Continuity of Care Makes Future Brighter for Young Heart Patient
Jul 01, 2018 Cedars-Sinai Staff
During Lara Borda's first seven years of life, her parents would listen to her heartbeat every night as part of their bedtime ritual.
We would tuck her in, read her a story, and then listen to that rhythm," said her mother, Regina Borda.
They adopted that routine because their little girl's future was seriously in doubt. Lara was born with a heart condition — aortic stenosis — that threatened to limit her growth and development and prompted different doctors to prescribe conflicting courses of action.
"We opened the aortic valve as a life-saving procedure. We knew it would not be her last procedure, but it would give her the time she needed to grow for the next surgery."
"Lara is a beautiful, healthy and active little girl," said Evan Zahn, MD, director of the Congenital Heart Program. "But this is a condition she is going to have for her whole life. And because of the way we do things here, she will always have this cohesive and comprehensive medical team behind her."
As with all congenital heart disease, Lara's condition will require life-long monitoring. But thanks to the Congenital Heart Program's unique approach, which treats patients from birth through adulthood, Lara will have continuity of care from the same team.
In fact, the Borda family's continuity with Zahn began shortly after Lara and her twin brother were born in Miami.
Lara's brother, Esteban, was fine.
"The doctors thought she was cold, so they put her in an incubator," Regina said. "And when her color didn't change they tried another incubator. Finally, someone listened to her heart."
A cardiologist ordered an ultrasound of the 1-day-old infant's heart and the diagnosis arrived: aortic stenosis, a condition in which narrowing of the aortic valve slows blood flow between the heart and the aorta. Instead of providing a pathway for oxygenated blood to flow from the left ventricle of the heart to the body, the malformed aortic valve creates a barrier.
The cardiologist told Regina and her husband, Carlos, that Lara would need immediate surgery. He referred them to Zahn, then a heart specialist at Miami Children's Hospital and an internationally recognized authority on minimally invasive techniques to repair structural disorders in children's hearts.
"When babies are born with aortic stenosis, they are deathly ill," Zahn said. "Unlike in adults, there is no good medical therapy for them, so we took Lara right to the cardiac catheterization lab for surgery."
In healthy hearts, the aortic valve is composed of three flexible leaflets that control blood flow between the left ventricle of the heart and the aorta, Zahn said. In Lara's case, two of those leaflets had fused together, which blocked blood flow into the aorta and caused leakage of blood back into the left ventricle.
The long-term fix for Lara's aortic stenosis would be a Ross procedure, an open-heart surgery in which her malformed valve would be replaced. However, because her infant heart would quickly outgrow an implanted valve, the approach was to manage her condition until she was large enough for the surgery.
Seven days after Lara was born, Zahn performed a balloon valvuloplasty on the infant, a procedure in which a catheter is threaded into the heart and a small balloon is then inflated to enlarge the valve opening. Though not a permanent repair, the procedure restores a reasonable amount of normal function and gives infants time to grow.
"We opened the aortic valve as a life-saving procedure," Zahn said. "We knew it would not be her last procedure, but it would give her the time she needed to grow for the next surgery."
That next surgery came seven years later, a span of time during which the Borda family moved from Miami to Germany, and then, in 2010, to Southern California.
When Lara was 6, the family met with a cardiologist who wanted to perform surgery immediately. Instead, the family reached out to a family friend at Cedars-Sinai for a referral.
"He called and told us about this new cardiologist at Cedars-Sinai who came from Miami — and it was Dr. Zahn," Regina Borda said. "We were so happy. There was never any question that this was where we were going to take Lara."
Zahn and the medical team in the Congenital Heart Program evaluated Lara's condition and concluded that immediate surgery was not necessary. Instead, they monitored Lara for a year with regular exams, MRIs and ultrasounds. When Lara continued to do well, the team postponed her open-heart surgery for another year to give her even more time to grow.
"Lara was a small girl, but her parents are small, and she was a twin, so we were able to rule out her heart condition as the main reason for her small size," Zahn said. "And although there was leakage in the aortic valve, children are able to tolerate that for quite a long time."
The Congenital Heart Program's multidisciplinary team — made up of 30 doctors, nurses, technicians and health professionals — met four times over two years to thoroughly evaluate Lara's case. During that time Lara grew from 42 pounds to 55 pounds, which made a significant difference in her chest size, Zahn said.
When Lara was 7, doctors concluded it was time to perform the Ross procedure to replace Lara's damaged aortic valve.
In June 2014, three months before she turned 8 years old, a surgeon performed the Ross procedure on Lara, replacing her badly formed aortic valve with her own pulmonary valve, which was then replaced with a valve from a human donor. By waiting, the team was able to place an adult-sized pulmonary homograft, which, when it deteriorates, can be replaced through a minimally invasive approach, not a reoperation.
Lara's open-heart surgery took eight hours and went flawlessly. For her mother, the stress of awaiting the outcome was significantly alleviated by the Congenital Heart Program team's practice of calling a patient's family with hourly updates.
"They called us and let us know everything was going well," Regina Borda said. "It was so nice, so much better than sitting there for eight hours and not knowing."
Since the surgery, Lara has grown in size, has more stamina, and is more active. Weekly check-ups have given way to monthly check-ups. Soon, six-month check-ups will turn into annual visits.