Addison’s story: How our daughter’s Tetralogy of Fallot journey led us to Children’s Hospital New Orleans

Every time Hannah Engel and her husband, John, lay eyes on their 2-month-old daughter, Addison, they can’t help but call her their rainbow baby. Just like a colorful rainbow, their little girl reminds them of the beauty and hope that follow a storm. For the Engels, it was finding renewed hope after four miscarriages.

“It wasn’t hard for me to get pregnant the first time with our son,” said Hannah. “When we tried to have a second baby to give our son a little sibling, I started having difficulty sustaining my pregnancy. I had a miscarriage when I was four weeks pregnant, then I miscarried two more times when I was five weeks pregnant. The last miscarriage was a chemical pregnancy. So, I had four miscarriages before I discovered I was pregnant with Addison. I remember taking an early pregnancy test and it came back positive. When I told my husband, we were both excited and extremely nervous at the same time. We were cautiously optimistic, but we knew there was still a chance that something might go wrong. So, we kept that in mind. I was reluctant about telling anyone I was expecting until we were out of the danger zone. I waited until I was 13 weeks before sharing our pregnancy news with our family and friends.”

As soon as Hannah found out she was pregnant with Addison, her OB-GYN prescribed the hormone, progesterone, to prevent pregnancy loss during the first 12 weeks, the most common time for a miscarriage to occur. Hannah took the hormone for six weeks, and when she returned for her baby’s 8-week ultrasound, tears streamed down her face when she heard her baby’s heartbeat for the first time.

“It was an amazing moment to hear her tiny heart beating inside of me,” said Hannah. “During my first trimester, everything was going smoothly. I started getting morning sickness which I never experienced with my miscarriages. This gave me a little bit more hope that my pregnancy would be fine. Then, of course, I ended up taking 40 pregnancy tests to ensure my hormone level was in the normal range. I wanted it to stay up instead of dropping back down. More than anything, I wanted peace of mind.”

Hannah’s second trimester was progressing well, and her baby’s 20-week anatomy scan showed no signs of any complications. But a few weeks into her second trimester, her baby had a subchorionic hemorrhage where bleeding occurs when the placenta partially detaches from the uterine wall. The hemorrhage was mild. The next day, it cleared up and was not visible on Addison’s anatomy scan.

Despite this little bump in the road, Hannah was relieved that she got this far in her pregnancy without any foreseeable problems. But things began to change during her third trimester. When she was 36 weeks along, she felt something wasn’t right. She went in to see her OB-GYN for a follow-up visit.

“I felt like my baby was having seizures in my stomach, but I found out that wasn’t the case,” said Hannah. “But still, I felt something was off, and I just couldn’t put my finger on it. The ultrasound appeared to be normal, but after sharing my concerns with my OB-GYN, he sent me to a high-risk clinic to have another ultrasound using a higher frequency. That’s when we found out there was something wrong with Addison’s heart. They scanned her organs and when they got to the heart, they spent 20 minutes just looking at her heart. Addison had an echocardiogram. It wasn’t until the next day, that I got a Zoom call and was told that my baby’s heart was developing abnormally. She had a ventricular septal defect.”

The Engels were referred to Dr. Lynn Batten, a pediatric cardiologist at USA Health in Alabama who specializes in high-risk pregnancies. It was about a two-hour drive from their home in Mississippi. Doctors in Alabama told the Engels they had high hopes that Addison could be delivered at a hospital in Alabama, and they could take their baby home and postpone surgery for a few months until Addison got a little older. But, Addison’s heart problem was more severe than expected.

On Oct. 11, when Hannah was 38 weeks pregnant, she gave birth to Addison who weighed 7 pounds, 6 ounces. Hannah and her husband held their newborn daughter briefly before she was transferred to the neonatal intensive care unit (NICU) because her oxygen levels were dropping dangerously low.

“While in the NICU, they were able to get her oxygen levels back up,” said Hannah. “They let her stay with us in our room because they thought she would be okay. It turns out she wouldn’t be. They were still tracking her oxygen and it kept dropping. So, she went back to the NICU. The day after she was born, she had her last echocardiogram in the NICU, which confirmed our daughter had Tetralogy of Fallot.”

Tetralogy of Fallot (TOF) is a set of four congenital heart defects that occur together. The set of four defects include narrowing (stenosis) of the pulmonary artery, ventricular septal defect (opening in the wall between the right and left ventricles), overriding aorta, and an enlargement of the right ventricle. If left untreated, these heart anomalies can cause a number of serious complications including blood clots, infection in the lining of the heart and heart valves, abnormal heart rhythms, heart failure and death.

“When I heard about our daughter’s diagnosis, I was terrified and speechless,” said Hannah. “I didn’t believe it was happening. I remember the cardiology specialist explaining to me what was going on with my daughter and why she needed immediate surgery. She drew me a picture of everything that was wrong with her heart. Since their hospital didn’t perform TOF surgeries, she told me Addison would have to go to Children’s Hospital New Orleans for the procedure. Immediately, I signed papers for my early discharge from the hospital so I could be with my baby. My blood pressure was up and down, but I felt fine. I just wanted to be with my baby. A day after her birth, Addison was airlifted to Children’s Hospital. We dropped our son off at my husband’s parents’ house so we could make the 1.5-hour drive from Mississippi to New Orleans. We rushed to the hospital to be with our newborn daughter in the NICU.”

As soon as Addison arrived at Children’s Hospital, her doctors immediately started running a bunch of tests on her. There were two TOF surgery options that they were considering – a complete repair or a partial repair now and then another surgery four months later. They wanted to redo her ECHO and run more tests to determine what surgical approach would be best for Addison to determine the severity of the pulmonary artery narrowing, the doctors tried to wean Addison off a medication (prostaglandin E) that keeps open a blood vessel which bypasses the narrowing. Her doctors wanted to try everything possible besides surgery on a baby this young.

“They took her off the medication to see how she would respond to it,” said Hannah. “Shortly thereafter, Addison’s oxygen levels were dropping again. She was put back on the medicine that evening. Since weaning her off the medicine didn’t work, the next step was figuring out which surgery would be best for our baby. Since one doctor can’t make a big decision like that, a group of doctors convened to determine the best surgical treatment approach for Addison. They agreed the complete repair was the best option.”

On Oct. 18 at only 8 days old, Dr. Frank Pigula, one of the country’s most accomplished congenital heart surgeons and Chief of Pediatric Cardiothoracic Surgery and Co-director of the Heart Center at Children’s Hospital, performed the TOF repair on one-week-old Addison. This was one of the first times Children’s Hospital has performed the TOF procedure on babies this small and Addison was one of the first babies to have it done at such a young age.

“For babies dependent on prostaglandin E, complete repair of tetralogy as a neonate is preferred when the anatomy is favorable,” explains Dr. Pigula. “A complete repair includes closure of the ventricular septal defect and enlargement of the connection between the heart and the arteries to the lungs. This approach provides normal blood oxygen levels immediately and avoids an intermediate operation that can be risky for the baby.”

“It took four hours to complete Addison’s surgery,” recalled Hannah. “Dr. Pigula was amazing – he and his team updated us on every stage of the surgery from prep to the surgery itself and to post surgery recovery. Remarkably, Addison made immediate improvements after surgery. She recovered in the NICU for one week before she was discharged. Prior to taking her home, she had her breathing tube removed and she passed the car seat test before we made the one-and-a-half-hour drive home to Mississippi.”

Today, 3-month-old Addison is doing well. She is the happiest baby. She loves to smile and loves to eat. Since her surgery, she has had no heart related issues at all. Her scar on her chest has healed up nicely, and the Engels says it’s hard to tell their daughter had open heart surgery soon after she was born.

“Our baby is just a living, breathing miracle,” said Hannah. “Our family had a great experience with the team at Children’s Hospital. The nurses were amazing. I just can’t thank them enough including Addison’s heart surgeon, Dr. Pigula, and her pediatric cardiologist, Dr. Kimball, for saving our baby’s life. Without them, she wouldn’t be here. I am glad our paths intersected so our baby could get the care she needed.”

Addison sees Dr. Thomas Kimball, co-director of the Heart Center and Division Chief of Cardiology at Children’s Hospital, for her cardiology follow-up appointments every three months. Life-long care and surveillance are important in a congenital heart patient’s care even when TOF is treated in childhood.

“Most children born with Tetralogy of Fallot are treated with a corrective repair that involves a patch to close the septal defect and enlargement of the pulmonary valve to facilitate blood flow to the lungs,” said Kimball. “In some cases, enlarging the pulmonary valve can cause it to leak, necessitating a valve replacement in young adulthood. That’s why periodic exams are needed to improve their long-term outcomes.”

For the Engels, nothing could have prepared them for this unexpected journey. They never imagined this situation would happen to them. But through it all, they learned important life lessons and hope their experiences will give strength to other patient families who are navigating through similar challenges.

“While our journey with congenital heart disease was hard – we had plenty of ups and downs -- I realized how important it is to be your child’s advocate,” said Hannah. “As a parent, you need to be comfortable with your decision and if there is something that makes you uncomfortable, you need to speak up – let your concerns be known. My biggest advice to families is to never be afraid to ask questions. I asked a lot of questions and everyone on Addison’s care team from her doctors to her nurses, were kind and graciously answered my questions and concerns. I also had a great support team in place, my husband, who helped me stay strong. It’s important to have a positive mindset and know everything will be okay.”

Click here for more information on our nationally recognized cardiothoracic surgery program at Children’s Hospital New Orleans. Click here to learn how our heart team provides specialized care to patients with Tetralogy of Fallot and other congenital heart conditions to improve our patients’ long-term outcomes.