Tricuspid Atresia: Olive's Story

Pediatric Care in Florida

Patient Story Highlights

  • Olive was born with a heart defect called tricuspid atresia and had only half of a functioning heart.
  • Olive underwent two heart surgeries and recovered well.
  • After Olive's surgeries, her skin began to turn a rosy, healthy pink.
Olive joining in a special Heart Walk at All Children’s Hospital
Olive joining in a special Heart Walk in November that the CVICU team put on for patients, families and staff.

A peaceful, productive morning in the cardiovascular intensive care unit (CVICU) of Johns Hopkins All Children’s Hospital …

Doctors are completing their rounds, nurses are multitasking, caring for patients and offering encouragement to families with some of the sickest children.

There is healing in these halls.

But something else, too. The faint strains of music, growing louder now … 

Not the gentle, tinkly tunes one might expect inside a children’s hospital, but grand, sweeping orchestral strains that progress to crescendo and swallow up the steady beeps and hums of the medical machinery in patients’ rooms.

Could that be … the Indiana Jones theme song?

Ah, yes. That’s just Olivia. She prefers “Olive.” Coming down the hall. Barely 4-years-old and all of 3 feet tall, she is pushing a toy shopping cart and sporting a 6-inch scar on her chest as if it were a statement necklace. Nurses pop out of their respective patients’ rooms to wave hello as she passes by.

Olive’s two favorite movies are Indiana Jones and Star Wars. She plays the theme songs when she takes her walks. And whenever else she can.

“I never thought I’d be ‘that mom’ begging her little girl to watch Frozen,” her mom, Kayla, says with a laugh.

Not on Olive’s playlist. Everybody on the CVICU floor knows that. They also know she likes to make people laugh. And they know she holds a special place in her heart for strawberry milk.

Olive’s heart … that’s where her story really begins.

Olive’s Heart

Olive was born with a heart defect called tricuspid atresia. It occurs when the heart’s tricuspid valve, between the right upper and right lower pumping chamber, doesn’t form correctly in the womb. Her left ventricle was well-developed, but her right ventricle barely developed at all.

Essentially, Olive had only half of a functioning heart.

“The result is an inability to divide the heart into a right and left side,” says Michelle Miller, M.D., a pediatric cardiologist based at Johns Hopkins All Children’s Outpatient Care, Sarasota. “So, her oxygenated and de-oxygenated blood — or her ‘red’ and ‘blue’ blood — mix as they leave the heart, some of it going into the lungs and throughout the body.”

The heart defect is life-threatening without intervention. A child may require two or three surgeries, in stages, to repair the defect and ensure there is an adequate blood supply to the lungs.

Olive spent the first 12 days of her life in Johns Hopkins All Children’s Olive spent the first 12 days of her life in Johns Hopkins All Children’s neonatal intensive care unit (NICU).

Fortunately, she was able to go home without requiring immediate surgery as a newborn.

But at a couple months old, a follow-up exam revealed troubling news. Olive’s blood oxygen levels were roller-coastering downward. It was time to act.

The unusual heavy fog on that fall day prevented the hospital’s transport team, The unusual heavy fog on that fall day prevented the hospital’s transport team, LifeLine, from taking Olive by air, so a ground team tenderly took the baby by ambulance to Johns Hopkins All Children’s. Two-month-old Olive underwent her first life-saving heart surgery — called a Glenn. A Glenn shunt connects the main vein of the upper body directly into lung circulation, allowing blood to get to the lungs for oxygen., from taking Olive by air, so a ground team tenderly took the baby by ambulance to Johns Hopkins All Children’s. Two-month-old Olive underwent her first life-saving heart surgery — called a Glenn. A Glenn shunt connects the main vein of the upper body directly into lung circulation, allowing blood to get to the lungs for oxygen.

Olive recovered well from the successful Glenn procedure — but her journey to wellness was not over.

Good Timing

Kayla knew her daughter needed one more major heart surgery to give her the very best chance at a full life. In the fall of 2020, Kayla received some welcome news from her daughter’s cardiologist. 

After practicing for three years at Kentucky Children’s Hospital and Cincinnati Children’s Hospital Medical Center, one of the country’s leading pediatric cardiothoracic surgeons, James Quintessenza, M.D., had decided to return to Johns Hopkins All Children’s where he had previously practiced for more than two decades.

The timing could not have been more perfect.

Olive was scheduled for a procedure called a Fontan. Sometimes referred to as the “completion” surgery for these patients — the Fontan procedure disconnects the large vein normally carrying deoxygenated blood from the lower body to the heart — and connects it directly to the pulmonary artery.

“Without the Fontan, she’d still have only half of her blood going through lung circulation,” Quintessenza says. “Her oxygen levels would remain too low. With the Fontan procedure, she’ll have almost all of her blood going through the lung circulation.”

Olive’s surgery was scheduled for Nov. 10, and as fate would have it, weather once again played a starring role. Tropical Storm Eta was moving through the Gulf and was threatening the Tampa Bay area. The Sunshine Skyway was closing.

The family traveled a day early to be in place for Olive’s big day.

The morning of her surgery, Olive’s mom and dad joined her to give her a kiss before the surgical team wheeled her back through the double-doors. 

It was an emotional moment for Kayla.

“I leaned over and said to her, ‘I love you!’” Kayla recounts. “Olive just looked at me … and then she burst out laughing. ‘Oh, mommy,’ she says.”

Perhaps it was the meds. But Kayla thinks maybe it was just Olive being Olive.

A Future

The Fontan procedure went beautifully, with members of the surgical team updating the family throughout the day to share their progress.

By the following morning, Olive was up and walking, asking for her preferred musical selections to accompany her.
“The nurses really made us feel like our hospital room was our little home away from home,” Kayla says.

Eight days after her surgery, Olive was able to go home.

Her family has delighted in watching their little girl get stronger each day.

“She wants to run, jump, tumble,” Kayla says. “She wants to go at 120 percent now.”

Historically, children born with tricuspid atresia did not survive past the first year of life.

But current procedures allow them to live well into adulthood, and perhaps into old age. Fontan procedures became a standard practice for these defects in the 1980s, and now those patients are in their 30s and 40s. These heart patients can have complications requiring other interventions as they move through life, but the future has never looked brighter for children who are born with tricuspid atresia.

“We hope and believe this will be a great long-term benefit for her,” Quintessenza says. “And if she needs things in the future, we will be here for her.”

Pink

There are moments in a child’s medical journey, which, however small, hold great significance for those who love them.

One such moment for Olive came shortly after her surgery. Olive’s skin had always looked “blue,” a common trait of kids with tricuspid atresia, due to their abnormal oxygen content. Olive had looked “bluer” than most.

But after her surgery, for the first time in her life, Olive’s skin began to turn a rosy, healthy “pink.”

“To see her pink like that,” Miller says, “it was remarkable. It was very emotional.”

Kayla is encouraged — and hopeful.

“She is perfect,” Kayla says. “I hope she stays strong.”

For Olive, the odds have now greatly improved for a fuller, healthier life — complete with adventures of her own making.

She will surely pick just the right epic soundtrack for her journey — and set it all to music.


Cardiovascular Intensive Care Unit at Johns Hopkins All Children's Hospital

Kids hand
Newborns, infants, children, and young adults with congenital heart defects, cardiovascular disorders, and those recovering from heart surgery or transplant receive care in the cardiovascular intensive care unit (CVICU) at Johns Hopkins All Children's Hospital in St. Petersburg, Florida.