Chenoah and Katie's Challenging Journey: Overcoming Hypoplastic Left Heart Syndrome

Chenoah Long’s pregnancy was high-risk. She had gestational diabetes, and as her last baby had been born with a genetic heart defect, she required careful monitoring. While the potential of Chenoah having another baby with a heart condition was very low, it was still a possibility.

At the 21-week ultrasound in Tulsa, an abnormality with her baby’s heart was detected, and Chenoah was sent to Oklahoma Children’s Hospital OU Health Heart Center. Further investigations showed that Chenoah’s baby did have a heart defect, and it was serious — she had Hypoplastic Left Heart Syndrome (HLHS).

In the United States, approximately 1 in 3,955 babies are born with HLHS each year, and this defect accounts for 23% of cardiac deaths during the first week of life.

What is Hypoplastic Left Heart Syndrome?

Hypoplastic Left Heart Syndrome (HLHS) is a rare and serious condition where the left ventricle, the heart’s main pumping chamber, doesn’t develop properly during pregnancy. This affects the mitral valve, which controls blood flow between the left atrium and ventricle, and the aortic valve, which allows blood to flow from the heart to the body. Both valves are either missing or very small. Babies born with HLHS need surgical intervention soon after birth, although surgery is not a cure.

During pregnancy, a fetus has two openings between the left and right sides of the heart, known as the patent ductus arteriosus (PDA) and the foramen ovale.

In babies with HLHS, the left side of the heart cannot effectively pump oxygen-rich blood to the body. In the first few days of life, the oxygen-rich blood bypasses the poorly functioning left side of the heart through the PDA and foramen ovale. The right side of the heart then pumps blood to both the lungs and the rest of the body. However, once these openings close, it becomes difficult for oxygen-rich blood to reach the rest of the body.

What Are the Signs & Symptoms of Hypoplastic Left Heart Syndrome?

A baby born with Hypoplastic Left Heart Syndrome (HLHS) may exhibit several signs and symptoms, including:

• Fast breathing
• Blue or grayish coloring of the skin and nails
• Trouble feeding
• Low energy and activity levels
• Fewer wet diapers than normal

What Causes Hypoplastic Left Heart Syndrome?

HLHS is a congenital defect that occurs while a baby is developing in the womb. The exact cause is unknown, but it is believed to result from a combination of genetic and environmental factors, including the baby’s DNA.

How is HLHS Treated?

Chenoah was induced at 38 weeks pregnant because of gestational diabetes, and on November 6, 2017, she gave birth to a 10-pound baby girl she named Katie. Research shows that babies with HLHS who have a normal birth weight and aren't premature do better than babies with lower birth weights. Katie’s birth weight meant that she had a good start going into her first procedure for HLHS.

The surgical treatment for HLHS involves three stages to improve blood flow and heart function:

1. Norwood Procedure: This first surgery is typically performed within the first 3-4 days of life. The right ventricle is reconfigured to act as the main pumping chamber, a shunt is placed to direct blood flow to the lungs, and the aorta is reconstructed to ensure blood flow to the body.
2. Glenn Procedure: The second surgery usually occurs between 4-6 months of age. The superior vena cava is connected directly to the pulmonary artery, allowing oxygen-poor blood from the upper body to flow directly to the lungs without passing through the heart.
3. Fontan Procedure: The final surgery, performed between 2-4 years old, completes the series. The inferior vena cava is connected directly to the pulmonary artery, enabling oxygen-poor blood from the lower body to flow directly to the lungs without going through the heart.

While there is no cure for HLHS, these surgeries significantly improve oxygen levels and heart function. Children with HLHS require regular follow-up care to manage their condition effectively.

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 performed Katie’s first surgery when she was only seven days old.

Caring for a HLHS Baby

When Katie was born, she was placed in the neonatal unit (NICU) at Oklahoma Children’s Hospital. Not only did Katie have HLHS but she also was born with lung problems and required a breathing tube. She was placed under the care of pediatric cardiologist, Dr. Arshid Mir, M.D., Chief of Pediatric Cardiology, Head of Cardiac Imaging, and associate professor in the Department of Pediatrics at the University of Oklahoma College of Medicine.

Dr. Burkhart performed Katie’s procedures, and she recovered well from each one, however, she still struggled with lung problems.

“Katie’s lungs were so sick. She would get these air pockets in her lungs,” said Chenoah. “After she recovered from her heart surgery the doctors would take her breathing tube out, and then she would crash so it would have to go back in again. Every time we took her home, she’d get a cold or the sniffles, and while we had oxygen at home for her, sometimes it just wasn't enough, so we'd end up back in the hospital.”

Katie was in Oklahoma Children’s Hospital for about a year being treated for HLHS and her lung condition. At times Katie’s lungs were so frail her surgery would be postponed until they were stronger. Chenoah was overwhelmed by the treatment she and Katie received.

“The nurses were incredible,” Chenoah shared. “One day we got caught up doing things and I missed lunch. One of the nurses found out that I didn't eat that day, and she gave me her lunch that she had packed from home. And I'll never forget her. Little things like that happened all the time.”

Continued Care for Katie

Children with Hypoplastic Left Heart Syndrome (HLHS) require intensive medical care from birth, including numerous follow-up doctor visits, tests, and surgeries.

“I’ve known Katie since before she was born, as a fetal patient, and she is now age six,” said Dr. Mir. “She was diagnosed with Hypoplastic Left Heart Syndrome (HLHS), and shortly after birth, she underwent a series of surgeries — four in total over the first three to four years of her life. Remarkably, she has done very well following these surgeries. Her final surgery was the Fontan procedure, and she has continued to thrive, even with additional catheter-based interventions.”

Dr. Mir typically sees Katie every six to nine months because she is at a slightly higher risk than most HLHS patients. Katie has an older sister and brother. Her brother, who was born with a genetic heart defect, is now 7 years old and is strong and healthy, and has been cleared to participate in any sport he chooses.

“From the get-go, the pediatric cardiology team at Oklahoma Children’s Hospital didn't hold back the punches,” said Chenoah.” “They let us know how serious Katie’s condition was, but they were always super supportive, very compassionate, and they looked after us so well.”

World Class Pediatric Heart Center

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 survival rate for children undergoing open-heart surgery at Oklahoma Children’s Hospital stands above the national average, with a 98.8% overall survival rate for all cardiac surgeries, regardless of their complexity. This remarkable achievement highlights the Oklahoma Children’s Hospital’s commitment to providing the highest level of care for young patients.

In addition, Oklahoma Children’s Hospital is part of the HLHS Consortium, and is one of 12 institutions across the country dedicated to finding breakthroughs to treat HLHS.

“The heart program at Oklahoma Children’s Hospital is unique,” said Dr. Mir. “Our heart program began almost 10 years ago when Dr. Burkhardt and I came to Oklahoma, and over the past decade we have gradually assembled an outstanding team of providers, including nurses and other specialists. We’ve grown from a small program into one of the larger programs in the country.

We care for children with heart disease of all ages, from infants to adults. Our program has steadily progressed and is now ranked among the top in the nation. Last year, out of 125 programs, we were ranked 37th—a remarkable achievement for a relatively new program. We aim to climb even higher in the rankings this year. Our pediatric cardiology program offers a full range of subspecialties, ensuring that children in Oklahoma receive the best possible care without needing to leave the state. We provide heart procedures and treatments that are on par with the best in the country.”

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 diagnostic tests, clinic visits, and the pediatric cardiac multidisciplinary team at OU Health by calling (405) 271-4411.