Iowa Baby Receives Complex Heart Surgery at Oklahoma Children's Hospital OU Health

Twenty weeks into her second pregnancy, Cedar Rapids, Iowa, resident Haley Copeland and her husband Steven were told that their unborn son Colton had Hyperplastic Left Heart Syndrome (HLHS), a congenital heart disease.

HLHS is a serious and rare condition where the baby’s main pumping chamber of the heart — the left ventricle — doesn’t form correctly during pregnancy. The mitral valve which controls blood flow between the left atrium and ventricle is either missing or very small. So too is the aortic valve which allows blood to flow from the heart to the body. Babies born with this heart condition require surgical repairs soon after they are born but surgery is not a cure.

Steven and Haley were advised to find a center of excellence to deliver their baby as the heart center in Cedar Rapids was not equipped to handle Colton’s needs. They transferred to the University of Iowa Children’s Hospital but at that time the hospital didn’t have a pediatric heart surgeon. Haley reached out to friends who had children with congenital heart defects for recommendations and she was given the name of OU Health pediatric cardiothoracic surgeon, Dr. Harold Burkhart, M.D., professor and Division Chief of Cardiac, Thoracic and Vascular Surgery in the Department of Surgery at the University of Oklahoma College of Medicine.

Seeking Expertise at Oklahoma Children’s Hospital OU Health

Haley’s pregnancy was further complicated as she has Type 1 diabetes and she developed preeclampsia and HELLP syndrome with her first pregnancy. Preeclampsia is a pregnancy condition marked by high blood pressure and protein in the urine that can lead to a serious complication called HELLP syndrome. HELLP syndrome involves a breakdown of red blood cells, called hemolysis, elevated liver enzymes and low platelet levels. The fact that Haley had preeclampsia and HELLP syndrome with the first pregnancy meant that she was at high risk for them again.

Steven and Haley traveled to OU Health in Oklahoma City to see an obstetrician-gynecologist for pregnancy care, and Dr. Burkhart for a consultation about Colton’s heart defect. However, as Haley’s pregnancy was such high risk and with her living 10-11 hours away, and unable to fly, the decision was made to find the care she and Colton needed, closer to home. This led Haley to a hospital in Minnesota, where Colton was safely delivered on October 29, 2023.

Surgery for Colton

About 1 in 3,955 babies in the United States are born with HLHS each year with the defect responsible for 23% of cardiac deaths during the first week of life.

The surgical treatments of HLHS involves three procedures to improve blood flow and heart function.

1. Norwood Procedure: This is the first surgery out of the three and it is usually performed within the first 3-4 days of life. During this procedure the right ventricle takes over as the main pumping chamber, a shunt is placed to direct the blood flow to the lungs, and the aorta is reconstructed to allow blood flow to the body.
2. Glenn Procedure: The second surgery is typically performed between 4-6 months old. In this procedure the superior vena cava is connected directly to the pulmonary artery which allows oxygen-poor blood from the upper body to flow directly to the lungs without going through the heart.
3. Fontan Procedure: The final surgery is performed between 2-4 years old and completes the series of surgeries. During this procedure the inferior vena cava is connected directly to the pulmonary artery allowing oxygen-poor blood from the lower body to flow directly to the lungs without going through the heart.

There is currently no cure for HLHS, but the three surgeries help improve oxygen levels and heart function. A child with HLHS requires regular follow-up care to help with the management of the condition.

Colton had his first surgery for HLHS at only eight days old — the Norwood procedure. The procedure was performed at Children’s Minnesota Hospital – Minneapolis, and it was a success. Colton healed well and was able to go home with Haley and Steven on December 21, 2023.

The Second Procedure

When it was time for Colton to have the Glenn procedure, he was taken to see the pediatric cardiac specialists at Mayo Clinic in Rochester, Minnesota. Colton’s pulmonary artery was too small, and his aortic arch needed repairing. Performing both the Glenn and the aortic arch repair together is a highly specialized and complex dual surgery. The Mayo Clinic surgeons sent out a call for help to the best pediatric cardiothoracic surgeons in the country, and Dr. Burkhart answered the call.

Steven and Haley took Colton to Oklahoma Children’s Hospital OU Health to undergo the complex surgery. The aortic arch is a critical part of the aorta’s structure and would normally be repaired separately from the Glenn procedure. Dr. Burkhart had the experience and expertise to perform both procedures at once, to save Colton from undergoing two surgeries.

“The surgery Colton needed was complex and high-risk,” said Dr. Burkhart, who has performed the dual surgery previously. “Many pediatric cardiac surgeons may want to avoid the risk to perform such an operation or may not have the clinical experience to do both together.”

Dr. Burkhart and the pediatric heart care team performed the successful surgery on April 16, 2024, and Colton was able to go back home to Iowa with his family just over a week later.

A Bright Future

At eight months, Colton is thriving. He meets most of his milestones and is a happy and healthy little boy who loves his big brother dearly. Colton requires the final procedure for HLHS — the Fontan, in the next few years to complete the surgical treatment. With the surgery that Dr. Burkhart performed, Colton is in good stead for the Fontan. He will require regular checkups after the third procedure, but he can live a life with few restrictions.

“The multidisciplinary approach is essential to our success,” explained Dr. Burkhart. “Our heart center is the only center for pediatric heart surgery in the state of Oklahoma. We are a high-volume program with excellent outcomes. We are a multidisciplinary team focused on the care of cardiac kids and we have the expertise and resources to deal with the most complex conditions.”

Oklahoma Children’s Hospital Heart Center is one of only 120 pediatric cardiac programs in the nation. The cardiothoracic surgical team has achieved outstanding surgical results well above the national average — a distinction very few programs in the country have achieved. The percentage of children who survive open-heart surgery at Oklahoma Children's Hospital stands above the national average, with a 98.8% overall survival rate for all who receive cardiac surgery, regardless of how complex the surgery is.

“We love Dr. Burhart and are so grateful that he was willing to help us when other agencies were unsure of what to do,” said Haley. “We can’t say enough about Dr. Burkhart and how talented he is.”

World Class Care at Oklahoma Children’s Hospital Heart Center

The Oklahoma Children’s Hospital Heart Center provides world-class care for children with cardiac problems and those in need of a heart transplant, close to home.

Learn more about the Oklahoma Children’s Hospital Heart Center services and treatment, request an appointment or get a second opinion, or find out more about the pediatric cardiac multidisciplinary team at OU Health by calling (405) 271-4411.