World-first treatment for rare heart defect saves baby born at Packard Children’s

Linda Luna was five months pregnant with her first child when she got the bad news: Ultrasound scans showed a deadly defect in her baby boy’s heart. He had a 90 percent chance of dying before or just after birth. But thanks to a groundbreaking treatment at Lucile Packard Children’s Hospital Stanford, two-month-old baby Liam, who just went home to San Jose last week, is beating those odds.

He is the first baby in the world successfully treated with prenatal maternal hyper-oxygenation for his rare heart defect: congenital Ebstein’s anomaly. This week, several local news outlets report on the success of Liam’s case.

The problem at diagnosis? Due to severe leaks in two heart valves, blood flowed backward through the right half of Liam’s heart. His heart became dangerously enlarged. Too little blood reached his lungs and the rest of his body. Left untreated, the defect would cause irreparable heart and lung damage.

“Once you see type of leakage Liam had, it’s usually a progressive process,” said Theresa Tacy, MD, the fetal cardiology specialist who treated Liam in concert with his mom’s high-risk obstetrician, Katherine Bianco, MD, and a team of other specialists from across the hospital. “It just gets worse,” Tacy said. “The fetus eventually develops heart failure and dies.”

The team gave expectant mom Luna 12 hours per day of oxygen therapy for the last three weeks of her pregnancy. The idea was to relax Liam’s lung blood vessels with the extra oxygen he’d get from his mom. This would make it easier for his heart to pump blood forward into his lungs and, the doctors hoped, let him survive until birth and surgery.

“We were trying to offer Liam’s parents hope but also remain realistic that their baby had a very high chance of not making it,” said cardiologist David Axelrod, MD, who cared for Liam in the cardiovascular intensive care unit after he was born. “We knew that even if he made it through pregnancy, his risk of dying during his first few days of life was very high.”

Immediately after his Nov. 22 birth, the doctors put Liam on an ECMO machine that delivered oxygen to his blood. Cardiothoracic surgeon Frank Hanley, MD, also closed a blood vessel near the heart to help Liam’s blood to flow forward. Finally, 11 days later, Liam was strong enough for a Dec. 3 surgery in which Hanley fully repaired his heart.

“It was a huge operation for a tiny baby fighting for his life,” Luna said. “The seven-hour wait during surgery was the longest wait of my life, but when they finally wheeled him out, he was a different baby. We were so thankful.”

Earlier this week, Luna and her husband, Jose Silva, returned to the hospital with baby Liam for a happy reunion with their doctors.

“I made a vow to stay strong for him,” Silva told ABC-7 news as he and Luna described everything that has happened since Liam’s heart defect was first diagnosed.

Said Tacy, “This is just one case, but it’s so exciting to move forward and feel a glimmer of what we think is the right path for treating other babies with this devastating heart defect.”

Photo of physicians and family courtesy of Lucile Packard Children’s Hospital Stanford; image of normal heart anatomy and blood flow (left) vs. blood flow in Liam’s heart before birth (right) drawn by Theresa Tacy, MD. (Red arrows show regions where blood flowed backward in Liam’s heart.)