Cedars-Sinai Specialists Are Champions for the Most Vulnerable Patients

Frances Jones thought she knew everything about the care and feeding of babies. While awaiting the birth of their fourth child last summer, Jones and her husband, Marcus, felt relaxed and confident. After all, they were experienced at juggling the needs, rivalries and scraped knees of their 12-, 7- and 4-year-olds.

But, following a routine ultrasound when Frances was 23 weeks into her pregnancy, doctors told the Joneses their new son, Bryce, would be born with a heart defect called tetralogy of Fallot.

"We were in shock for hours," Jones said. "Neither of our families has heart disease or anything remotely like that, so it really scared us. We didn't know what to do."

Fortunately, Jones' team of doctors at her community hospital had a plan. Step 1: Put Jones on bed rest so her baby could be born as close to full term as possible. Step 2: Put the Guerin Family Congenital Heart Program in the Cedars-Sinai Heart Institute and Department of Pediatrics on standby just in case the baby's condition worsened.

At first, Bryce did fine when he was born in August at just 32 weeks' gestation, although he weighed barely 3 pounds. But Bryce's underlying heart disease began causing episodes where he wasn't getting enough blood flow to his lungs. His pediatric cardiologist realized that Bryce needed a procedure to improve pulmonary blood flow.

"We work closely with community hospitals and pediatric cardiologists throughout Southern California to provide the highest level of personalized care for the most vulnerable babies," said Evan Zahn, MD, a recognized international authority on minimally invasive techniques to repair structural disorders in children’s hearts. "With the most advanced treatments from the moment of diagnosis, including advances in nonsurgical techniques, we can be a lifesaving resource to give these babies the best chance for a normal, healthy life."


"It's very humbling to know that there are doctors out there who really treat children as though they were their own," said Frances Jones. With her and Bryce are Alistair Phillips, MD, co-director of the Guerin Family Congenital Heart Program, (left) and Shreya Sheth, MD, a pediatrician with the program.

Zahn co-directs the Guerin Family Congenital Heart Program with pediatric cardiothoracic surgeon Alistair Phillips, MD, known for developing novel treatment approaches that reduce the need for multiple open-heart surgeries. Their patients range in age from infancy through adulthood because even after Zahn and Phillips treat newborns’ cardiac emergencies, most born with structural heart disease require specialized care throughout their lives.

Bryce's diagnosis, tetralogy of Fallot, is a condition characterized by four structural heart defects that prevent sufficient oxygen from reaching the blood. Infants and children with that condition usually have blue-tinged skin due to oxygen deficiency.

These "blue babies" were the first congenital heart patients treated surgically in the 1940s. However, it wasn't until the 1960s that a permanent surgical correction was developed. Today, most of these children will receive surgical correction within the first year of life. Yet for babies like Bryce who are premature and tiny, there are very few options to improve pulmonary blood flow.

"Traditional approaches for very small babies that are premature can be performed but are very high-risk procedures. These babies are very fragile and are at increased risk of having organ dysfunction, notably brain injury and kidney injury, especially if cardiopulmonary bypass is needed," Phillips said.

Bryce was transferred to Cedars-Sinai under the care of Zahn and Phillips, each of whom played a significant role in developing and performing a minimally invasive procedure. "We made a 1-centimeter incision in the baby's lower chest. Through that, we were able to get access to the heart and we were able to put a stent into place and increase blood flow to the lungs," Phillips said.

"The advantage to the hybrid procedure is that the baby would not have to recuperate from open-heart surgery, we avoid the need for cardiopulmonary bypass, and it could be done through a small incision that will be part of the larger incision when he has his full repair," Zahn said. "He could therefore grow bigger and stronger; his other organs will be more mature and unlikely to be injured."

His parents plan for Bryce to undergo a complete repair procedure when he's about 6 months old.


Bryce Jones was born with a heart defect called tetralogy of Fallot. The news frightened his parents, Frances and Marcus. But once Frances Jones met the doctors at
Cedars-Sinai, “I knew they had Bryce's best interests in their own hearts,” she said.

Giving approval for the procedure, believed to be the first of its kind in Southern California, was tough on the Joneses. "We were petrified — there's no other word for it," Frances Jones said, "but once I met Dr. Zahn and Dr. Phillips, I knew they had Bryce's best interests in their own hearts. I knew we had to go forward."

Two days after the procedure, baby Bryce was discharged from Cedars-Sinai and transferred back to his community hospital in the San Fernando Valley, the same hospital where the doctors and the nurses recognized the need for an intervention. Within a week, Bryce was home in Santa Clarita and his siblings were fighting over who could hold him. By the time he was 2 months old, Bryce tipped the scales at close to 7 pounds, took in 5 ounces of milk every four hours and, it seemed to his mother, smiled constantly.

He's scheduled for regular checkups with Zahn as he grows strong enough for his next procedure. And throughout his life, Bryce will require focused care. To accommodate the healthcare needs of growing patients like Bryce, the Guerin Congenital Heart Program provides treatment to patients of all ages with structural heart disease.

"It's very humbling to know that there are doctors out there who really treat children as though they were their own," Jones said. "I think of Dr. Zahn and Dr. Phillips every day when I tell my 12-year-old that he needs to study hard so he can be a cardiologist like them."

Android app on Google Play