Difficult decisions: Baby with rare condition needs complex heart surgery

Kayla Read was 22 years old, thrilled to be expecting her first child, when a doctor’s appointment led to a frightening revelation. “When I was about 20 weeks pregnant, they saw something on the anatomy scan.”

Something – but it was unclear what. That led to appointments with maternal fetal medicine specialists in the Upstate of South Carolina, near her home in Anderson, and then to a heart doctor who suspected the baby had a rare condition called Ebstein’s anomaly.

That doctor knew Read needed a higher level of expertise for what would be a birth followed by the need for immediate medical attention. So she referred the young mother to the Pediatric Cardiology team at the MUSC Shawn Jenkins Children’s Hospital.

Highly ranked heart program

Read didn’t know it at the time, but that hospital’s heart program was ranked No. 4 in the country by U.S. News & World Report and would soon move up to No. 2.

 

The heart team achieved that, in part, by taking on cases that not many other hospitals would attempt because of their complexities and achieving excellent results. Its outcomes are No. 1 nationally. (Outcomes refers to how well patients fare.)

Read just knew she wanted her baby to get the best outcome possible. Rowan was born April 23. His medical journey was just beginning. “We were in the NICU for nine days,” Read said, referring to the neonatal intensive care unit. “They kept a check on his heart, did echocardiograms. They thought everything looked fine. He was eating fine. So they sent us home.”

Rowan’s doctors wanted him to grow a little more before they performed surgery on a heart that was about the size of a large acorn. But they weren’t able to wait for long.

Rowan’s condition worsens

“We ended up going back to MUSC about two months later because he was having trouble eating. He started eating about an ounce. When we brought him home at nine days, he was eating 3 ounces,” Read said.

 

Minoo Kavarana, M.D., division chief of Pediatric and Adult Congenital Cardiothoracic Surgery at the Medical University of South Carolina, remembered just how sick the baby was. “When Rowan presented, he was breathing really fast, like a freight train, not gaining weight, not tolerating feeds.”

Eric Graham, M.D., Children's Heart Program Chair in Pediatric Cardiac and Congenital Heart Disease at MUSC Children’s Health, described the condition that was causing so much trouble for Rowan.

“Ebstein's is when the tricuspid valve, the valve on the right side of the heart, is displaced down into the ventricle. That can make the right ventricle muscle thin and weak. The valve usually leaks a lot and causes the right atrium to become large and dilated. This results in blood essentially going backward into the right atrium instead of going out to the lungs as it's supposed to.”

Doctors and parents face an important decision

Kavarana and his team had some decisions to make in terms of how to try to repair the baby’s heart. Graham said the surgeon was thorough in exploring all possibilities, including positives and potential problems.

 

“What really impressed me with Dr. Kavarana is that he asked all our surgeons' and cardiologists’ opinions; he went back and forth and actually called several surgeons across the country to get their opinions as well before deciding what was the best operation for Rowan. I think that's part of the secret to achieving great outcomes. You put egos aside and do everything you can to provide the best care for the child.”

Kavarana also consulted Read and Rowan’s father, Brandon Cooley. Then, they settled on what they believed was Rowan’s best option.

“The most common and most successful operation is something called a Cone repair, which is reserved for older, larger children,” Kavarana said.

Younger babies that are smaller are typically treated using a single ventricle approach, with long-term outcomes that Kavarana said are not as good.

 

“The Cone repair involves taking down the entire tricuspid valve, rotating it, reconstructing it and remodeling the right ventricle because the right pumping chamber becomes large, baggy and dilated. In addition to this, we had to close the hole in the heart known as a VSD, which added to the complexity. So overall, this was a significantly complex operation,” the surgeon said, then explaining that VSD stands for a ventricular septal defect.

Adding to the complexity, according to Graham: the baby’s fragile state. “Working on valves … valves are like tissue paper, especially in babies. And so they're very hard to do operations on because they're so thin and can tear very easily.”

Kavarana had never performed a Cone repair on a patient so young. “I think there are very few surgeons and centers in the world that have attempted to successfully perform this operation in a baby that size.”

But Kavarana felt his team had the experience to do it – and, thanks to his efforts, a wealth of advice from colleagues across the country.

Read was still terrified. “Of course, I tried to stay positive. But as a parent, you’re automatically going to think the worst. I was a nervous wreck.”

Kavarana and the team operated on Rowan on July 22. The eight-hour surgery was a success, to everyone’s relief. “Dr. Kavarana made it into a normally functioning heart,” Read said with amazement.

 

Kavarana said Rowan’s outlook is strong. “He is gaining weight. His valve looks good. His heart function is actually very good. So it's a very normal life that he's looking toward in the future.”

Graham called it another example of why the heart program has earned not only high rankings but also high regard from parents and peers at other heart centers. “We are an extremely collaborative team that works together to provide the highest-quality care tailored to every individual,” he said.

“We offer the entire breadth of options from the relatively simple to the most complex; there’s nothing we send away. And no matter how well we have done it before, we are always striving to be better.”