In November 2014, Pete drove Natalie the five hours to Philadelphia so she could spend the next few weeks close to The Children’s Hospital of Philadelphia, where she was planning to give birth. But shortly after he returned to their home in Virginia, Natalie called: He needed to come back right away. Their baby was going to be born the next day.
Prenatal diagnosis and care at the Fetal Heart Program
Natalie and Pete had made their first trip to Philadelphia when Natalie was about 31 weeks pregnant. They had just learned that the baby Natalie was carrying had transposition of the great arteries (TGA) — a form of congenital heart disease (CHD) in which the aorta and the pulmonary artery are attached to the heart in the wrong places — and a pediatric cardiologist had referred them to the Fetal Heart Program at The Children’s Hospital of Philadelphia.
On that first visit to CHOP, Natalie had a comprehensive evaluation with the Fetal Heart team and with the obstetrical team from CHOP’s Center for Fetal Diagnosis and Treatment (CFDT). The evaluation included a fetal echocardiogram, which provided detailed information about her baby’s heart, as well as a level 2 ultrasound, which provided detailed information about all of the baby’s other organs. That same day, Natalie and Pete also met with the team that would be caring for Natalie and their baby: Denise Donaghue, RN, MSN, coordinator of the Fetal Heart Program; Shobha Natarajan, MD, a pediatric cardiologist; Jennifer Diem-Inglis, MSW, LSW, a social worker; and Juan Martinez-Poyer, MD, a maternal-fetal medicine specialist at the CFDT.
During that first meeting, the couple learned that their baby would need open heart surgery in her first week of life. There was also a chance, the team explained, that immediately after birth she would need a balloon atrial septostomy, a lifesaving procedure that was created at CHOP in the 1960s.
“It's very difficult to predict before birth who's going to need the balloon procedure,” says Jack Rychik, MD, medical director of the Fetal Heart Program. “That's why all babies with TGA should deliver at a place where an evaluation can take place immediately at birth and, if needed, the balloon procedure can be performed.”
CHOP is one of the few hospitals in the world that is fully equipped to care for babies with TGA and their mothers. The Hospital’s Garbose Family Special Delivery Unit (SDU), a first-of-its kind facility designed for mothers carrying babies with prenatally diagnosed birth defects such as CHD, is located on the same floor as the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU) and the Cardiac Operative and Imaging Complex, so babies can undergo lifesaving procedures immediately after birth — and parents are never far from their newborns. Natalie and Pete knew that CHOP was the best place for their baby — and for them.
Over the coming weeks, Natalie and Pete traveled from Virginia to CHOP two more times to meet with the Fetal Heart team. They toured the SDU and the CICU, and spent hours talking to Dr. Natarajan, Dr. Rychik, Donaghue, Diem-Inglis, and the other experts who were part of their care team.
“They were really great at explaining everything to us, including their thought process. They really took the time to answer all of our questions,” says Natalie. “We knew we were in good hands.”
A very special delivery — and a lifesaving procedure
Just a few days after Natalie relocated to Philadelphia for Ayla’s birth, her CHOP OB/GYN team discovered that her amniotic fluid was dangerously low – and scheduled her c-section for the next day.
Ayla was born in the Special Delivery Unit on Nov. 11, 2014. A large team that included obstetricians, midwives, nurses, neonatologists and anesthesiologists was there to stabilize Ayla and care for her and her mom. “There was a lot going on,” says Natalie, “but everyone was so calm. We felt very reassured.”
Minutes after Ayla was born, the medical team determined that she would, in fact, need a balloon atrial septostomy. They rushed her to the nearby Cardiac Operative and Imaging Complex, where interventional cardiologist Andrew Glatz, MD, performed the balloon procedure to stabilize Ayla prior to her open heart surgery.
After the procedure, Ayla’s medical team brought her to Natalie’s room in the SDU, and she and Pete were able to hold her for the first time.
Arterial switch operation to repair transposition of the great arteries
Two days later, Ayla had open heart surgery to repair her TGA. During the surgery, called the arterial switch operation, Thomas Spray, MD, chief of the Division of Cardiothoracic Surgery, reconstructed Ayla’s heart so that her aorta and her pulmonary artery were attached to the heart in the correct places.
CHOP’s team of four cardiothoracic surgeons performs more than 20 arterial switch operations each year, giving children with TGA a chance at a healthy life. With this surgery, says Dr. Rychik, “Surgeons can essentially take transposition of the great arteries, which is a lethal condition, and turn the heart back into something that’s almost identical to a normal heart.”
Ayla recovered from her surgery quickly. “Every day, she would improve so much. She just kept getting better and better,” says Natalie, who is quick to praise the nurses who cared for her daughter in the Cardiac Intensive Care Unit.
“Everyone was so helpful and encouraging. We knew if we had to leave her for a bit, they were on top of things.” Dr. Natarajan, too, stopped by often. “We really felt she was taking special care of Ayla,” Natalie says.
Discharge and follow-up care
Natalie, Pete and Ayla went home to Virginia in late November — and today, at 3 months old, Ayla is thriving. “She was really quiet in the Hospital, but now that she’s home, she’s developed quite a personality,” says Natalie.
Ayla is followed by a pediatric cardiologist in Virginia who works closely with her CHOP care team. In April, she’ll come back to CHOP for her first visit with the Cardiac Kids Developmental Follow-up Program, which provides screening and care for children with CHD who are at risk for neurodevelopmental problems. The team focuses on identifying issues early so that care can begin as soon as possible.
Natalie and Pete are already looking forward to the trip — it will give them the opportunity to reconnect with the team that cared for their family with such compassion and expertise.
“We feel so lucky that we were able to come to CHOP for Ayla’s care,” says Natalie. “I can’t say enough good things about our experience there.”
Originally published March 2, 2015