Overcoming the Odds: Azrael's Fight Against Hypoplastic Left Heart Syndrome

When Tiara Thompson, of McAlester, Oklahoma, was 20 weeks pregnant, a routine ultrasound failed to detect any issues with her baby's heart. It wasn't until a last-minute ultrasound at eight months that doctors discovered her unborn son had a serious heart condition.

Tiara's son, Daxton Azrael, was diagnosed with hypoplastic left heart syndrome (HLHS) before birth. HLHS is a rare and complex congenital heart defect where the left side of the heart is severely underdeveloped. Azrael, whose name fittingly means "one who God helps," was not expected to survive.

"They said it was so bad, I would have to go to Oklahoma Children’s Hospital OU Health’s Heart Center," Tiara said. At Oklahoma Children's Hospital, the family met with specialists who confirmed the diagnosis but also witnessed something remarkable – some of Azrael's heart defects appeared to have healed on their own in utero.

"The doctor came in and told me he had never seen anything like it," Tiara said. "There were things in his heart, like issues with his ventricle and septum, that were just gone. There were valve issues that were just no longer there. He had healed himself.”

A Challenging Start

Not only did Azrael heal himself, but his heart would make history, opening a path of healing that can impact the health of other pediatric patients in Oklahoma well into the future.

Despite this in-utero healing, Azrael still had significant heart defects when he was born on Jan. 15, 2019. At just 1 week old, he underwent his first heart procedure to place pulmonary artery bands. This was quickly followed by open-heart surgery at 1 month old: the Norwood procedure, the first in a series of three staged surgeries to treat HLHS.

Pediatric cardiothoracic surgeon Dr. Harold Burkhart, M.D., professor of surgery and Chief of the Division of Cardiac, Thoracic and Vascular Surgery at the University of Oklahoma College of Medicine and director of the pediatric cardiothoracic surgical program at the Oklahoma Children's Hospital, performed all of Azrael's surgeries.

At Oklahoma Children's Hospital, Dr. Burkhart and his team are pioneering innovative heart surgeries that are changing the lives of children born with complex cardiac conditions. One such groundbreaking procedure is helping children like Azrael who are born with only one functional heart chamber.

A Three-Step Journey to a Healthy Heart

To repair Azrael’s condition, Dr. Burkhart and his team, including pediatric cardiologist Dr. Andrew Cave, M.D., planned a three-step procedure for children born with only one functioning heart chamber. The first step, called the Norwood procedure, involves reconstructing the aorta and creating a new pathway for blood flow. This is typically done soon after birth.

"Dr. Burkhart is the best," Tiara said. “The Norwood was particularly challenging. It was one of our hardest surgeries. Azrael was very swollen. His heart rate was erratic. It was kind of a scary couple of days."

After 60 days in the hospital, Azrael was finally able to go home. However, the journey was far from over. The family made weekly trips from their home in McAlester to Oklahoma City for checkups. Eating was a major struggle, with Tiara having to feed Azrael small amounts every hour or two for nearly two years to help him grow and thrive.

At 11 months old, Azrael underwent his second open-heart surgery: the Glenn procedure.

"We take half of the blue blood from the upper body and connect it directly to the lung artery," Dr. Burkhart said. "This allows oxygen-poor blood to bypass the heart and go straight to the lungs for oxygenation."

The final step, the Fontan procedure, completes the circulatory redesign.

"We connect the lower half of the body's blue blood directly to the lung artery," says Dr. Burkhart. "After this, all the oxygen-poor blood in the body goes directly to the lungs, gets oxygenated, and then returns to the heart. This allows the single pumping chamber to function as if there were two."

Innovative Approach for High-Risk Patients

For high-risk babies, Dr. Burkhart and his team have developed an additional step.

"We place bands around the lung arteries to limit blood flow and allow the baby to grow stronger before the Norwood procedure," he said.

This approach, published in the Journal of Thoracic and Cardiovascular Surgery, has shown promising results in reducing risks for these vulnerable patients.

In addition to these surgical innovations, Oklahoma Children's Hospital is participating in a multi-institutional clinical trial using stem cells to potentially strengthen the heart. Dr. Burkhart initiated this trial at Mayo Clinic before joining OU Health.

"We take stem cells from the baby's umbilical cord at birth, process and preserve them, and then inject them into the heart during the Glenn procedure," he said.

While it's too early to draw conclusions, Dr. Burkhart is optimistic.

"So far, we've proven that this procedure is safe. Now we're working to determine if these cells can make the heart stronger in the long term," he said.

The success of these complex procedures relies on a dedicated team of professionals. Nurse navigator Juanita Underwood, R.N., herself a "heart mom" who lost a child to heart disease, plays a crucial role in guiding families through this challenging journey.

Dr. Burkhart's innovative work is not only saving lives but also inspiring the next generation of heart surgeons. Azrael now dreams of becoming a heart surgeon himself, often dressing up like Dr. Burkhart and playing with medical tools instead of toys.

“The staff at Oklahoma Children’s Hospital are the best, and we are so lucky to have them here in Oklahoma,” said Tiara.

Thriving Against the Odds

Today, at 5 years old, Azrael is thriving. He’s happy and friendly and loves hugging his sister and playing with his brother.

"He's healthy. You would never know he had a heart defect unless you just knew," Tiara said. "He's like a little ping pong ball. He's always bouncing off the walls and never runs out of energy.

With his new deep fascination with medicine, Azrael also dresses up in scrubs and a doctor's coat and loves playing with medical supplies.

"When we went to the cardiologist last time, he didn't even want the toy or the sticker,” said Tiara. “They gave him medical supplies to play with."

Azrael's experiences have also shaped his spiritual perspective in touching ways.

"The lady came up to me after church and said, 'Your son said something strange,’” Tiara said. “She had asked them about feeling the presence of God, and he said, 'I have. When the doctor was fixing my heart, Jesus was there. Dr. Burkhart fixed my heart and Jesus helped him.’”

Looking to the Future

While Azrael has completed the planned series of surgeries for HLHS, his journey is ongoing. Children with HLHS often face the possibility of heart transplants later in life. However, Tiara has unshakeable hope about Azrael's future.

"His heart is doing so well for his condition," she says. "I know some [HLHS patients] who are in their 30s and 40s who haven't had to have transplants, who are still going strong. I think Azrael will be one of those."

As Azrael prepares to start school, Tiara credits his health to the care he received at Oklahoma Children's Hospital.

"We love OU Health. It's truly been amazing," she said. The impact has been so profound that Tiara, already a nurse, is returning to OU College of Nursing to further her nursing education.

For parents facing similar diagnoses, Azrael's story offers hope. With expert care, groundbreaking treatments and a strong support system, children with complex heart conditions can not only survive but thrive – running, playing and dreaming of becoming doctors themselves.

“It takes a huge, dedicated team – from nursing to cardiology to critical care to the OR team and anesthesia – to get to where these high-risk kids do well, and I'm just thankful that we have it all here at the Oklahoma Children's Hospital,” said Dr. Burkhart.

Learn more about comprehensive, multidisciplinary pediatric care at Oklahoma Children’s Hospital or speak to an expert by calling (405) 271-KIDS or visiting OklahomaChildrens.org.