To Save a Budding Heart, Innovative Surgery Performed for the First Time in the Pacific Northwest

Shanae Ceja, first pediatric patient in the Pacific Northwest region to undergo an innovative heart surgery called the Ozaki procedure, with Dr. Jonathan Chen, who performed the complex surgical technique.

As she watched her daughter being wheeled into the operating room, a striking memory overcame the flood of anxious thoughts going through Marisela Barragan’s mind.

“Just a few months before the heart surgery, my daughter Shanae was telling me how desperately she wanted to try out for her school’s volleyball team,” said Barragan. “Her doctors were advising against doing any type of strenuous sport because it could damage her heart, so I kept telling her ‘no.’”

“Then she turned to me and said, ‘Mom, please allow me to try out. If I’m going to die, I want to have done something in my life that I loved.’ Those words truly broke my heart.”

Barragan knew the only way her daughter could pursue volleyball along with her many athletic passions, like any other healthy 13-year-old, was to take a leap of faith with an innovative surgical technique that has only been performed on a small number of pediatric patients in the world. Called the Ozaki procedure, the complex surgical technique would help repair and put a stop to the disease that was causing ongoing damage to Shanae’s heart.

Shanae would be Seattle Children’s Heart Center’s first-ever patient to undergo the Ozaki procedure.

“I was urgently searching for answers that would provide healing for Shanae,” said Barragan. “My daughter was dying in front of me and I felt hopeless. But when we learned about this procedure and how it could give her the treatment she truly needed for her heart, it was both a blessing and a relief.”

A disheartening discovery

Shanae grew up in Toppenish, Washington, a small town southeast of Seattle. Being an active and energetic girl, Barragan had no reason to think her daughter’s heart wasn’t normal.

“When she was born, everything seemed to be fine,” said Barragan. “It wasn’t until Shanae was about to turn 9, and I took her to her doctor for a yearly routine check-up that we learned something was wrong. During the exam, I noticed the doctor was listening to her heart for a long time — then he told me that it didn’t sound normal and Shanae needed to see a cardiologist.”

The first cardiologist that examined Shanae’s heart determined that two of her heart valves were leaking, and the valve that appeared more problematic would eventually need to be repaired or replaced.

Shanae was excelling in sports like gymnastics, swimming and cheerleading at the time, and she felt crushed to know she could no longer participate in these activities due to her heart condition.

“After learning about this, Shanae’s health continued to be stable for a while,” said Barragan. “Then two years ago, she started feeling sick, lost a lot of weight and developed a fever that lasted for 10 days. We thought it may be related to her heart issues, but we didn’t have a clear answer.”

“It seemed like we took her to every hospital in our area, and no one knew what was causing her to be so sick,” said Barragan. “It wasn’t until we were referred to Dr. Salaam Sallaam at Seattle Children’s Tri-Cities Clinic that we finally started to get answers.”

Seattle Children’s Tri-Cities Clinic, located in Richland, Washington, serves children and their families in Eastern Washington and the surrounding region, including Benton, Franklin, Lincoln and Walla Walla counties.

“Dr. Sallaam said that the fever may have been caused by her heart valves leaking again,” said Barragan. “They did an ultrasound and found fluid had formed around her heart, which could cause further damage to her heart. She ended up being admitted to a hospital nearby the clinic for treatment.”

A rare diagnosis leads to uncharted territories of treatment

At the hospital, the fluid around her heart accumulated and began to travel down to her left lung.

“Shanae’s condition became so bad that we were life-flighted to Seattle Children’s,” said Barragan. “We spent about five days at the hospital while doctors further investigated the cause of the fever and fluid buildup.”

Barragan learned that Shanae had a throat infection that led the fever to develop, which put stress on her heart causing the fluid build-up. The fever, known as rheumatic fever, is an inflammatory disease that sometimes occurs following a group A streptococcus bacterial infection, like strep throat or scarlet fever. Rheumatic fever happens when the infection is not completely treated with medicine and may affect the heart, joints, skin and brain.

Rheumatic fever may cause serious, permanent damage to the valves of the heart. Most often, it damages the mitral valve, aortic valve or both.

“I remember Shanae had a terrible fever when she was about 7 years old, a few years before they first discovered her heart valves were leaking,” said Barragan. “It occurred to me that this type of fever may have caused the leaking originally.”

Shanae’s formal diagnosis was rheumatic aortic insufficiency, a heart valve disease in which the aortic valve does not close tightly, causing some of the blood to flow backward instead of out to the aorta and the body. This means the left ventricle never fully empties before the next load of blood arrives from the left atrium.

As a result, the left ventricle must expand to accommodate the leftover blood and the new blood. The heart muscle also has to work extra hard to pump the blood throughout the body. The extra work strains the heart muscle and raises the blood pressure in the heart.

“Shanae’s condition wasn’t something we commonly see,” said Dr. Jonathan Chen, chief of pediatric cardiovascular surgery and co-director of the Seattle Children’s Heart Center. “Rheumatic heart disease is relatively rare in patients in developed countries because of treatments like penicillin and advancements in diagnoses.”

Chen and the Heart Center team proceeded with careful evaluation of Shanae’s heart to determine the best option for treatment.

“Typically, I would choose to do the Ross procedure, which involves repurposing a patient’s pulmonary valve to replace the diseased aortic valve, and then putting a donor valve into the pulmonary position,” said Chen. “Because Shanae had evidence of rheumatic disease on several of her valves, you have to make the assumption that the disease has affected all of her valves, which would make doing a procedure like this ineffective.”

Among the other available options included implanting a mechanical valve. However, this method would mean Shanae would have to undergo a lifelong regimen of anti-coagulants, commonly known as blood thinners.

“Shanae wasn’t enthusiastic about that option and neither was I,” said Chen. “We try to avoid using mechanical valves in children at all costs, as there’s a serious risk of bleeding complications, which would mean she couldn’t play any type of contact sport for the rest of her life.”

With the conventional methods off the table, Chen presented an unfamiliar, yet promising, option to Barragan and Shanae.

“I thought, ‘what kind of valve would be the most durable and one that she wouldn’t have to take anti-coagulants for the rest of her life?’” said Chen. “That’s when I knew Shanae could be the right candidate for the Ozaki procedure.”

The Ozaki procedure is an innovative surgical technique 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 procedure 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.

“While the Ozaki has mostly been done in adults, there’s been some enthusiasm in the U.S. about applying this technique to kids within recent years,” said Chen. “About a year and a half ago, Dr. Shigeyuki Ozaki came to Seattle Children’s to teach a course on the procedure using a 3D heart model.”

Seattle Children’s Heart Center is one of the very few centers in the nation trained to perform the Ozaki procedure.

The Heart Center’s outcomes are among the best in the nation for simple to complex heart procedures and transplants for children, and its heart surgeons perform more pediatric cardiac procedures than any other providers in the Washington, Wyoming, Alaska, Montana and Idaho region.

Shanae wouldn’t only be the first patient to undergo the procedure at Seattle Children’s — she would also be the first pediatric patient to receive it in the Pacific Northwest region.

As the Ozaki procedure was being explained to Barragan, she was hesitant about the idea at first.

“I was nervous knowing Shanae would be the first patient to have this surgery, but it really helped that Dr. Chen explained the surgery and recovery process so well that Shanae and I felt confident with the decision,” said Barragan.

Resilience comes from the heart

On Oct. 17, 2017, Chen performed Seattle Children’s first Ozaki procedure.

After a successful surgery, Shanae was transferred to Seattle Children’s Cardiac Intensive Care Unit (CICU) where Barragan noticed her daughter’s steadfast resilience.

“We spent four days in the CICU and I believe there was only one time I ever saw her cry, and it was when she first woke up from the surgery,” said Barragan. “But after that, she was completely back to herself. You couldn’t even tell she went through surgery; she stayed so strong and recovered perfectly.”

Chen is happy with the success of Seattle Children’s first-ever Ozaki procedure, and credits part of it to the bravery shown by the 13-year-old patient who benefited from it.

“The thing I always marvel at with families like Shanae and her mom is their remarkable sense of courage,” said Chen. “It takes a lot of courage to say ‘yes’ to being the first person to undergo a surgical procedure and ‘I trust you’ with your decision to do this.”

A girl’s transformed heart paves the path to innovation

Today, Shanae is putting her athletic skills to the test with a healthy and revitalized heart.

“It’s great to see Shanae being really active again,” said Barragan. “She’s back at school and currently trying out for the volleyball team, participating in practice three times a week.”

For regular check-ups, Shanae sees Sallaam at the Tri-Cities Clinic, which is close to her home.

“I’m so grateful for the way Seattle Children’s treated us through this entire process,” said Barragan. “The care Shanae received was amazing and I wouldn’t change a thing about it.”

Chen is hoping procedures like the Ozaki will open doors to new technology and innovation in the field of pediatric cardiology. He also finds inspiration in caring for patients like Shanae.

“It’s important that we continue to introduce cutting-edge techniques that push the envelope — that’s how the field moves forward,” said Chen. “You develop a profound connection to patients and their families, and with successful outcomes, it’s incredibly rewarding.”

Barragan is overjoyed to see her daughter happy and healthy.

“Shanae feels so proud to be the very first patient to have this procedure at Seattle Children’s,” said Barragan. “It’s given her the ability to do what she loves again.”

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