

Lisa and Jim Williams found out something was wrong with Matthew's heart before Matthew and his twin brother, Michael, were born. Even though Michael's heart was okay, it was hard news to hear. "I went from being a proud father who was going to have twins to pretty much devastation," said Jim Williams, Matthew's father.

That's when the Williamses were referred to the nationally recognized Heart Center at Cincinnati Children's Hospital Medical Center, and a world-class team of heart specialists went to work to lay out the best treatment plan for Matthew. "Children's Hospital, I don't think we can say enough about them. They're wonderful," said Lisa.

A Complicated Diagnosis

"Matthew's heart was so complicated it was hard for us to understand, but they sat with us as long as we needed them to and talked with us," said Lisa. One of the first Cincinnati Children's doctors the Williamses met was Peter Manning, MD, a pediatric cardiothoracic surgeon.

"Matthew was born with a relatively complex heart problem," said Dr. Manning. "One of the two ventricles of the heart is very underdeveloped and can't do the work that it needs to do long term. We're only left with one pumping chamber to essentially do the job the heart needs to do and we can't rely on that one pumping chamber to do double duty because it would wear out." To make sure Matthew's heart didn't wear out would require a number of surgeries.

"They thought he was going to have surgery the first week of his life. They had it all planned - what surgery he was going to have. They took us through all the steps and we were ready for surgery. But everything bad that Matthew had he had something good that helped him," said Lisa.

Some Good News

"Matthew was fortunate that when he was born he had a reasonable balance of blood flow to the body and to the lungs so he was able to do well for several months prior to having any surgical intervention," said Timothy Knilans, MD, director of the Electrophysiology Program in the Division of Cardiology at Cincinnati Children's. Dr. Knilans has been Matthew's heart doctor since the day Matthew was born.

"Hi Matt. How you feeling? You've been doing pretty good since your procedure?" Dr. Knilans asked following surgery. "You look nice and pink today."

"It isn't clearly known exactly what causes this or other types of congenital heart disease. It used to be thought it was just bad luck," said Dr. Knilans. "We are learning that these are caused by genetic factors that are acted upon by environmental factors. Matthew's case would be a good one to say it's not entirely genetics, because he has a twin and his twin has a normal heart."

Most children with a heart defect like Matthew's need surgery right after they're born. But because Matthew's blood flow was relatively balanced, doctors were able to wait until he was 3 months old before performing the first procedure.

During that first surgery, Dr. Manning and his team rerouted some of the blood flow to Matthew's heart. Blood from the top of his body was rerouted directly to his lungs. They also combined and reconstructed some of Matthew's arteries to help with blood flow. Matthew came through the surgery with flying colors.

"The hospital's great to you the whole time you're in there. Anything you need, even not just for the baby, but for the parents. 'Do you need a drink, take a break, go take a walk and we'll watch him.' Anything to help you out," said Lisa.

More Heart Surgery for Matthew

During the next couple of years, the doctors at Cincinnati Children's monitored Matthew's condition. And while the surgery had helped, Matthew's heart was still not pumping blood through his body as it should. It was hard for him to keep up with his twin and older brother, Jimmy.

"I remember when I was little he couldn't do anything," said Jimmy Williams, Matthew's brother. "He couldn't walk. He'd scoot around wherever he'd go."

"Matthew couldn't even run around this room. He'd get blue. As he got older, he'd get bluer. His lips got very blue. You'd be out and people would say, 'did he just eat a blue sucker?' He was that blue. So we knew time was coming for the next surgery," said Lisa.

"At 3 he underwent the completion Fontan operation which is designed to channel blood from the lower part of his body out to the lungs so that at his first stage, one component of the operation he underwent involved channeling blood from the top half of his body into his lungs," said Dr. Manning. "Our goal with all of these children's reconstructions is to have all the blood come back from the blood and go directly back out to the lungs."

"People many times ask why are all of these procedures staged? Why can't you do everything all at once and get it over with?" said Dr. Knilans. "If you were to do everything at once at a young age the body wouldn't tolerate it. The blood would back up in the veins and it wouldn't go through the body properly." Doctors also put a hole in Matthew's heart to allow for more blood flow, and once again Matthew came through the surgery with flying colors.

Another Setback with Matthew's Heart Rhythm

Before Matthew could go home, a problem arose. The problem was with the part of Matthew's heart that initiates the heart's rhythm. It wasn't working right. "We went back and put in a permanent pacemaker, something completely implanted under his skin that would help initiate his heart's rhythm and keep it in a regular synchronized rhythm to help improve the efficiency of his heart," said Dr. Knilans.

Matthew's pacemaker is on his left side under his rib cage and is a bit bigger than a quarter. Though the surgery was a success, Matthew was not bouncing back as he had before. It was a rough time for the Williams family. At one point Matthew wasn't walking or eating and had to have a feeding tube.

"He was at the age where he would recognize what was going on. They'd have to wheel him out for X-rays and he couldn't take it anymore. He'd cry and cry and wanted out of there," said Lisa. "I wanted him here and not at the hospital," added Michael, Matthew's twin brother.

After 18 days in the hospital, Dr. Knilans decided it was best for Matthew to recover at home, which now included new baby sister, Paige. It took a few weeks, but soon Matthew was on the road to recovery.

Recently doctors implanted yet another device in Matthew's heart, this one to close the small hole in his heart. And since then, there's been no stopping Matthew.

A New Chance at Life

You would think a child that has to go to the hospital so much and do everything he's had to do would be a little more shy, quiet, reserved and he's not. "Every chance he gets to play a sport, he comes up to me and says, 'Jimmy, do you want to play football or baseball or basketball?'" said Jimmy Williams, Matthew's older brother.

"I think he played three years, I played three years," said Michael. "You can tell by if you count the trophies…yeah three years."

Matthew still has regular checkups and needs to have his pacemaker checked every few months. The pacemaker will have to be replaced from time to time as Matthew grows. He can't play contact sports like football, but otherwise there are no limitations for Matthew.

"The important thing that Cincinnati Children's had to offer for Matthew was to provide the services and surgeries and device and pacemaker interventions that could not be provided in other hospitals to let him have the pretty normal life he has," said Dr. Knilans.