Lifesaving Care for HLHS: Thomas’ Story

Photo/Jenni Fritz Photography Sarah and Yaniv’s first pregnancy was routine, but their 20-week anatomy scan at Bryn Mawr Hospital showed signs that their son’s heart wasn’t developing as expected.

Through Main Line Health’s alliance with CHOP, families on the Main Line receive CHOP newborn and cardiac care at hospitals close to home. At Bryn Mawr Hospital, the couple was referred to CHOP’s Fetal Heart Program. There, Thomas was diagnosed with hypoplastic left heart syndrome (HLHS), a serious congenital heart defect in which the left side of the heart is underdeveloped. Typically, the left side of the heart pumps oxygenated blood into the aorta and throughout the body, but in Thomas’ case, the left ventricle was too small to function correctly. This syndrome is fatal without early intervention. HLHS patients undergo a series of three staged reconstruction heart surgeries between the ages of 2 days and 4 years to reconstruct the cardiac circulation and allow support of both systemic and pulmonary circulation.

Through the Fetal Heart Program, Yaniv and Sarah gained a dedicated team of fetal sonographers, fetal cardiologists, nurse coordinators and social workers to guide them through every step of their child’s treatment.

“What stands out after the initial diagnosis was the shock,” says Yaniv. “Our doctors told us that we couldn’t know exactly what to expect until the baby developed more.”

“Because we had the same team, there was a lot of consistency in the information we were given, which helped us better understand what was happening,” says Sarah.

It’s complicated information, she says, and Dr. Shivani Bhatt, MD, an attending physician in CHOP’s Cardiac Center, and Dr. Jack Rychik, MD, Director of the Fetal Heart Program, explained everything in layman’s terms to make it easy to understand.

After the success of Thomas’ first surgery, Yaniv and Sarah made the decision to share their story publicly – Yaniv is the mayor of Conshohocken, and the couple has been very outspoken about their journey since that point.

“We intentionally chose to share our story publicly because of how helpful it was during our journey to talk to other families who had gone through this before,” says Yaniv.

Starting the treatment journey

Due to the severity of his heart defect, Thomas was given only a 50% chance of making it to his first operation. Every four weeks, the couple went in for an echocardiogram to evaluate the structure of Thomas’ developing heart.

“All of us were learning about his condition as he developed, and [the medical team] was straightforward about all of the possibilities,” says Yaniv. “We were initially concerned that the lungs wouldn’t develop properly, as there was a concerning atrial septal defect. At every scan, they pulled out an image and a marker to walk us through every detail of how his heart was developing and what it should look like.”

“Every week, we felt encouraged by the growth and stability we saw in the scans,” says Yaniv. “But we knew we wouldn’t know the full extent of his condition until he was born.”

Thomas was born on Jan. 18, 2023, in CHOP’s Center for Fetal Diagnosis and Treatment’s Garbose Family Special Delivery Unit (SDU), which offers specialized care before, during and after delivery for babies prenatally diagnosed with birth defects. It’s the world’s first birth facility in a pediatric hospital specifically designed for that purpose.

“I was so scared, wondering what they were going to find,” says Sarah. “And then he came out, and I heard him cry, and all I could think was, ‘Thank God. He has lungs.'”

Staged surgeries

After he was born, Thomas gained a new team at CHOP, spearheaded by Dr. Emmanuelle Favilla, MD, a CHOP pediatric cardiologist. At 2 days old, Thomas underwent the first of the three surgeries – a Norwood procedure, which ensures that blood flow to the lungs and body is stabilized and controlled.

“The hardest part was handing Thomas over to the surgery team and watching him be wheeled away,” says Yaniv. “He wasn’t even 48-hours-old.”

The Norwood procedure is a temporary solution intended to keep the child’s circulation stabilized until the second surgery a few months down the line. Because his heart was not stable during this time, Thomas required intense monitoring. He spent the next two weeks recovering in the Cardiac Intensive Care Unit (CICU). That’s when he entered CHOP’s Infant Single Ventricle Monitoring Program, which provides specialized care and monitoring for high-risk infants between the first two surgical palliation procedures. The program’s goal is to prevent life-threatening events and improve growth between the first and second operations among children with single ventricle heart defects. Yaniv and Sarah worked with Therese M. Giglia, MD, FAHA, ISVMP Medical Director; Alyson Stagg, MSN, CRNP, nurse practitioner and Program Director; and a team of pediatric cardiologists and dietitians.

“Babies are tough, but you never know how their bodies will respond,” says Sarah. “We worked with the program to keep a close eye. Every day, we communicated with our team, checking his weight, his pulse oxygen levels, medication responses, incision healing – anything that gave us a glimpse into how he was recovering.”

“This is our first child, so it was hard enough to figure out life with a newborn, let alone one in such a vulnerable position,” says Yaniv. “They helped us set up appointments, track medications, track stats, recognize side effects or signs that something isn’t right. It was comprehensive and intense, but it made us feel extremely supported from all angles.”

At 4 months old, Thomas underwent the second reconstructive surgery – the Glenn operation, which reconstructs the heart’s venous system to redirect deoxygenated blood flow from the upper body and brain, to enter the lungs without passing through the heart. The operation went smoothly, but a complication during recovery prompted doctors to manage his surgical site incision more intensely. After another month of recovery, Thomas was finally able to go home.

Finding a new normal

Yaniv, Thomas and Sarah on Fayette St, Conshohocken. Photo/Mike Gray Foto. Now 8 months old, Thomas is out of the continuous monitoring phase and living as close to a normal infant life as possible.

“He can do just about anything a typical baby can do,” says Sarah.

Around the time Thomas turns 3, he’ll return for the third and final surgery – the Fontan procedure, which performs a similar function as the Glenn operation, but reconstructs the heart’s major venous system to allow deoxygenated blood from the lower body to enter the lungs without passing through the heart and further increase the amount of oxygenated blood that can then be delivered to the body.

In the meantime, Thomas has graduated from the Infant Single Ventricle Monitoring Program. He is now followed by CHOP’s Cardiac Kids Developmental Follow-Up Program, which provides developmental screening, evaluation and coordinated care for children with complex congenital heart disease.

Sarah and Yaniv are continuing to raise awareness by sharing Thomas’ story and by connecting with other families whose children have congenital heart defects.