Hypoplastic Left Heart Syndrome: Lilly’s Story

Laura and Bryan Moore of Peoria, AZ, believe the best cardiac care in the world is worth traveling for.

For months, the Moores pored over information on the internet, spoke with other families, and consulted with pediatric cardiac experts across the United States to find the best-qualified team to care for their daughter, Lilly, who has hypoplastic left heart syndrome (HLHS).

In the spring of 2014, they made their decision — and traveled 2,300 miles to Children’s Hospital of Philadelphia (CHOP).

Choosing Children’s Hospital of Philadelphia

The trip was a turning point in what had already been a long and challenging journey. Lilly had been diagnosed with HLHS in utero, and had undergone two of the three surgeries required to repair the defect at a hospital near her family’s home.

“It was so overwhelming at first that we didn’t think to look out of state,” says Laura.

But as Laura and Bryan learned more about Lilly’s condition and met other “heart parents,” they decided to explore other options for Lilly’s third surgery, the Fontan procedure.

More About HLHS

Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect in which the left side of the heart is underdeveloped.

Everywhere they turned, the Moores heard very good things about the CHOP team, including Thomas Spray, MD, chief of the Division of Cardiothoracic Surgery, and interventional cardiologist Andrew Glatz, MD. They were also impressed with the team’s vast research expertise and robust, proven protocols for care.

“I want what’s tried and tested,” says Laura. “I didn’t want Lilly to be an experiment.” The opportunity to enroll Lilly in CHOP’s Single Ventricle Survivorship Clinic, part of the Fontan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) Program, which provides ongoing screening and care for patients after the Fontan, also played a big part in their decision.

Says Laura:

I knew in my heart that CHOP was where we would be taking Lilly for continued care.”

Part of the team

The Moores arranged for Lilly’s medical records to be sent to CHOP pediatric cardiologist Anita Szwast, MD, and worked closely with Dr. Szwast and the CHOP team to develop a care plan for Lilly. In May 2014, they traveled to Philadelphia for Lilly’s Fontan surgery and began meeting with the experts who would care for their daughter — including Dr. Glatz, who performed Lilly’s preoperative catheterization in CHOP’s state-of-the-art Catheterization Laboratory.

Dr. Glatz’s compassionate, thoughtful approach immediately put the Moores at ease. “We were not just a number,” says Laura. “We were treated with care and concern — and we left that meeting fully understanding what was going on with her heart and what would happen the next day.”

Fontan procedure

Early on the morning of May 13, the Cardiac Center’s team of specialized pediatric cardiac nurses and anesthesiologists got Lilly ready for surgery, and Laura and Bryan met with Dr. Spray, the world-renowned pediatric cardiac surgeon who would perform Lilly’s Fontan.

The Cardiac Center’s four cardiothoracic surgeons perform more than 800 surgeries each year, giving them a level of expertise that few can match. “We had so much confidence in the team,” says Laura.

Lilly’s surgery went smoothly — and after a 10-day stay in the Cardiac Center’s inpatient units, she was discharged from the Hospital. She and her parents returned to Arizona a few days later.

The Moores were very impressed with the entire team that cared for Lilly: Dr. Spray, Dr. Glatz, the nursing staff, and pediatric cardiologists Dr. Szwast and Meryl Cohen, MD. And it wasn’t just their clinical skills that made an impression: “To this day, Lilly remembers Dr. Cohen getting on the floor with her and playing Mr. Potato Head,” says Laura. “And Dr. Szwast visited us each day while we were inpatient. Everyone involved in Lilly's care was extremely attentive and caring. The team’s compassion, their knowledge, their communication — it’s really quite remarkable.”

Follow-up care

Lilly is now an active 4-year-old who loves riding bikes and playing with her older sister, Alex. “Lilly is flourishing,” says Laura. “She loves people, she loves life — she’s a joy.”

Lilly’s CHOP team works with her pediatric cardiologist in Arizona to manage her follow-up care, and Lilly will continue to come to CHOP once a year for checkups with Dr. Szwast. When she’s a little older, she’ll enroll in the Single Ventricle Survivorship Clinic, part of the Child and Adolescent Mood Program (CHAMP), which has provided care for more than 400 patients — many of whom, like Lilly, travel to CHOP from outside the Philadelphia area.

“We want her to have the best care and the best sets of eyes on her,” says Laura.

At CHOP, you are treated as an equal and listened to as part of the care team for your child. I will recommend CHOP wholeheartedly to anyone seeking the best medical care for their loved one.