Baby Boy Triumphs Over Tetralogy of Fallot Following Successful Heart Surgery

When 18-month-old Ace Coustol was born, he seemed perfectly healthy. At his six-week checkup, he was diagnosed with a heart murmur, but it didn’t present any immediate cause for concern.

What started out as a common cold in March of 2023 turned life threatening when Ace stopped breathing. His mother, Chantelle, brought him to the emergency room at Cook Children’s Medical Center – Prosper, where he was immediately put on oxygen supplementation and sent to a cardiology consultation. 

Upon completing an echocardiogram and an electrocardiogram, Ace was diagnosed with a congenital heart defect, Tetralogy of Fallot (TOF). Though he didn’t require urgent intervention and was released from the hospital after a few days, he was scheduled for mandatory open heart surgery a couple of months later.

About Tetralogy of Fallot (TOF)

The root word “tetra” translates to “four,” which means Tetralogy of Fallot consists of four heart defects. 

“It’s probably the most common cyanotic heart disease, meaning it can cause low oxygen levels,” said Ace’s cardiologist, Pamela Burg, M.D. with Cook Children’s Heart Center – Prosper. 

The most common indicators of TOF include a large hole between the bottom two chambers of the heart, as well as thickened muscle bundles below the pulmonary valve that obstruct blood flow to the lungs. Sometimes, the valve itself can be too small or too thick, causing it not to open properly. 

“What happens is that some of the low oxygen blood can’t get to the lungs to get oxygen because of the obstruction in blood flow,” Dr. Burg said. “Since there’s a wide open hole between the two bottom chambers, some of that blue blood gets pumped out to the body and causes the low oxygen level.”

Congenital heart defects like TOF are typically detected in prenatal ultrasounds or directly after birth. Ace, however, was a special case, having passed his newborn heart screen without any cause for concern. 

“It is possible that the amount of valve obstruction was mild enough when Ace was first born that it didn’t cause a problem until he got a little bit older,” Dr. Burg said. “Once every couple of years, we’ll have a case that escapes all those other means of detection and gets picked up when they’re older, but it’s usually picked up in infancy at the latest.”

Symptoms of TOF 

Without surgical intervention, TOF can be fatal. Hypercyanotic spells, commonly known as “tet spells,” happen when a child with TOF has a dangerous drop in blood oxygen levels, causing them to turn blue and sometimes resulting in seizures, difficulty breathing or even death. These spells often happen with increased activity and exertion, which is why doctors try to surgically repair the defects as early as possible.

Ace was considered a “pink tet” baby, meaning he did not experience drops in oxygen levels severe enough to make him appear blue. He did, however, show symptoms that concerned Chantelle before he was diagnosed.

Any time Ace got sick, his oxygen levels would drop to concerningly low levels. Occasionally, Chantelle would notice his body going limp and his skin color looking slightly off. He was frequently fatigued, extremely sensitive to changes in temperature and slept more than usual. 

“I thought he was just an easy baby, but now I realize his heart was just so tired,” Chantelle said.

Ace’s Surgery

Last June, after postponing his initial appointment due to sickness, Ace finally had his heart surgery with Cook Children’s Medical Director of Cardiothoracic Surgery, Vincent Tam, M.D. The surgery lasted almost an entire day, but everything went as smoothly as possible. 

“Everything we prayed for, God answered,” Chantelle said. “Dr. Tam is incredible, his whole team was great and the nurses were amazing.”

According to Dr. Burg, Ace’s surgery consisted of closing the hole between the bottom two chambers of his heart and opening up the muscle bundles below his pulmonary valve. Fortunately, Ace’s pulmonary valve itself was functioning quite well and didn’t require any intervention.

Care Team Goes Above and Beyond

Though Ace’s procedure was a resounding success, he experienced a rare reaction to anesthesia—severe delirium—his first night in the hospital. He was crying nonstop in his sleep, and the only way to wake him up was to take him off all pain medication. Stressed, distraught and exhausted, Chantelle didn’t know what to do—but the Cook Children’s care team came to the rescue. 

Ace’s nurse didn’t leave his side the entire night so that Chantelle could get some much-needed rest. She pulled out all the stops—including soothing toys and heat packs—to calm Ace down.  

“I was sobbing and felt so bad that I was trying to rest, but the nurse just looked at me and said, ‘Mama, go sleep. It’s okay,’” Chantelle said. “As a parent, it’s so challenging seeing your child like that and I was just super thankful knowing she didn’t leave his side.”

A Heart Hero

Now that Ace has recovered, he joins a community of people who are living remarkable lives with TOF—including three-time Olympic gold medalist, Shaun White.

“We anticipate Ace to have an excellent prognosis,” Dr. Burg said. “He should be able to grow and run and play just like any other kid, but we’re still going to have to keep monitoring him throughout his whole life.”

This past February, Ace was honored as the “Heart Hero” at his sisters’ elementary school during American Heart Month. His sisters, Lennon and Indy, got to bring him on stage during a presentation from the American Heart Association and tell their classmates all about his journey.

“Ace is doing great, and he’s really proud of his heart,” Chantelle said. “He’s so busy, so smart and so strong.” 

“Everything we do for congenital heart disease is to ensure these kids can have as normal of a life as possible,” Dr. Burg said. “We want them to be playing and going to school with no limitations, and we want them to be able to do everything they want to do in life.”