Hypoplastic Left Heart Syndrome: Xavier’s Story
Prenatal diagnosis of hypoplastic left heart syndrome
Jackie was 20 weeks pregnant with her first child when she and her husband, Nate, received the devastating news: The baby they already loved so dearly would be born with half a heart.
“I saw it as a death sentence,” Jackie says. “We went through all the stages of mourning.”
But as she and Nate learned more about their son’s condition, hypoplastic left heart syndrome (HLHS), they found that they had options — good ones. And when they watched a video about CHOP’s world-renowned Fetal Heart Program, which cares for babies with heart defects while they are still in the womb, Jackie says, “We finally realized that this might all be OK.”
Choosing the Cardiac Center at The Children’s Hospital of Philadelphia
Though the couple had the option of going to a top hospital in New York City, they chose CHOP’s Cardiac Center because it is at the forefront of advancing care for babies with HLHS, a complex defect in which the left side of the heart is underdeveloped. And they were thrilled to learn that Jackie could deliver in the Hospital’s Garbose Family Special Delivery Unit (SDU), the world’s first birth facility designed for mothers carrying babies with known birth defects. The SDU is just steps away from the Hospital’s Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit, so Jackie would be close to her son as he awaited his first open-heart surgery.
“As soon as we walked into the SDU,” Jackie remembers, “I said, ‘This is exactly where we’re supposed to be.’”
Lifesaving open-heart surgery
Xavier Thomas was born in the SDU on May 28, 2011. ”It was amazing,” Jackie says. “My biggest fear was that it wasn’t going to be like ‘real’ labor because of his heart. But they did everything they could to make it as normal as possible.” Jackie and Nate spent hours cuddling and rocking Xavier. Their families visited. And that night, Jackie was able to breastfeed her son.
Four days later, Xavier was wheeled to the operating room where J. William Gaynor, MD, would perform the Norwood procedure, a lifesaving reconstructive surgery that was pioneered at CHOP, on his tiny heart. CHOP’s cardiothoracic surgery team, which includes three cardiothoracic surgeons and eight pediatric cardiac anesthesiologists, performs more than 800 open-heart surgeries each year – and their outcomes are among the best in the nation.
The Norwood procedure is the first of the three open-heart surgeries needed to repair HLHS. When Xavier’s surgeries are completed, the right side of his heart will be able to pump oxygenated blood to his body – a job normally done by the left side. Like most kids with HLHS, he will face many challenges, but he is expected to live a full and happy life.
Thirty years ago, his future wouldn’t have been nearly as bright. At that time, many children with heart defects like Xavier’s died within days of birth. “We were basically trying to get the kids to survive — period,” says Gil Wernovsky, MD, Xavier’s cardiologist at CHOP. “Now, care has advanced to the point that the vast majority of these patients survive, and we’re focused on making sure they have a good quality of life as they grow up.”
Going home
Xavier’s surgery went well, and after a month at CHOP he went home. It was a joyous but nerve-wracking time for Jackie and Nate. Xavier needed constant care: changing his feeding tube, monitoring his oxygen levels, weighing him every other day, tracking every milliliter he ate. It was exhausting. And it was very serious work, because babies with HLHS are at especially high risk for complications during the time between their first and second surgeries. It helped that Alyson Stagg, MSN, CRNP, coordinator of the Cardiac Center’s Infant Single Ventricle Monitoring Program (ISVMP), called Jackie and Nate every week to track Xavier’s progress and was always available to answer their questions. As part of the program, Jackie and Nate had also taken several classes at CHOP to learn how to care for their fragile baby. “I really think that was why he did so well at home,” Jackie says. “I was so prepared.”
Looking ahead
Tattoo reads: "Half your heart makes me whole"
Honoring Xavier’s “heart journey” is now a huge part of his parents’ lives. To raise awareness about congenital heart disease, Jackie wears a necklace with a charm in the shape of a bandaged, broken heart, and Nate appeared on the TV show NY Ink to get a tattoo in honor of his son. As for Xavier, he had his second open-heart surgery at CHOP in October, and he now comes to CHOP every three months for follow-up visits as part of the NeuroCardiac Care Program, which provides ongoing care and monitoring for cardiac patients who are at risk for neurodevelopmental problems. After his third open-heart surgery — which he’ll most likely have sometime in the next few years — he’ll enroll in CHOP’s Single Ventricle Survivorship Program to receive care from a multidisciplinary team of specialists, including cardiologists, gastroenterologists and hepatologists, as he grows up. It’s another part of CHOP’s commitment to caring for children with congenital heart disease at every stage of their lives.
For now, though, this always-smiling survivor is focused on the things he loves most in life: eating, playing with toys, taking baths and going for rides in the car. And during a recent trip to Florida, he experienced many new things. He went to the beach. He went to the zoo. And, for the very first time, he started waving hello.
Contact Us
For a second opinion, cardiac referral, or for more information.
267-426-9600
To schedule an outpatient appointment.
215-590-4040
