Double Inlet Left Ventricle and Interrupted Aortic Arch: Hailee's Story

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Not much stops Hailee — at least not for long. She’s had three open heart surgeries and had a pacemaker implanted — all by the age of 5. Despite it all, Hailee remains the active, silly and competitive girl she’s been all along.

Hailee Now 8 years old, Hailee is in third grade. She takes three types of dance lessons, is a cheerleader, Girl Scout, and avid animal lover. Along with her siblings, Hailee cares for her family's variety of pets at their home in Hellertown, PA.

“She’s very adventurous and lives every single day to the fullest,” says Andria, Hailee’s mom. “She wants to do everything — and I have no doubt she will eventually.”

"Hailee is always moving," says Johnathan, Hailee's dad. "She dances and flips around the house. If you just met her, you'd never know she has a heart condition."

Rare combination of heart malformations

Andria and Johnathan learned about Hailee's heart condition before she was born. During a routine 20-week prenatal ultrasound, doctors discovered a problem: One side of her heart wasn't developed.

"They said we had two choices: End the pregnancy or go to Children's Hospital of Philadelphia (CHOP)," Andria says. "There was no debate: We went to CHOP."

At CHOP, the couple met with a team from the Fetal Heart Program and additional tests were performed, including a fetal echocardiogram. Cardiologist David J. Goldberg, MD, explained that their baby had a rare combination of cardiac defects: double inlet left ventricle and interruption of the aortic arch. Their unborn baby had only one pumping chamber of her heart — instead of the usual two — and there was a break in the aorta that blood couldn't flow through.

'Dr. Goldberg drew us a picture of Hailee's heart and said she'd need surgery soon after she was born," Johnathan says.

Because of the need for specialized care before, during and after birth, doctors encouraged the couple to give birth at CHOP's Garbose Family Special Delivery Unit (SDU), the first birth facility in a pediatric hospital for moms having babies with known birth defects.

For the remainder of Andria's pregnancy, the couple were dually monitored by CHOP and their local obstetrician.

Birth and first surgery

On Feb. 18, Andria gave birth to Hailee in the SDU. A team of cardiac and neonatal experts were on hand to immediately care for Hailee, confirm her medical conditions, and administer treatment to support her heart.

When she was 4 days old, Hailee underwent the first of three staged reconstructive heart surgeries. Thomas Spray, MD, then-chief of the Division of Cardiothoracic Surgery, performed a modified Norwood procedure to reconstruct and connect Hailee's aorta.

While Hailee came out of surgery with a functioning heart, her recovery was complicated by her unique combination of heart defects and the fact that her body didn't respond to the typical treatment for children with her condition. Because of this, Hailee was in the Cardiac Intensive Care Unit (CICU) for three months, and needed breathing support for 2 ½ months.

"The three months after Hailee's first surgery were the roughest imaginable. We were so scared to lose her, but she never gave up," Andria says. "Hailee was a fighter — even then."

In May, a few days after Mother's Day, Hailee was discharged. She began intensive physical and occupational therapy to help build her developmental skills and endurance.

Second and third heart surgeries

When Hailee was 5 months old, she returned to CHOP for her second heart surgery, the Glenn procedure. During this surgery, surgeons rerouted the blood supply in Hailee's heart so deoxygenated blood from the upper body would go directly to the lungs instead of passing through the heart.

This time, her recovery from surgery was much smoother. After about two weeks in the hospital, she was back home with her parents and older brother.

Hailee's developmental progress improved dramatically after her second heart surgery. She was soon sitting up, then crawling, and was walking at 13 months old. She continued to receive early intervention services at home to build her developmental skills.

Hailee was 2 ½ year old when doctors determined it was time for her third reconstructive heart surgery, called the Fontan procedure. This time, she was only in the hospital for about a week before going home.

"She really took off after the third surgery," Johnathan says. Hailee began preschool, loved reading, doing crafts, and interacting with her new friends.

Things were going so well that Hailee's parents weren't too worried when they first learned Hailee had a first-degree heart block at age 3 ½. The heart block was found during a routine follow-up visit using an electrocardiogram (EKG), which measures the electrical activity in the heart.

Heart block

During another follow-up visit about a year later, Dr. Goldberg noticed that Hailee seemed "a little off," Andria says. Hailee and her parents said she seemed fine — they hadn't seen any of the typical symptoms that would indicate her heart condition was worsening.

Nonetheless, Dr. Goldberg ordered an EKG. "It showed she had a second-degree heart block," Johnathan says. "But she didn't have any symptoms," Andria adds. "There were no red flags."

Hailee's heart wasn't failing, Dr. Goldberg explained. Instead, her heart was beating too slowly. The electrical currents that tell the heart to contract were partially blocked between the atria and ventricles. Dr. Goldberg suggested a treatment Hailee's parents had never even considered for their 4 ½-year-old daughter: a pacemaker.

"We thought only older people got pacemakers," Johnathan says. "We never once considered she'd need this — as a child — when we learned she had heart problems."

Hailee had the pacemaker implanted in August of 2018 and continues regular follow-up appointments at CHOP every three months. It's important to make sure the pacemaker is working properly and check for any broken wires.

"They've told us it's very rare to fracture the wires in a pacemaker, but Hailee has done it twice already," Andria says.

"We don't know if it's because she's growing or because she's always flipping and dancing around the house. She cannot sit still," adds Johnathan.

Active lifestyle

The past few years have passed in a whirlwind. Hailee is now in third grade and has fully caught up with her peers. She enjoys school, but avoids math whenever possible. She loves dolls, playing dressing up, and watching television, but she's just as likely to get dirty playing in the yard. She takes ballet, jazz and acro dance lessons, and is active in cheerleading and Girl Scouts.

Andria says:

Hailee is very adventurous. She wants to do everything.

Adds Johnathan, "She's also very imaginative. She can make a game out of anything."

Hailee's favorite thing to do? "Hanging out with my family," Hailee says with a shy smile.

Her family includes her parents, brother Dillard, 10, sisters Brielle, 6, and Faye, 2, as well as a dog, pig and bearded dragon.

"It's good we have animals because I want to be a vet," Hailee says.

Hope for the future

Hailee's parents want her to be happy, healthy, and accomplish her goals.

"I want her to be able to explore, to travel, to live her life to the fullest, and do whatever she wants," Andria says, "But I also want her to do it in a safe environment."

They say they're grateful to CHOP for the care Hailee and their family have received — and look forward to a continued relationship with her cardiology team.

2019 Philly Spin-In

Hailee is a patient ambassador for the 2019 Philly Spin-In. Join us at Philly Spin-In — a high-energy, indoor team cycling party to change and save the lives of children with heart disease. It truly is a big workout to help little hearts! Sign up your team for one of the three four-hour, relay-style sessions:

  • Saturday, March 9, 2019: 3-7:00pm
  • Sunday, March 10, 2019: 8 a.m - noon
  • Sunday, March 10, 2019: 2-6:00pm

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