Pregnancy can be a physically and emotionally difficult experience for many women. But when finding out the baby has a rare, potentially life-threatening heart condition, the experience becomes even more challenging.
“We got the diagnosis around my 20-week appointment. It was very shocking.”
Nikki Livingston was preparing to have her third child in February 2020. About halfway through her pregnancy, she and her husband, Sean, found out their baby girl had tetralogy of fallot, a rare congenital heart condition where there is a combination of four heart defects that can cause poor oxygen flow to the heart.
“Because I have Crohn’s disease, it was already considered a high-risk pregnancy,” Livingston said. “I was seeing a maternal-fetal medicine specialist (MFM) and they found it on their ultrasound machine.”
There are fewer than 20,000 cases of tetralogy of fallot in the United States per year. The condition includes four defects in the heart, affecting the third and fourth chambers, pulmonary artery and aortic artery. Because of the defects, when the heart’s pumping action is overworked, it can cause the muscular wall of the right ventricle to thicken over time. That can cause the heart to stiffen, weaken and eventually fail. While there is no significant impact on the mother’s health, she can be more likely to have a baby with a congenital heart defect in future pregnancies.
Livingston’s obstetrician-gynecologist referred her to MFM specialists at Indiana University Health and pediatric cardiology specialists at Riley Hospital for Children.
“They said ‘you need to go to Riley and we need to transfer your care, because they’re the experts,’” Livingston said. “Even though this diagnosis can be a bit overwhelming, of all the places to be, it’s so perfect that we’re here in Indianapolis, because they are really skilled in this area.”
Livingston’s birth plan changed to delivering her daughter at IU Health Methodist Hospital and transferring her to Riley soon after the birth. Her prenatal care also had to change. For the last couple months of her pregnancy, Livingston had to complete biophysical ultrasounds and non-stress tests twice a week. These tests monitor the baby’s heart rate as it relates to activity level to make sure the heart is still responding properly.
“It’s like if you’re running on a treadmill, your heart beats faster, so with my baby, they checked to see that her heart rate had the appropriate spike level when she had a lot of movement,” Livingston said.
“Typically, we diagnose tetralogy of fallot in utero on a fetal echocardiogram,” Farrell said. “If the mom does not get a fetal echo to diagnose the disease before the baby is born, typically the baby would be blue after birth or have a murmur that would prompt getting a heart echocardiogram and the baby would be diagnosed that way.”
Livingston said she’s thankful that today’s technology was able to provide them with an early diagnosis. In the past, she said, patients who suffered from tetralogy of fallot found themselves in an even scarier situation.
“You didn’t know until after baby was born and you’d possibly go home and the baby would turn blue or have a hard time breathing, and then you’d have to rush the baby to the hospital and that’s when they would find out it was a heart issue,” Livingston said. “So to be able to prepare while she’s still in utero is much better.”
They also developed a plan to care for the baby after birth, which included at least one surgery at some point. Depending on how a baby with tetralogy of fallot looks after birth, Farrell said they may or may not need a shunt surgery in the first few weeks. But all babies with the condition need a surgery to fix the heart defects at about six to nine months of age. After that, they need regular follow-up appointments with a cardiologist, and some may have heart rhythm problems or need further valve replacements later in life, but they often go on to live normal, healthy lives.
“After they have their definitive surgery, they typically are not on medications long-term,” Farrell said. “They can lead nearly normal lives from an activity standpoint—play sports, go to school—with no significant restrictions.”
Livingston was induced on February 10th, 2020, and her daughter, Janae Shalom, was born later that day. Unfortunately, Janae’s oxygen levels were too low for Livingston to hold her once she was born, and she had to be taken to the neonatal intensive care unit (NICU) immediately after being cleaned and swaddled.
“They brought her to my bedside so I could look at her, but I couldn’t hold her yet,” Livingston said.
Thankfully, Janae’s oxygen levels climbed back up, but she still wasn’t allowed to breastfeed, because feeding is a strenuous activity for newborns and it could have been too much for her heart. Instead, she needed intravenous therapy (IV) for fluids. Doctors and nurses also used monitors to keep an eye on her heart rate and oxygen levels. A few hours after she was born, she was transferred to Riley. Livingston’s husband went with Janae. Livingston, however, remained at IU Methodist recovering in the postpartum area and using a breast pump to help establish her milk supply in case Janae would be able to nurse later.
“It was hard not being able to be with Janae, but I had a direct line I could call for information and updates, and of course Sean kept me in the loop,” Livingston said. “I also had a friend from church stay with me overnight at the hospital for emotional support.”
Janae with her father Sean.
When Livingston recovered, she was able to join Janae and her husband at Riley. After monitoring her for a few days, the doctors said no shunt surgery would be needed, and Janae was given the okay to go home on February 14, 2020, in time to celebrate Valentine’s Day with her parents and two older siblings.
“For now we will continue to follow up with Dr. Farrell,” Livingston said. “We were given instructions on what to look out for, like her lips turning blue, skin turning blue between the eyebrows and labored breathing. We also got an Owlet baby monitor, which allows us to monitor her heart rate and oxygen levels while she sleeps.”
Livingston said she is thankful for the advanced level of care she and her daughter are both receiving from their team at IU Health and Riley Hospital for Children, which includes faculty, fellows and residents from IU School of Medicine.
“I went to my appointment and was sitting there just asking, ‘is there anything I could have done to prevent this?’ and they said, ‘no, it’s just a mystery and something that just develops,’” Livingston said. “We decided to just hope for the best and prepare for anything that may happen, but we know all is not lost.”
The name Janae is a modern elaboration of Jane, which was developed in the Middle Ages as an English female equivalent to the name John. John comes from the ancient Hebrew “Yochanan,” meaning “God is gracious.” Janae’s middle name, Shalom, also comes from ancient Hebrew, and means peace. Livingston says those two names felt fitting for what their daughter has already endured in life and the perspective of hope and peace they’ve tried to keep throughout Livingston’s pregnancy.
“I wanted to find a name that kind of represents the spirit of strength, love and heart,” Livingston said. “She’s such a fighter.”
The views expressed in this content represent the perspective and opinions of the author and may or may not represent the position of Indiana University School of Medicine.
As a communications coordinator with the Office of Strategic Communications, Christina develops and implements strategic communications plans and projects for internal and external audiences. Before joining IU School of Medicine, Christina worked as an a...