It was 5 a.m. on the Monday after Thanksgiving, and 5-year-old Tyler and his mom, Danielle, were getting ready to leave for his monthly appointment at Children’s Hospital of Philadelphia (CHOP).
“I’ll see you after I get my new heart,” Tyler told his grandparents as he said goodbye.
“I said, ‘No, buddy, you’re not getting your new heart today,’” Danielle remembers.
But 40 minutes later, Tyler’s cardiologist, Joseph Rossano, MD, called with some incredible news: They had identified a potential donor heart for Tyler.
“This looks like a good heart for Tyler,” he said. “Come in with him as soon as you can.”
“I was really excited — and really nervous,” says Danielle. “We’d been waiting so long — and I knew there were more hoops to jump through before it was definitive.”
A frightening 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 (HLHS), a complex congenital heart defect. “He would cry all night,” says Danielle. “He would say, ‘I'm sick. I'm sick.’"
More About HLHS
After examining Tyler and performing a series of tests, the team at the Cardiac Center at CHOP 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 him to health — he would need a heart transplant.
That 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.
The Cardiac Center team helped pioneer the use of VADs in children: 60 children have received a VAD at Children’s Hospital. The team 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 Dr. Rossano. “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.”
At the time of Tyler’s VAD surgery in June 2016, 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 had 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
For the next year and a half, Tyler came to CHOP regularly for checkups with his medical team and received physical and occupational therapy. He also went to preschool, played soccer and learned to ride a bike and a scooter. Every day, he got stronger and stronger.
So when the call came early that November morning, he was ready.
A life-changing surgery
Forty-five minutes after Dr. Rossano called with the news that a donor heart was available, Danielle and Tyler arrived at CHOP. And that afternoon, after running a number of tests, Tyler’s doctors told Danielle and Joe that the donor heart was, in fact, a good match for their son.
At 11:30 p.m., Joe and Danielle walked alongside Tyler as he was wheeled into one of the operating rooms in the Cardiac Operative and Imaging Complex, where Dr. Mascio would perform his heart transplant.
Then they waited. And waited.
Tyler’s surgery was particularly challenging because a great deal of scar tissue had formed around his heart, as sometimes happens after VAD therapy or implantation. Dr. Mascio removed the scar tissue before placing Tyler’s new heart in his chest. And at 7 a.m., Danielle says, Tyler’s heart “started beating and all that beautiful stuff.”
Like many patients who undergo cardiac surgery, Tyler had a few challenges immediately after his transplant, but his medical team worked hard to stabilize him. Danielle and Joe were able to visit him in the Cardiac Intensive Care Unit around 4 p.m.
From that point on, Tyler did very well, says Danielle: “He hit all the milestones they were hoping for.”
And on Dec. 16, just 18 days after his transplant, he went home.
Life with a new heart
Tyler comes to CHOP every few weeks for follow-up, and has heart catheterizations regularly so doctors can take tissue samples and ensure that his body is accepting his new heart.
Every time they arrive at CHOP, his parents are reminded of how much Tyler’s medical team cares for their son — and for them.
“There’s always a genuine amount of positivity and support for Tyler, and for our whole family,” says Danielle.
“Everyone is phenomenal at their jobs from a technical standpoint, and they’re also so personable. It seems like they have a huge stake in his well-being — and that’s so important to me as a mom.”