TYLER’S STORY

Single Ventricle Heart Defect

Four-year-old Tyler wears his bright orange backpack everywhere: to preschool, doctor’s appointments, and even the playground, where his mom, Danielle, spends hours pushing him on the swings.

Tyler Eating an Ice Cream Sandwich

“Strangers will stop us and say, ‘He’s really ready for school. He looks like he’s ready to go.’ And I think to myself, ‘Oh, if you only knew,'” she says.

Tyler’s backpack doesn’t contain notebooks and pencils: it holds the batteries and controller for the small device in his heart that’s keeping him alive.

A Life-changing Diagnosis

Danielle and her husband, Joe, first realized that something was wrong with Tyler in March 2016, a few years after he had his third open-heart surgery to treat hypoplastic left heart syndrome, a complex congenital heart defect.

“He would cry all night,” says Danielle. “He would say, ‘I’m sick. I’m sick.'”

After examining Tyler and performing a series of tests, the team at the Cardiac Center at Children’s Hospital of Philadelphia delivered some devastating news: Tyler was experiencing heart failure. They changed his medications, but he got sicker and sicker.

Tyler’s medical team determined that medication or conventional cardiac surgery would not be enough to restore Tyler to health — he would need a heart transplant.

In May, Tyler was listed for a transplant, and his doctors faced a new challenge: keeping him alive until a heart became available.

A Treatment Breakthrough

Children can spend weeks, even months, waiting for a new heart — and until recently, they had to remain in the hospital the entire time. Today, however, some children receive a ventricular assist device (VAD), a mechanical pump that is implanted in the heart. The device circulates blood throughout the body when the patient’s heart is too weak to do it alone.

Support Little Hearts

Can’t make it to Philly Spin-In? You can still help kids with heart disease by donating to a participant, a team, or the event.

Ways to Donate

The Cardiac Center team helped pioneer the use of VADs in children. Sixty children have received a VAD at Children’s Hospital — and they thought Tyler was a good candidate for a VAD called the HeartWare® device. But it took some creative thinking to make it work.

“Most VADs are not designed for children or for individuals with complex circulations,” says Tyler’s cardiologist, Joseph Rossano, MD. “They’re designed for adults with structurally normal hearts — and when we try to use these technologies for small children and for children with complex forms of congenital heart disease, it can be challenging.

“We’ve been able to do it successfully because of some very innovative strategies and techniques that our surgical team has developed,” he adds.

At the time of Tyler’s surgery, he was the smallest child to receive the HeartWare® device, and his case was particularly challenging.

After carefully analyzing the structure of Tyler’s heart using sophisticated surgical modeling techniques, cardiothoracic surgeon Christopher Mascio, MD, devised an approach that was uniquely suited to Tyler’s anatomy. He removed one of Tyler’s heart valves and placed the VAD in his right atrium. VADs are typically implanted in one of the ventricles of the heart, but Tyler’s team decided to take a different approach because he has only one functioning ventricle. The procedure was a breakthrough — and Tyler went home with his family to wait for his new heart.

“I was surprised at how quickly he went back to being himself,” says Danielle. “It only took about two weeks before he was smiling and joking and running around. It was amazing.”

Thriving at Home and in School

Today, Tyler comes to CHOP regularly for checkups with his medical team and receives physical and occupational therapy at the preschool he started attending in December. Every day, he gets stronger and stronger.

“He’s gained 9 pounds since he had the VAD placed,” says Danielle. “And he’s getting taller — I had to replace all his pants!”

Because Tyler is thriving at home, his doctors expect that he will do very well after his heart transplant. In the meantime, he’ll continue doing the things he loves: traveling with his family (they visited Sesame Place and New York City over the holidays), playing with his friends, reading books and coloring.

“We’re so lucky he’s alive — and we’re lucky he’s at home,” Danielle says. “We’re grateful for every day.”