Cardiac Center

Hypoplastic Left Heart Syndrome: Xavier’s Story

Prenatal diagnosis of hypoplastic left heart syndrome

Xaiver with Mom and Dad

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 a life-threatening heart condition.

“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 begins care for babies with congenital heart disease (CHD) 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 they had the option of going to a top hospital in New York, where they were living at the time, Jackie and Nate chose CHOP’s Cardiac Center for their son’s care because it is at the forefront of advancing care for babies with HLHS, a complex condition 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 four cardiothoracic surgeons and eight pediatric cardiac anesthesiologists, performs more than 850 cardiothoracic surgeries each year — and their outcomes are among the best.

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.

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 ounce 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

Xaiver with Dad
Xavier at 2 years old

Honoring Xavier’s “heart journey” is now a huge part of his parents’ lives. Jackie works with several advocacy organizations in the Philadelphia area to raise awareness about congenital heart disease, and Nate appeared on the TV show “NY Ink” to get a tattoo in honor of his son. As for Xavier, he had the Glenn procedure — the second of the three surgeries he needs to repair his HLHS — at CHOP in October 2011, and in June 2013 he had surgery to repair a leaky heart valve. He also had a pacemaker implanted to help stabilize his heart rate.

Now 2 ½, Xavier comes to CHOP every three months for follow up with the NeuroCardiac Care Program (NCCP), which provides ongoing care and monitoring for cardiac patients who are at risk for neurodevelopmental problems. Xavier’s NCCP clinic visits include sessions with CHOP’s physical therapists, who gave Jackie and Nate tips to help their son learn to walk. Xavier took his first steps a few days before Thanksgiving, and a month later, he became a big brother — his little sister, Elliott, was born on Christmas Eve.

“Xavier is in the best shape he’s ever been in,” says Jackie. “We’re seeing huge developmental gains for him, which is really exciting.”

Xavier will most likely have the final surgery he needs to repair his HLHS in the next year or so, after which 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. The program is part of CHOP’s long-term commitment to caring for children with congenital heart disease — and Jackie and Nate take comfort in the fact that CHOP will always be part of their lives.

“At CHOP, it’s not just about Xavier,” says Jackie. “It’s about all of us.”


 

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Hypoplastic Left Heart Syndrome (HLHS)

Learn more about hypoplastic left heart syndrome, including symptoms and treatment options.

Single Ventricle Survivorship Program

Our Single Ventricle Survivorship program provides evaluation and coordinated, multidisciplinary care for kids with single ventricle defects. Learn more»

HLHS Survival Stories

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