Research collaboration gives hope for future of babies with rare heart defect

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Dec 19, 2017 | Suzanne Ferguson | @suzannerferguson

"He's amazing," Andrea Sexton says, beaming about her infant son. "He is the strongest little boy ... If you were just looking at him, you'd never know anything was wrong."

Sexton and her husband, Heath Sexton, never imagined that would even be possible for their son Ryals after he was diagnosed with a congenital heart defect while still in the womb.

"All of a sudden, to see the ultrasound tech blinking and staring at the monitor, and then getting a little frantic and rushing out to get the doctor, I knew something was wrong," Heath Sexton says.

The diagnosis: hypoplastic left heart syndrome, or HLHS. Essentially, there was no left side of baby Ryals' heart.

It's a relatively rare condition. Only about a thousand children are born with HLHS each year in the U.S.

Coming home from the hospital we were wondering how is this going to change our lives ... Obviously, the care of a child that possibly would need a lot of help, maybe a lot of long-term help," Heath Sexton says. "And then, of course, in the back of your mind, obviously, you've read that a lot of children don't make it past three months. And, so, just thinking about, you know, am I going to be prepared ... for a funeral?"

The Sextons packed up and temporarily left their home in Alabama to move to Philadelphia. The plan was to place Ryals' hopes of survival in the hands of a team of doctors at Children's Hospital of Philadelphia (CHOP), one of the leading medical centers for HLHS surgeries and treatment.

There they met Dr. Joe Rossano, who heads up the cardiac center at CHOP.

"Yeah, they're a very complicated surgery," Dr. Rossano says. "You know, prior to the 1980s and '90s, there were essentially no good surgical options for these patients. But a number of very innovative surgical techniques were developed that have allowed many of these children to survive and thrive."

Children born with HLHS go through a series of three surgeries to essentially rework the plumbing of the heart. The first surgery comes within days of birth. The second surgery generally occurs three to six months later. And the third surgery is usually performed about three years later.

This series of surgeries allows children with HLHS to live relatively normal lives, but it isn't perfect.

Having the right side of the heart perform the tasks normally handled by both sides of the heart puts tremendous stress and pressure on the right side.

As patients with HLHS are aging, doctors are realizing many of their hearts are unable to continue to function on their own, and some patients need a heart transplant.

But Andrea Sexton found out about a clinical trial going on at Mayo Clinic aiming to solve that problem.

"Quickly they set me up with an interview over the phone with Dr. Nelson," Andrea Sexton says. "He was amazing. He gave me hope."

Dr. Tim Nelson oversees the Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome. He leads a team of roughly 60 people who research and conduct clinical trials for new HLHS treatments.

Read the rest of the story on the Mayo Clinic News Network.

The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of delaying or preventing heart failure for individuals affected by congenital heart defects including HLHS. The specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies.