When Christy Davis was 20 weeks pregnant, she and her husband went to her ultrasound, full of anticipation like any expectant parents.
UVA heart patient Sydney Davis, right, with her big sister Aubrey in Richmond, Va.
“It was a planned pregnancy and we were excited to find out that we were going to have a baby. We were excited to find out the baby’s gender,” she says.
But on that visit, they found out the little girl they were expecting had a structural heart defect—an underdeveloped heart ventricle.
Davis, who lives in Richmond, was referred to a cardiologist and then a perinatologist (an obstetrician-gynecologist who specializes in the management of high-risk pregnancies).
“They told us that UVA was the best place to go,” Davis says.
Birth and Surgery at UVA
Sydney Davis was born on December 4, 2006, at UVA, and eight days after her birth, she underwent the first of three surgeries to repair her heart.
Sydney’s condition is called tricuspid atresia. Babies with the condition have a missing or abnormally developed tricuspid heart valve. It’s a type of heart disease that’s present at birth (also called a congenital heart defect), and according to the National Institutes of Health, tricuspid atresia affects around 5 in every 100,000 live births.
Sydney’s first surgery included a Blalock-Taussig (BT) shunt placement, an atrial septectomy and pulmonary artery ligation.
“She actually had a rough go, but she was only in the hospital three weeks,” Christy Davis says. The new addition to the family made it home for her first Christmas. “That was a fantastic Christmas,” Christy says.
Congenital heart surgery on babies at UVA is performed in a particular operating room, where the goal is teamwork. The unique needs of babies with heart defects requires a set group of experienced people who specialize in treating these conditions, according to Daniel Schneider, MD, director of UVA’s Fetal Heart Program.
The program specializes in detecting, managing and treating fetal heart conditions and fetal heart rhythm disturbances.
The team at UVA starts work before the parents’ first visit. Carol Tatum, RN, a fetal cardiac nurse specialist, coordinates and supervises input from all members of the team.
“She rocks,” Davis says of Tatum. “She was always available via email to help with any questions we had.”
At UVA, fetal echocardiograms are performed by a group of four sonographers who have special training in imaging of congenital heart disease and advanced skills in identifying heart defects. The parents meet and get to know the entire UVA team so they are well prepared for the birth and the surgeries that will follow.
Two More Heart Surgeries at UVA
Sydney’s second surgery, the Glenn shunt came when she was just 4 months old. It made a big difference, Christy says. Feeding little Sydney was a long process for Davis. The baby needed to be fed from 6 a.m. until midnight each day. Each time she tried to feed, she would tire out and have to stop for a break.
But right after the Glenn procedure, the baby had more energy to drink a full bottle. “It was the first time she cried for food, and she guzzled that bottle down after surgery. She felt just so much better.”
There was still one more surgery to go. At 3 years old, Sydney underwent a Fontan procedure. Before the surgery, her blood saturation levels (the level of oxygen in the bloodstream) were low and she was literally blue, according to Christy. That’s normal for babies with Sydney’s condition.
But the Fontan procedure changes the blood flow in the body so babies can get that pink glow that most parents are familiar with, says Dr. Schneider. Fontan surgery separates virtually all of the deoxygenated blood (blue blood) returning from the body from the oxygenated blood (red blood) returning from the lungs. The oxygen saturations in the body are near normal and the child is no longer blue, but instead has the pink glow parents are familiar with, he says.
“When she came out, it was one of the prettiest pinks I’ve ever seen. Pink toes, pink tongue, pink fingers,” Davis recalls. Sydney’s saturation levels were in the 90s (normal is 98-100).
For Sydney, though, a case of swine flu three weeks after her surgery slowed down her progress, according to her mom. She spent a month in the hospital and was put on sildenafil citrate (commercially known as Viagra), which opens up the vessels and treats pulmonary hypertension. Her oxygen levels were down in the 40s at that point, Christy says. Doctors also inserted a pacemaker. It’s not placed in her shoulder, like it would be for an adult. Instead, it’s underneath the left side of her rib and she can feel half of it. Wires are attached to the outside of her heart.
Sydney refers to her pacemaker as her “computer.” The device allows doctors at UVA to review Sydney’s information remotely over the phone. The little girl even has a model of her implanted pacemaker. “Sydney shows it to everybody. She could not be prouder,” Christy says. In about 8 years, Sydney will return to get her pacemaker replaced.
Mended Little Hearts
Christy is active in the Mended Little Hearts Richmond chapter and previously served as the group’s co-coordinator.
She attended her first Mended Little Hearts meeting when she was 8 months pregnant. “I wanted to meet people in my same situation. It was such a lonely feeling. I didn’t know anyone with a child with a heart defect.
“I walked in that room and I got surrounded. It was the best thing ever. I knew after that meeting that there would be light at the end of the tunnel. It was uplifting.”
Mended Little Hearts also helps families in other ways. The Richmond chapter puts together care bags for families that have to travel for treatment and surgery. The bags contain items like phone cards, toothpaste and toothbrushes, gas cards, games and magazines. The group holds fundraisers to pay for the bags and to donate to research. They’ve also lobbied in Washington, D.C., for a bill that would make pulse oximetry tests mandatory for all infants before they leave the hospital. This test can spot congenital heart defects that might otherwise go undetected. (All babies born at UVA Children’s Hospital already receive pulse oximetry screening.)
A Normal Little Girl With a Special Heart
Sydney has follow up visits with her local cardiologist in Richmond, who communicates with specialists at UVA, but she’s living the life of a normal 5-year-old, her mom says. She’s in preschool and takes ballet and tap classes. She does need a lot of fluids to help with her blood flow.
“She runs around like most kids. She will slow down when she’s tired.” Dance is a great activity for her, Christy says, because it’s not high aerobic activity the entire time.
Her big sister, Aubrey, just finished second grade. And like most little sisters, Sydney wants to do everything her big sister does, like join the swim team.
“We do the best we can do to treat her as normally as possible,” Christy says. “Our little motto is, ‘We didn’t save her life for her to not have one.’ We tell her she’s got a special heart.”
Find out more about Mended Little Hearts and congenital heart defects: