Although Katarina Anja, or “Katja,” De Groot was born with a rare heart defect called truncus arteriosus, the active toddler never let her condition stop her from running, playing and going to the park.
However, when she was 2 years old, Katja began sleeping for most of the day and withdrawing from the activities she usually enjoyed. That was when her mother knew something was wrong.
“She wasn’t herself anymore,” said Jennifer De Groot. “My daughter is usually a happy-go-lucky child who loves to live life to the fullest. I didn’t know how much longer we could see her like that. It’s like we had reached the point of no return.”
De Groot’s concerns grew, and she knew she needed to seek help for her daughter. Dr. Maggie Likes, Katja’s cardiologist at Seattle Children’s, noticed a difference in her echocardiogram. It showed her aortic valve was not functioning properly.
“We had hit a tipping point,” Likes said. “Between the concerning changes on her echocardiogram and what her mom was seeing at home, it was time to do something about her aortic valve.”
De Groot knew her daughter wouldn’t be able to lead an active lifestyle with a mechanical valve because it would mean that Katja would have to undergo a lifelong regimen of anti-coagulants, commonly known as blood thinners. She made a bold move and asked the Seattle Children’s Heart Center team if they could perform an innovative surgical technique that would repair her daughter’s aortic valve – an unexpected suggestion that led to a collaborative decision.
At 2 years old, Katja would become the youngest child at Seattle Children’s to undergo the Ozaki procedure.
From happy-go-lucky to miserable
It all started when De Groot learned that her baby had truncus arteriosus at her 20-week ultrasound while the family lived in Chicago.
In babies with truncus arteriosus, the aorta, the main artery of the body that carries oxygenated blood from the heart to other parts of the body, and pulmonary artery, which carries deoxygenated blood from the heart to the lungs, are joined, forming a large common artery. Babies with this condition also have a ventricular septal defect, a hole in the wall of her heart that allows oxygen-rich blood from the left ventricle to mix with oxygen-poor blood from the right ventricle.
The poorly oxygenated blood is then pushed out to the rest of the body, causing a baby with this condition to be blue or cyanotic.
The other main problem is that the lungs may get too much blood. This makes breathing hard, and it can lead to high blood pressure in the lungs, or pulmonary hypertension, and heart failure.
“Everything changed from being excited about a pregnancy to being terrified knowing that my daughter would be born with a rare congenital heart defect,” said De Groot.
Shortly after she was born, Katja had surgery to create two separate arteries and close the hole in the wall of her heart.
She returned home 10 days later and quickly recovered.
“Katja is a rock star,” De Groot said. “People prepared us to expect developmental delays and eating issues. From day one, she’s hit every milestone early or on time. Three days after she came home, she rolled over. Her heart condition has never held her back.”
In April 2017, the family relocated to Seattle for a job opportunity, and Katja’s care was transferred to Seattle Children’s.
At their first appointment, Likes noticed changes in Katja’s echocardiograms. As months went on, the tests showed that Katja’s aortic valve was narrowing and leaking. Her left ventricle was starting to dilate and worsening with time.
“Her valve was being problematic and not functioning well,” said Likes. “Something needed to take that load off her left ventricle.”
In December 2017, De Groot began noticing changes in Katja’s behavior at home that suggested her heart was taking a toll on her.
“She was tired, less active, more irritable, and her fingers would turn blue,” said De Groot. “She was just miserable all the time.”
After hearing about Katja’s symptoms at home, Likes agreed that it was time to intervene. De Groot remembers the gratitude she felt.
“Dr. Likes took what we had to say seriously and knew it was time to move forward,” said De Groot. “It was invaluable having somebody that truly listens and takes into account what you are seeing as a parent on a day-to-day basis, because nobody knows your child better than you do.”
Since she was small for her age, Katja needed to grow before doctors could perform surgery, so the family waited until spring 2018.
Katja’s aortic valve needed to be repaired or replaced. Conventional options include a mechanical valve, which would limit her activity, and a tissue valve, which is tissue typically taken from a cow or pig and could degenerate within about five years. Another option was the Ross procedure, which involves replacing the damaged aortic valve with the pulmonary valve, but that would not work because Katja did not have a pulmonary valve.
“We wanted her to live as normal of a life as possible,” De Groot said.
De Groot had read about the Ozaki procedure, and she suggested it as a potential option – something the Heart Center hadn’t considered for Katja because it would have to be adapted in a way it had never been done before.
“I made an offhanded joke,” said De Groot. “I knew many children’s hospitals don’t do the Ozaki procedure, but I said, do you think you’d be up for making some kind of bizarre procedure and ‘Frankensteining’ her a valve?”
The family was surprised to learn that Seattle Children’s performed the Ozaki procedure, and had been the first in the Northwest to provide the technique to a pediatric patient. The innovative surgical technique was developed by Dr. Shigeyuki Ozaki at the Toho University Ohashi Medical Center in Tokyo, Japan. Unlike the conventional valve replacement of implanting a mechanical or prosthetic valve, this technique uses the patient’s own pericardium, which is the membrane that surrounds the heart. Using a piece of the pericardium, a surgeon will cut out three leaflets with a stencil specifically designed for the Ozaki procedure. These leaflets are then used to reconstruct the aortic valve.
“We agreed that this was her best shot at continuing to be a normal child who can run, jump, and play,” De Groot said. “It was nerve racking. It’s hard enough to send your child to open heart surgery, but it’s harder when the procedure involves unknown territory.”
Hospital staff helped alleviate the family’s fears. To prepare Katja for the procedure and prevent separation anxiety, an anesthesiologist nurse gave Katja stickers and a mask swabbed with a bubble gum scent. Before her surgery, Katja was able to decorate her mask and practice putting it on.
The Ozaki procedure has been used in just a handful of Seattle Children’s patients, with Katja currently the youngest patient at the hospital to have the procedure.
“We have used the Ozaki procedure in adolescents and teens, primarily because of their active lifestyles,” said Dr. Muhammad Nuri, a cardiac surgeon at Seattle Children’s who assisted with Katja’s Ozaki procedure. “Repairing Katja’s valve wasn’t possible and she was a very energetic child, which is why her parents opted for the Ozaki procedure.”
Typically, the aortic valve has three leaflets that allow blood to eject out of the heart. During the Ozaki procedure, surgeons remove the old leaflets and measure the size of each. Based on the width of the leaflet, surgeons use a template and create new leaflets out of the pericardium. Since Katja’s pericardium was used up in a previous surgery, doctors used bovine pericardium instead.
“If you don’t size the leaflets well, the template is imperfect and the valve is going to leak,” Nuri said. “We approached this intervention thoughtfully and reached out to Professor Ozaki for his opinion.”
Like one-quarter of children with truncus arteriosus, Katja had four leaflets, creating a new challenge for her surgeons.
“The Ozaki procedure has been described for two and three leaflets but does not have a template for a four-leaflet valve,” said Nuri.
For the procedure, the Heart Center team identified Katja’s two largest leaflets and sized them, creating two templates. Then, they combined her two smaller leaflets into one leaflet, creating three.
“It’s reassuring that we’re able to provide the Ozaki procedure as an option to families,” said Nuri. “We might not have embarked on this endeavor if the family had not brought up this option because it was a unique application. Sometimes, the families tell us the best way to go about this.”
De Groot’s idea to “Frankenstein” a valve for Katja had become a reality.
“Katja’s story is an example of the collaboration we like to have at Seattle Children’s,” Likes said. “Parental input helps us make decisions, because there’s often more than one right way to do something. What is right for the child depends on a combination of the family and the medical team.”
Back to normal
By the next morning after her five-hour surgery, Katja was back to her normal self.
“Katja is a very tenacious and determined child,” De Groot said. “She’s a fighter. The first thing she asked for after surgery was water, and the second thing she asked for was a hot dog. That was when we knew she was okay.”
By the next morning, Katja was eating breakfast and happily chatting with the nurses. Four days later when she returned home, she was back to being a lively toddler. Within several days of being home, Katja even tried to “surf” down the stairs on her stomach.
“You can’t even tell she had open heart surgery now,” said De Groot. “She is running and tumbling around, and doing all the things she loves to do, which wouldn’t be possible if it weren’t for this procedure. It’s wonderful to see how happy and healthy she is now, and I am so thankful that Seattle Children’s was able to do this.”
Likes also noticed the transformation in Katja after the procedure. Her aortic valve is now functioning properly and her left ventricle has returned to a normal size.
“Before the surgery, I remember Katja’s mom telling me she just really wanted her kid back,” Likes said. “It was fun to see how much more energetic Katja was and how good she felt after we got things back under control. She was the kid her mother had remembered.”
Although her daughter experienced a rough start at life, and will continue to have her heart valve function monitored through echocardiograms, De Groot now knows Katja has a bright future ahead of her.
“It’s been an absolutely wild journey,” said De Groot, “but through it all, Katja is stronger than ever. We’re grateful that her care team at Seattle Children’s gave her a second chance at life.”