Hypoplastic Left Heart Syndrome: Caila's Story
Natasha Fencl will never forget looking at the ultrasound at her obstetrician’s office. She knew immediately: her baby had a severe heart defect.
A respiratory therapist, she had been working for the 
Cardiac Center at The Children’s Hospital of Philadelphia for three years. She was five months pregnant with her first child.
“They had her heart up on a screen and I could tell she was a hypoplast,” she remembers. “I was petrified.”
“Hypoplast” is a term for a patient with hypoplastic left heart syndrome (HLHS), in which the left side of the heart is underdeveloped. After the ultrasound, Ms. Fencl called the Cardiac Center to tell her friends and colleagues the news.
Minutes after she hung up, her phone rang. It was pediatric cardiologist Gil Wernovsky, MD. “He named all of these appointments for the following day,” she recalls.
For the next four months, as she continued working in the Cardiac Center, Ms. Fencl was also a patient. She visited Fetal Heart Program staff every two weeks, and then every week. The team made a plan for delivery, and educated about what to expect: at least three open-heart surgeries. “They gave us the rundown without having it be too overwhelming,” Ms. Fencl says.
Caila was born full-term, Nov. 16, 2000, 7 pounds and 10 ounces. Delivered at the Hospital of the University of Pennsylvania at 7:01 p.m., she was immediately taken next door to Children’s Hospital, where a team was waiting.
If Caila were born today, she may have been delivered in Children’s Hospital’s new Garbose Family Special Delivery Unit (SDU), for babies with birth defects. The unit is on the same floor as the cardiac units, so parents and baby stay close. “It’s hard to have a baby and not be able to hold her,” Ms. Fencl recalls. “That’s what’s great about the SDU. They’re right here.”
Caila’s father, George, visited her that night and Ms. Fencl was able to see her the next morning. “They had put a little bow in her hair,” she recalls. “They doted on her. It was like they were taking care of one of their own.”
That was a Friday. The first surgery would be Monday. “We had her baptized and my parents came down,” Ms. Fencl recalls. “We got to hold her. Monday was the hardest day of my life by far.”
Thomas Spray, MD, performed the first surgery. During the four-hour procedure, he created a Blalock-Taussig shunt, connecting another artery to the pulmonary artery to allow extra blood flow to the lungs. He also enlarged Caila’s aorta, which was abnormally small.
Caila spent 12 days in the Hospital. After she came home, she had difficulty eating at first, but gradually began to gain weight.
When Caila was three months old, Ms. Fencl returned to work full-time. She educated the daycare staff about HLHS and verified that they were CPR-certified.
Caila had her second and third surgeries at 5 months and 18 months. Her circulatory system was reconfigured so that de-oxygenated blood that has circulated through her body flows directly to her lungs. Normally, this blood would flow into the right side of the heart and be pumped to the lungs. In patients with HLHS, after the series of surgeries, the right side of the heart does what is normally the job of the left side — pumping oxygenated blood to the body — because the left side is too underdeveloped to function.
Caila is now 8 and in third grade. She visits the Cardiac Center approximately four times a year. As a result of her surgery, she developed heart block, a problem with the electrical system that controls the heart’s beating; she requires a pacemaker. Caila has been in dance class since she was 3, but other activities such as gymnastics and soccer that could damage pacemaker wires are discouraged.
Caila is a healthy, happy child. “She enjoys going to the playground, watching movies and singing,” her mom says. “She loves coloring and just pretending. She’s a great kid.” She does struggle in school. “She has a hard time with math and some of her grammar but we’ve had her in tutoring and she gets the extra help she needs.”
Her experience gives Ms. Fencl a deep appreciation of her workplace. “I’m a lifer,” she says. She works in the Tabas Cardiac Intensive Care Unit, putting patients on ventilators and caring for children with special respiratory needs.
When appropriate, she discusses her experience with parents. “If I sense that somebody wants to talk I will bring it up,” she says “They can look at me and know that I understand what it’s like to be scared to death for your child.”
“The parents ask me simple things like, ‘How is she doing?’ I’ll show them a picture. I preface it by saying all of these kids are different. But for the majority now, they do really, really well. They’re going to run and play and go to school.”
Thirty years ago, children born with HLHS couldn’t survive. Great strides in research and care at pediatric institutions such as The Children’s Hospital of Philadelphia changed that. “To see the patients go home and thrive, that’s a huge change,” Ms. Fencl says. “My experience has made me appreciate where I work even more.”
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