Hypoplastic Left Heart Syndrome: Lily’s Story

When Lily’s parents watch her play soccer, they experience love and pride, like every mom and dad. But they also feel amazement, because they know their 5-year-old runs with a heart unlike any other on the field.

lily“It’s incredible to see her be able to play soccer with all the heart-healthy kids,” says her mom, Julia. "She looks like every other kid out there. People don’t even realize what she’s been through, and I think that’s the coolest thing.”

Choosing CHOP

When Julia was 19 weeks pregnant, an ultrasound showed the baby’s heart was not forming properly. A cardiologist at the local hospital told Julia and her husband, Jeremy, that their baby likely had hypoplastic left heart syndrome (HLHS), a single ventricle heart defect. In this complex congenital heart defect, the heart is unable to pump properly because its left side is underdeveloped.

Julia and Jeremy knew right away where they wanted to go for care: The Children’s Hospital of Philadelphia (CHOP), home to one of the best pediatric cardiac care teams in the nation and a leader in the care of children with HLHS.

“Before this happened, we didn’t realize how lucky we were to have CHOP so close by,” Julia says. “We took it for granted. But through this whole journey, I’ve met a lot of other families who come from very far away so their child can be cared for at CHOP. To be a half-hour away is incredible.”

Julia and her husband made an appointment with the Fetal Heart Program at CHOP, a program that specializes in the detection, evaluation and management of fetal heart defects prior to a baby's birth.

They met with Jack Rychik, MD, medical director of the Fetal Heart Program, and Denise Donaghue, MSN, RN, program coordinator. From the moment Denise came to greet them in the waiting room, they felt they were in the very best hands.

“It was a very difficult and stressful time, but I felt so comfortable with the team we had chosen,” Julia recalls.

For the rest of the pregnancy, Julia visited Dr. Rychik once a month. The Fetal Heart team carefully monitored the development of her baby’s heart, and made a plan for care immediately after birth so the baby would have a better chance at survival

Lily’s journey

After Lily was delivered full-term at a local hospital on Feb. 25, 2006, she was stabilized and taken by ambulance to CHOP.

At 2 days of age, Lily had her first open heart surgery. Children with HLHS must usually undergo three operations, known as staged reconstruction.

During the reconstruction of a heart with hypoplastic left heart syndrome, the surgical team alters the heart and circulatory system. Deoxygenated blood, which is normally pumped through the right side of the heart to get to the lungs, instead travels directly to the lungs. The heart becomes a one-sided pump rather than two-sided.

This altered system, called Fontan circulation, was applied to critically ill newborns through the pioneering work of doctors at CHOP and other institutions. The Fontan circulation drastically increases survival rates for children with hypoplastic left heart syndrome. Thirty years ago, these babies did not survive.

Lily's first surgery

Before the surgery, Julia and Jeremy met with Lily’s surgeon, Thomas Spray, MD, chief of the Division of Cardiothoracic Surgery at CHOP.

“Dr. Spray has that personality that makes you feel comfortable,” Julia says. “He’s very reassuring and explained exactly what he would do. I was always confident that everything was going to work out.”

After Lily’s first surgery for HLHS, she was at CHOP for one month. She was unable to breastfeed or feed from a bottle, which contributed to her unusually long recovery. She was fed instead through a nasogastric feeding tube, while a team of nurses and speech therapists, along with her parents, worked with her until she could drink from a bottle.

“The nurses were amazing,” Julia recalls. “Everything they would do, from drawing blood to giving a new type of medicine, they would explain it step by step. I would sit there all day long with Lily, and the nurses were always there for us.”

Finally, Lily was able to come home.

Follow-up surgeries

When she was 5 months, she had her second operation. A few months later, she experienced tachycardia (racing heart), and Dr. Spray operated again, this time to place a pacemaker.

When Lily was 3½ she had her third operation for hypoplastic left heart syndrome, known as the Fontan operation. That surgery completed the alteration to Fontan circulation and this time her recovery was quick.

“We were in and out of the hospital in three days,” Julia recalls.

Hope for the future

Many children who have complex congenital heart defects, like Lily’s HLHS, experience delays in growth and development. Lily was delayed as an infant, but occupational therapists and physical therapists helped her, and she caught up quickly.

“She’s right on target now,” Julia says. “She started kindergarten this year and she loves it.”

Julia and Jeremy informed Lily’s teachers of her single ventricle heart defect. “I told them, ‘Don’t limit her. I don’t want to limit her. I want her to be able to try to do everything she can.’”

Lily takes one medicine daily for blood pressure and visits CHOP every six months to have her pacemaker checked and to see her primary cardiologist, Richard M. Donner, MD.

“I can’t say enough good things about Dr. Donner,” Julia says. “He’s fabulous. He’s down-to-earth. He explains everything, and when we’re upset or concerned he tells us not to worry. He is always available when we have questions.”

CHOP by her side

The oldest patients to survive infant surgery for single ventricle heart defects are in their late 20s. As more and more children with Fontan circulation survive and grow, doctors are recognizing that some are experiencing health problems affecting the lungs, gastrointestinal system and other systems of the body.

CHOP is a leader in providing care for single ventricle survivors and pursuing research to discover why the side effects are occurring. Experts at CHOP are also developing innovative solutions to continue to improve the quality of life for these patients.

Julia and Jeremy take comfort in knowing the CHOP team will be there for their little girl for many years to come.

“We talk about it,” Julia says. “She shows people her incision. She calls it her zipper. She’ll talk about it at school, and she will show it to everyone. For the most part now she’s proud of it. Lily knows she has a special heart — that’s what we call it.” 

Video update from Lily

Catch up with Lily today and hear her talk about how she feels about her doctors and nurses at the Cardiac Center.

Originally posted: October 2011
Updated: September 2013