After a routine ultrasound at 24 weeks, Brailey and Louis Valenzuela learned unexpected news about their baby, Arley: he had an olive-sized tumor in his heart, known as a pericardial teratoma.
“We were terrified,” Brailey Valenzuela, 32, of Maryville, Tennessee, told .com. “We had never heard of this diagnosis. “We had no idea it existed, and unfortunately, the obstetrician and cardiologist in Tennessee knew about the disease, but there was nothing they could do.”
The couple were stunned to learn that, without treatment, the tumor would continue to grow until it would crush Arley’s heart.
“We were waiting for him to die,” Louis Valenzuela, 35, told .com.
But then her local cardiologist sent the case to doctors at Children’s Hospital of Philadelphia (CHOP) in hopes that they could perform fetal heart surgery to save the baby’s life.
“It was one of those situations where you want to do everything you can. You want to fight for yourself and your son as much as you can,” Brailey Valenzuela said. “But we literally had no idea what to do because it was such a rare diagnosis.”
An “easy pregnancy”
When Brailey Valenzuela became pregnant with the couple’s second child, they were happy. Everything started well.
“It was a super easy pregnancy,” she said.
It wasn’t until the 24-week ultrasound that the couple realized something was wrong with the baby.
“They called it dough,” explained Brailey Valenzuela. “That was on a Friday and my gynecologist wanted me to come back on Monday to have another ultrasound and see if they could better determine what it was.”
The second ultrasound confirmed the mass, and doctors sent Brailey Valenzuela to the nearest children’s hospital for another ultrasound by a pediatric cardiologist. That’s when they learned their baby had a pericardial teratoma.
“We were told several times that the prognosis was grim and that if we didn’t do something soon, in a matter of days, the baby could die,” said Brailey Valenzuela. But only a few children’s hospitals in the country can perform such complicated operations. Luckily, the pediatric cardiologist realized how important it was to act quickly and sent the case to CHOP, who agreed to operate on Brailey Valenzuela’s baby.
“It was very fast,” Brailey Valenzuela said. ‘They told me: ‘The doctors have reviewed your file and think they can help you.’ “It was a huge relief.”
However, the Valenzuelas did not realize how quickly things had to move. When they arrived on a Friday in December 2023, Brailey Valenzuela underwent “tons of tests,” including an MRI so doctors could see what was wrong with the baby’s heart.
“They explained to me, ‘Listen, your daughter’s heart function is already declining, and it will be declining even more in a matter of days,’” she said. “I remember asking, ‘Can we wait? Can we think about it over the weekend?’ And they told me: ‘Of course you can think about it. But if they wait until Monday, we fear it will be too late.’”
The Valenzuelas decided to undergo the operation, which took place the next day, a Saturday.
“Less than 12 hours later, they operated on us,” said Brailey Valenzuela. “It was overwhelming.”
Pericardial teratoma
Fetal heart tumors “are pretty rare,” said Jack Rychik, one of the doctors involved in Brailey Valenzuela’s operation. A pericardial teratoma is a tumor but differs from cancer. Still, it is deadly.
“It’s a tumor that’s growing wildly because very young cells…are misbehaving,” Rychik, director of CHOP’s fetal heart program and the Robert and Dolores Harrington Chair in Pediatric Cardiology, told .com.
In a pericardial teratoma, the germ cells – young cells that become organs – haven’t received the right messages about what they should be, he says. Instead, they transform into masses.
“They could have become different organs, but they remain immature and decide to grow on their own,” he explained. “The worrying thing about pericardial teratoma is that it grows quickly and uncontrollably in a very small space.”
This teratoma grows in the lining of the heart, preventing the lining from being able to lubricate the heart properly. This makes it difficult for the organ to expand and contract normally. As the tumor increases in size, pressure on the heart increases.
“These tumors can grow so large that they can be two, three or four times the size of the heart, compressing the heart, (which) does not allow the heart to fill,” Rychik said. “You cannot survive without your heart being full. “So that’s what makes these tumors lethal.”
Often, pericardial teratomas “seem to manifest and present between 20 and 24 weeks of gestation.” At that point, doctors must act before the tumor grows too large and becomes fatal. Although Rychik and his colleagues have seen dozens of cases, Arley was the fourth patient whose condition had not progressed enough for surgery to be an option. The other three children have progressed favorably.
“There is a narrow window of opportunity to operate,” Rychik said. “If you get there too early you don’t see it… But when it is detected and identified it is almost as if the house is on fire. “We have to act very quickly.”
Fetal heart operation
In order to remove the heart tumor, doctors remove the baby’s chest and arms from the uterus, essentially “sneaking in without the placenta and uterus knowing we’re there,” explains Holly Hedrick, a doctor who participated in Brailey’s surgery. Valenzuela. One arm has an IV for medication and the other has a device to monitor the baby’s heart rate.
“The strategy is to be minimalist in terms of disruption,” Hedrick, an attending surgeon in the division of pediatric, general thoracic and fetal surgery at CHOP, tells .com.
Doctors try to leave as much of the baby as possible inside the mother while they perform the operation.
Doctors use special devices to keep uterine volume high and “maintain amniotic fluid,” and anesthesia techniques so that the uterus and placenta don’t realize that anything out of the ordinary is happening, Hedrick says. Cardiac surgeons then remove the tumor. At the time, Arley weighed only about 1.8 pounds. Doctors immediately noticed that the pericardial teratoma was “loosely attached” to the aorta, Rychik says.
“When (the tumor) was gently separating from the aorta, there was a small tear in the aorta and it started bleeding. Not only was it bleeding, but it was pumping and you could see drops of blood,” adds Rychik. “The cardiac surgeons saw it and got to work immediately and, using microsurgical techniques, were able to close it very quickly.”
After removing the tumor and repairing the tear, doctors closed Arley’s chest, returned her to the uterus, and then closed Brailey Valenzuela’s abdomen. In total, the surgery took about an hour.
“You have to be quick. Again, it’s part of gaming the whole system,” Hedrick says. “The faster you are… the fewer complications there will be.”
High-risk pregnancy and childbirth
When Brailey Valenzuela woke up, she immediately pointed to her belly, concerned that Arley might not have survived.
“I remember (my husband) telling me: ‘The baby is fine. The baby is fine,’” he says. “That was all I needed to hear… it was such a blessing.”
For five days, Brailey Valenzuela recovered in the hospital before being transferred to the Ronald McDonald House where she remained for 91 days in “strict rest.”
“I could walk for five to ten minutes at a time,” he says. “They didn’t want me to go into labor.”
Brailey Valenzuela’s pregnancy was high risk and needed intense monitoring. Going into labor could cause serious complications.
“The baby being born early is what we fear the most,” says Hedrick. “You really shouldn’t go into labor without being at high risk of uterine rupture, so we always have to be prepared to intervene if you’re starting to go into labor.”
Louis Valenzuela flew back and forth from his home to Philadelphia so he could work and care for his oldest daughter, LonaAnn. Her mother stayed with Brailey Valenzuela, but it was still difficult for her.
“I was very, very sad to be away from my family, especially my daughter,” he says. “It was extremely hard.”
She also underwent weekly ultrasounds to make sure the baby was progressing well.
“They were monitoring heart function and making sure it expanded back to a normal size,” says Brailey Valenzuela. “The tumor had been crushing her. And very quickly, in a matter of days, it began to function completely and normally.”
At just over 35 weeks pregnant, Brailey Valenzuela experienced pain and an ultrasound revealed that the lining of her uterus was very thin. Worried about a rupture, doctors performed a C-section on February 17, 2024. Arley spent her first few days in the neonatal intensive care unit to receive extra oxygen and to help her learn to eat. But her mother was excited by how well Arley seemed to be doing.
“His heart was very good,” says Brailey Valenzuela. “He’s functioning like any other baby.”
After this good news, the doctors shared a wish with the family.
“The doctors said, ‘We just hope it stays medically boring,’” Louis Valenzuela says. “We don’t pray for much. We just pray that there is no news.”
After spending about three more weeks in Philadelphia, the family returned to Tennessee. While coming home was a wonderful feeling for Brailey Valenzuela, she also worried.
“You’re anticipating that something is going to go wrong,” he says. “Once we got over that initial feeling of fear, we settled into a normal routine.”
Arley recently visited his local cardiologist and is doing so well that he won’t have to return for a year.
“It’s very good,” says Brailey Valenzuela. “I hope people can have hope when they read Arley’s story.”
