Beach baby: UF Health Congenital Heart Center gives young Riley chance for an active life

At first, it felt like the end of the world. Then, together, they rebuilt it.

When Erin found out her daughter, Riley, was diagnosed with Hypoplastic Left Heart Syndrome (HLHS), her family’s vision of the next few months — and years — shifted. The congenital heart condition meant the left side of her daughter’s heart did not develop properly, affecting the blood flow and necessitating a series of complex, delicate heart surgeries beginning almost immediately after her birth, with the Norwood.

A red merle Australian Sheperd lies curled up next to infant Riley.
After Riley’s surgeries, the family’s Australian Shepherds were always by her side.

“It was a huge shock to us, and we were really devastated,” Erin recalled. “We thought we couldn’t have our baby, and by that point in the pregnancy we already had a relationship with her — we saw a whole future. And at that point, it kind of shattered.”

Erin’s stepsister encouraged her to learn more about the UF Health Congenital Heart Center. The CHC included surgeons who specialized in HLHS, like Dr. Mark Bleiweis, the center’s director, and a team of physicians whose collective expertise ensured that the center’s HLHS interstage mortality, or the rate of death after discharge after the Norwood procedure, has remained at 0% for the past decade.

baby riley campbell sits in a kiddie pool with a scar on her chest
Always wanting to play in the water or run on the sand, Riley has been a beach-loving girl from the beginning.

Erin got in touch with Connie Nixon, a clinical nurse coordinator who has worked with the center for almost 30 years. Nixon has seen it all, and has distilled her experience into a formula she doles out to each worried family on their first visit to the center: 1) a piece of advice: to take care of themselves as they go through this with their child, and 2) her cell phone number.

“As soon as we had our first appointment and I met Connie and Dr. Co-Vu, we knew we wanted this team to take care of our daughter. She wasn’t even born yet, but I trusted them — they answered all my questions, and even some I didn’t even know I had!” Erin said. “They said, ‘Call us anytime.’ They felt like family.”

The Campbell family on the fourth of July.

Riley already had two surgeries: The first, her Norwood procedure, six days after birth in December 2018, and the second surgery, called the Bidirectional Glenn procedure, six months later. Each time, Erin recalled, she felt prepared. Her team had informed her of every scenario, and they had a plan for each one; and Rory, Riley’s dad, remained her steadfast rock in between every single appointment, visit, surgery, and recovery.

“I don’t think I would have a fraction of the strength to go through all of this without him,” Erin said.

After Riley’s discharge, the family made the four-hour trip back to their home in Fort Pierce, where any worries they had about their daughter’s recovery were alleviated with the help of an iPad — and Dr. Jennifer Co-Vu’s Single Ventricle Program team.

As the director of the UF Fetal Cardiac Program, Co-Vu has a front row seat to how difficult it can be for families to feel secure after surgery. The UF CHC’s Single Ventricle Program, which she established and now leads, enables families to virtually share their child’s data with their physician.

“Our program understands that part of the Congenital Heart Center’s successful outcomes stem from the connection we are able to provide between family and physician,” Co-Vu explained.

Dr. Co-Vu, left, and Connie Nixon, far right, look forward to each of Riley's visits.
Dr. Co-Vu, left, and Connie Nixon, far right, look forward to each of Riley’s visits in clinic. “The first thing I tell our families is that we’re a family,” Nixon said. “I’ve seen patients for 30 years, and I’ve gone to birthday parties and weddings. We become a part of their lives.”

The home monitoring system allows families to reach their care teams at any time, notifying them of their patient’s weight, how much the baby is eating, pulse oximetry and more.

“They put their trust in us as soon as they walk through our door, and it’s our responsibility and privilege to honor that by providing the best care that we can,” Co-Vu said.

Now, Riley and her family are preparing for her Fontan procedure, the last surgery in addressing her HLHS. There are some jitters, of course — but Erin knows she’s in good hands. Nowadays, Riley is a regular preschool attendee, and loves to swim and go to the beach, flanked by the family’s two Australian Shepherds.

Riley and her dad surf some small waves on the beach.
For Riley and her family, a healthy heart means more sun, sand and waves.

She sports a zipper-like scar on her chest and Erin is so used to seeing it that she forgets it’s not the norm.

In fact, it’s kind of what makes Riley special.

“When I think of past me, in that moment of diagnosis, I just want to tell her that it’ll all happen,”

Erin recalled. “You’ll have those boat rides and golden family days you’re dreaming of.  Going through this will make you and your family strong. You won’t be stuck in a hospital forever. And your daughter will get to be that silly kid who doesn’t have a care in the world.”

About Hypoplastic Left Heart Syndrome (HLHS)

Definition: Hypoplastic left heart syndrome occurs when parts of the left side of the heart (mitral valve, left ventricle, aortic valve, and aorta) do not develop completely. The condition is present at birth (congenital).

What is a Norwood operation?

This occurs within the baby’s first few days of life. The Norwood procedure consists of building a new aorta by:

  • Using the pulmonary valve and artery
  • Connecting the hypoplastic old aorta and coronary arteries to the new aorta
  • Removing the wall between the atria (atrial septum)
  • Making an artificial connection from either the right ventricle or a bodywide artery to the pulmonary artery to maintain blood flow to the lungs (called a shunt)

A variation of the Norwood procedure, called the Sano procedure, may be used. This procedure creates a right ventricle to pulmonary artery connection.

What is a Glenn shunt?

The second operational stage is called the Glenn shunt or hemi-Fontan procedure. It is also referred to as a cavopulmonary shunt. This procedure connects the major vein carrying blue blood from the top half of the body (the superior vena cava) directly to blood vessels to the lungs (pulmonary arteries) to get oxygen. The surgery is most often done when the child is 4 to 6 months of age.

What is the Fontan procedure?

In Stage III, the final step of surgically treating HLHS, surgeons proceed with the Fontan procedure. The rest of the veins that carry blue blood from the body (the inferior vena cava) are connected directly to the blood vessels to the lungs. The right ventricle now serves only as the pumping chamber for the body (no longer the lungs and the body). This surgery is usually performed when the baby is 18 months to 4 years old. After this final step, the child is no longer cyanotic and has a normal oxygen level in the blood.

For more information about HLHS, click here.