Tetralogy of Fallot: Ben's Story

The memory of seeing their unborn baby for the first time stays with parents forever. The first time Corey and Diana saw their son Ben, Diana was 22 weeks pregnant. 

They had come to her obstetrician’s office for a routine ultrasound. And they were expecting nothing but good news.

But as the ultrasound technician brought up images of their unborn baby on the screen, they realized something was wrong.

ben “She kept going back in on the same area, zooming in and out and taking images from different angles,” says Corey.

Diana’s doctor told them there was something wrong with their baby’s heart.

A few days later, the couple came to Children’s Hospital of Philadelphia to meet with the clinicians of the world-renowned Fetal Heart Program, who are experts in diagnosing and managing congenital heart disease (CHD) before a baby is born.

While a prenatal diagnosis of CHD is an incredibly stressful event for expectant parents, it provides them with an opportunity to understand more about the diagnosis, and to learn about treatment options and outcomes.

In addition, infants who receive a prenatal diagnosis of CHD have been found to be more stable and have better outcomes than those whose CHD is not detected before birth. When parents and clinicians know about a CHD diagnosis before birth, they are better able to plan and prepare to ensure that the infant’s transition from prenatal to postnatal life is as smooth as possible.

Prenatal diagnosis of tetralogy of Fallot

At CHOP, Diana had a fetal echocardiogram through the Fetal Heart Program, which performs more than 3,500 such studies each year.

She and Corey met with program coordinator as well as pediatric cardiologist Stanford Ewing, MD, who confirmed that their baby had a type of congenital heart disease called tetralogy of Fallot (TOF). TOF is a condition characterized by a constellation of four different heart anomalies.

In addition, Ewing explained that their baby’s left pulmonary artery was not connected to his lung. He would need open-heart surgery to repair both problems in the first few days of life.

“It was a lot to take in,” says Corey, “but Denise put us at ease. She explained that it’s something the team is fully equipped to handle.”

“When we first got the diagnosis,” says Diana, “we had no idea what it was, no idea what it meant.”

Luckily, the couple had the support of the experts in the Fetal Heart Program, who provided them with all of the information they needed.

“We were always asking questions,” Diana says. “And if we didn’t understand something, they’d explain it again.”

It also helped that, as a designer on CHOP’s Web team, Corey was very familiar with the Hospital — and he knew how much families trusted and appreciated the doctors and nurses at CHOP.

“We knew we were in the right place,” he says.

Expert cardiac care in the first days of life

Ben was born Jan. 18, 2006, at the Hospital of the University of Pennsylvania. Minutes later, he was transferred to the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU) at Children’s Hospital of Philadelphia, where Corey and Diana were able to visit him the next day.

Being separated from their baby is a heart-wrenching experience for new parents — especially when they are facing a frightening diagnosis like tetralogy of Fallot. But today, couples like Corey and Diana have an important option: CHOP’s Garbose Family Special Delivery Unit (SDU), the world’s first birth facility for mothers carrying babies with known birth defects.

The SDU is just steps away from the CICU, so mothers are never far from their babies and can visit them often. Close to half of the babies born in the SDU each year are patients of the Fetal Heart Program, whose clinicians work closely with SDU staff to ensure optimal care for both mother and baby.

Open heart surgery to repair tetralogy of Fallot

When Ben was 2 days old, Thomas Spray, MD, chief of cardiothoracic surgery at CHOP, repaired the baby's tetralogy of Fallot and connected his left pulmonary artery to his lung during an intricate open-heart surgery. Ben spent the next week recovering at CHOP with his parents and extended family by his side.

Like most babies with congenital heart disease, Ben required special care even after he went home. He had regular sessions with speech, physical and occupational therapists, and his parents had to keep track of how much he ate and how often he needed a diaper change. They shared that information with his pediatrician, Deborah White, MD, during follow-up appointments at CHOP Care Network Primary Care in Chestnut Hill, PA. Ben also had regular appointments with Ewing, who remains his primary pediatric cardiologist and whom he continues to see once a year.

“Dr. Ewing is easy to talk to,” says Corey. “We really feel like he knows us — and we know him.”

Growing up — and going strong

When he was 2 1/2 years old, Ben had a catheterization procedure to expand his left pulmonary artery, and the Cardiac Center team will continue to monitor his health as he grows up. He takes medicine to strengthen his heart muscle, and will eventually need surgery to replace the heart valve he received as a baby.

But for now, this happy, energetic 10-year-old is focused on fifth grade and enjoying some of his favorite things: camping, video games, music, Pokémon, and hanging out with friends.

Ben’s heart isn’t holding him back in any way. He loves the thrill and speed of massive rollercoasters at Hersey Park (which doctors approved). He regularly skateboards in his neighborhood, and plays pickleball at the local Y.

“If you met him, you wouldn’t know that he’s been through so much,” says Corey. “He’s a normal kid — and that’s all we can ask for.”


Originally posted: August 2012; updated September 2016
 


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