Makenna Maskey

“I would love for parents to see MaKenna,” says her mother, Lois Ann Maskey. “I am pretty proud of her. She’s such a good kid.”

Mom wishes to boast not of MaKenna’s musical ability, however, but the health and life she has enjoyed since she was born with a complex heart disorder.

MaKenna was diagnosed with hypoplastic left heart syndrome almost immediately after her birth in a hospital near her home in Wellsville, Mo. She came to SSM Health Cardinal Glennon Children’s Hospital when she was one day old.

“When we went through this, I didn’t know what to expect,” Lois Ann says. “I had never read about hypoplastic left heart. We didn’t meet any older kids with the same condition. We didn’t know what the outlook was.”

“We were thinking the worst,” adds her father, Ryan.

Hypoplastic left heart results from the incomplete development of the heart’s left-side structures, leaving the heart unable to pump an adequate blood supply throughout the body. As the right side of the heart struggles to pump blood through the lungs and body, babies born with the defect begin to turn blue within a few hours of birth. Without surgical intervention, the condition is not survivable.

The anatomy of MaKenna’s heart was studied and diagrammed by Saadeh Al-Jureidini, M.D., director of cardiac catheterization and interventional programs in the division of pediatric cardiology and the Dorothy and Larry Dallas Heart Center at SSM Health Cardinal Glennon. A physician at the center since 1984, he is a professor of pediatrics at Saint Louis University School of Medicine.

After her diagnosis, MaKenna underwent the first of three surgical procedures that altered her heart’s anatomy to enable it to better pump blood through the lungs and body. The surgeries were performed by Andrew Fiore, M.D., director of cardiothoracic surgery at SSM Health Cardinal Glennon since 1995 and professor of surgery at Saint Louis University School of Medicine. MaKenna underwent the final operation when she was 4 years old.

“She was blue a lot until the third surgery. She couldn’t do physical activities at all. Her lips would turn blue and we would have to tell her to sit down,” her mother says. “The third surgery was amazing.”

“After the third surgery she was pink,” her father agrees. “She turned into a regular kid.”

Such interventional cardiology procedures and medications will continue to improve the outcomes for heart patients, sometimes with reduced need for surgery,” Fiore says. “Some of the defects we used to repair in the operating room are done in the operating room. They are going to be done in the cardiac catheterization laboratory by the cardiologists or by cardiologists working together with the surgeon.”

In recent years, interventional cardiologists and surgeons have realized the need for another evolution. Occasionally, an interventional procedure could not be completed because the defect was nearly impossible to access via catheterization. In other cases, the surgeon realized the benefits of performing his work with fluoroscopy, which produces highly-detailed, real-time X-ray images that enable the cardiologist to guide his instruments.

MaKenna is now a seventh-grader at Wellsville-Middletown R1 School. She participates in most school activities but sometimes has to take a break during physical education classes.

MaKenna takes piano lessons and plays drums in her school band. She practices piano daily. “I can’t play sports so the piano is my thing,” she says.

“Piano is very important to her. She has a great piano teacher,” Lois Ann says. “She performed at our church at Christmas.”

MaKenna is an honors student who loves school and working with kids. She tutors first-graders after school and helps with the children’s ministry at her church. She also loves to write and thinks she would like to become an author of children’s books.

MaKenna comes back to SSM Health Cardinal Glennon every December for an annual exam. She has been seeing cardiologists Al-Jureidini and Fiore all of her life.

“I remember some things, but not all things” about her hospitalizations and surgeries, MaKenna says. “I remember my mom rocking me and pulling me around the hospital in a wagon.”

“She remembers the good stuff,” Lois Ann says. “We enjoy coming back here to see all the people. Dr. Fiore was a wonderful doctor. We love him. Dr. Al-Jureidini has been with her since day one. He is the best. We had awesome nurses here. I don’t remember a bad experience.”

When MaKenna returned for her second and third surgeries, the Maskeys met families whose children were newly diagnosed with hypoplastic left heart syndrome.

“They got to meet MaKenna and talk to her and see her outlook. That would have been reassuring for us,” says Lois Ann. “I want parents to see how great she is doing and know there is a future for these kids.”