Hypoplastic Left Heart Syndrome: Xavier’s Story

Jackie Ross 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 The Children's Hospital of Philadelphia'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 CHOP's Cardiac Center 

Though they had the option of going to a top hospital in New York, where they were living at the time, the couple chose Children's Hospital'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 Ross 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 on his tiny heart. This lifesaving reconstructive surgery was pioneered at Children's Hospital. The Hospital'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 staged reconstruction heart 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.

That wouldn’t have been the case 30 years ago, when many children with heart defects like Xavier’s died within days of birth. Advancements in care since then mean that today most of these patients survive, allowing doctors to focus on providing a good quality of life as they grow up.

Going home

Xavier’s surgery went well, and after a month at Children's Hospital, 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. I was so prepared.

Raising awareness 

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. Jackie wears a pendant 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 the Glenn procedure — the second of the three surgeries he needs to repair his HLHS — at Children's Hospital in October 2011. In June 2013 he had surgery to repair a leaky heart valve. He also had a pacemaker implanted to help stabilize his heart rate.

Looking ahead

xavier Now 2½, Xavier comes to the Hospital every three months for follow up with the Cardiac Kids Developmental Follow-up Program, which provides ongoing care and monitoring for cardiac patients who are at risk for neurodevelopmental problems.

Xavier’s Cardiac Kids 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. He'll then enroll in CHOP’s Single Ventricle Survivorship Clinic, part of the Fontan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) Program, to receive care from a multidisciplinary team of specialists, including cardiologists, gastroenterologists and hepatologists, as he grows up.

The program is part of the Hospital's long-term commitment to caring for children with congenital heart disease — and Jackie and Nate take comfort in the fact that Children's Hospital of Philadelphia will always be part of their lives.

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

Originally posted: June 2012
Updated: September 2013

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