After a routine ultrasound at 24 weeks, Brailey and Louis Valenzuela learned some unexpected news about their baby, Arley -- she had an olive-sized tumor on her heart known as pericardial teratoma.
"We were terrified," Brailey Valenzuela, 32, of Maryville, Tennessee, tells TODAY.com. "We had never heard of this diagnosis. We had no idea that it existed and unfortunately the OB and the cardiologist here in Tennessee, they knew about the condition but there wasn't anything they could do."
The couple was stunned to learn that without treatment, the tumor would continue growing until it crushed the Arley's heart.
"We were basically waiting for her to die," Louis Valenzuela, 35, tells TODAY.com.
But then their local cardiologist sent the case to doctors at Children's Hospital of Philadelphia (CHOP) in the 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 as much as you can. You want to advocate for yourself and your child as much as you can," Brailey Valenzuela says. "But we literally had no idea what to do because it was such a rare diagnosis."
When Brailey Valenzuela became pregnant with the couple's second child, they felt happy. Everything started out well.
"It was a super easy pregnancy," she says.
It wasn't until the 24-week ultrasound that they couple even realized anything was wrong with the baby.
"They were calling it a mass," Brailey Valenzuela says. "That was on a Friday and my OB wanted me to come back Monday to do an additional ultrasound to see if they could try to pinpoint what it was a little better."
The second ultrasound confirmed the mass and doctors sent Brailey Valenzuela to the nearby children's hospital for a pediatric cardiologist to perform another ultrasound. That's when they learned their baby had a pericardial teratoma.
"We were told multiple times the outlook is grim and if you don't do something soon -- in a matter of days -- the baby could die," Brailey Valenzuela says.The baby needed to undergo fetal heart surgery, while Brailey Valenzuela was still pregnant. But only a handful of children's hospitals in the country can conduct such complicated surgeries. Luckily, the pediatric cardiologist recognized how important quick action was and sent the case to CHOP, who agreed to perform surgery on Brailey Valenzuela's baby.
"It was so fast," Brailey Valenzuela says. "They said, 'The physicians have looked over your file and they think they can help you.' It was a huge relief."
Still, the Valenzuelas didn't realize how quickly things needed to move. When they arrived on a Friday in December 2023, Brailey Valenzuela underwent "tons of testing," including an MRI so doctors could see what was happening to the baby's heart.
"They explained, 'Listen your child's heart functioning is already declining, and the functioning is declining even more in a matter of days,'" she says. "I remember asking, 'Can we wait? Can we think about it over the weekend?' And they said, 'Well, of course, you can think about it. But it you wait until Monday, we're afraid it will be too late.'"
The Valenzuelas decided to undergo the surgery, which took place the next day, a Saturday.
"Less than 12 hours later, we have the surgery," Brailey Valenzuela says. "It's overwhelming."
Fetal heart tumors "are quite rare" says Dr. Jack Rychik, one of the doctors involved in Brailey Valenzuela's surgery. A pericardial teratoma is a tumor but differs from a cancer. Still, it is deadly.
"It is a tumor that is growing wildly because very very young cells ... are misbehaving," Rychik, director of the fetal heart program at CHOP and the Robert and Dolores Harrington Endowed Chair in Pediatric Cardiology, tells TODAY.com.
In a pericardial teratoma germinal cells -- young cells that turn into organs -- haven't received the correct messages of what they should be, he says. Instead, they transform into masses.
"(They) could have turned into different organs but remain immature and decide to grow on their own," he says. "The concern about the pericardial teratoma is that it is growing rapidly and uncontrollably in a space that's very confined."
This teratoma grows on the lining of the heart, which prevents the heart lining from properly lubricating the heart. That makes the heart struggle to expand and contract normally. As the tumor increases in size, it increases the pressure on the heart.
"These tumors can grow so large they can get two, three, four times the size of the heart, compress the heart, (which) doesn't allow the heart to fill," Rychik says. "You cannot survive with the heart not being able to fill. So that's what makes these tumors lethal."
Often pericardial teratomas "seem to manifest and present themselves in about 20 to 24 weeks gestation." That's also the time when doctors need to act before the tumor grows too large and becomes deadly. While Rychik and his colleagues have consulted on dozens of cases, Arley was only the fourth patient whose condition had not progressed so much that surgery was an option. The other three children are thriving.
"There's a narrow window of opportunity to operate," Rychik says. "If you're too early you don't see this ... But once it's picked up and identified it's almost like the house is on fire. You've got to act very very quickly."
To remove the tumor from the baby's heart, doctors take the baby's chest and arms from the uterus essentially "sneaking in without the placenta and uterus knowing we're there," explains Dr. Holly Hedrick, a doctor involved in Brailey Valenzuela's surgery. One arm has an IV for medications and the other has something to monitor the baby's heart rate.
"The strategy is really about being minimalistic in terms of disruption," Hedrick, an attending surgeon in the division of pediatric, general thoracic and fetal surgery at CHOP, tells TODAY.com.
Doctors try to leave as much of the baby inside of mom as they can while performing the surgery.
The doctors use special devices to keep uterine volume up and "maintain the amniotic fluid," and anesthesia techniques so the uterus and placenta do not realize anything out of the ordinary is happening, Hedrick says. Then the cardiac surgeons remove the tumor. At the time, Arley weighed only about 1.8 pounds. Doctors immediately noticed that the pericardial teratoma was "ever so slightly attached" to the aorta, Rychik says.
"As (the tumor) was being gently pulled off the aorta there was a small tear in the aorta, and it started to bleed. Not just bleed but pump and you could see drops of blood," Rychik adds. "The cardiac surgeons saw that and immediately jumped right in and using microsurgical techniques were able to very quickly close that."
After removing the tumor and fixing the tear, surgeons closed the Arley's chest, returned her to the uterus and then closed Brailey Valenzuela's abdomen. All told, the surgery lasted about an hour.
"You need to be quick. Again, it's part of tricking the whole system," Hedrick says. "The faster you are ... the less complications."
When Brailey Valenzuela woke, she pointed to her belly immediately, worried that Arley didn't survive.
"I remember him saying, 'The baby is fine. The baby is fine,'" she says. "That's all I needed to hear ... it was a huge blessing."
For five days, Brailey Valenzuela recovered in the hospital before moving to the Ronald McDonald House for 91 days on "strict bedrest."
"I could walk five to 10 minutes at a time," she says. "They didn't want me to go into labor."
Brailey Valenzuela's pregnancy was high risk and she needed close monitoring. Going into labor could cause serious complications.
"Having the baby come early is the thing that we fear the most," Hedrick says. "She really shouldn't labor without having a high risk of uterus rupture, so we have to be always ready to intervene if she's starting to go into labor."
Louis Valenzuela flew back and forth from home to Philadelphia so he could work and care for their older daughter, LonaAnn. His mom stayed with Brailey Valenzuela but it was still tough for her.
"I was super super sad being away from my family, especially my daughter," she says. "It was extremely hard."
She also underwent weekly ultrasounds to make sure the baby was progressing well.
"They were monitoring her heart function and making sure it was expanding back to a normal size," Brailey Valenzuela says. "The tumor had been crushing it. And very quickly, within a matter of days, it started functioning fully and normally."
A little over 35 weeks pregnant, Brailey Valenzuela experienced pain and an ultrasound revealed that her uterine lining was very thin. Worried about a rupture, the doctors performed a C-section on February 17, 2024. Arley spent her first few days in the neonatal intensive care unit for some extra oxygen and to help her learn how to eat. But her mom was thrilled by how well Arley seemed to be doing.
"Her heart was great," Brailey Valenzuela says. "It's functioning like any other baby."
After this good news, the doctors shared a wish with the family.
"The doctors said, 'We just hope that she continues being medically boring,'" Louis Valenzuela says. "We don't pray for much. We just pray to be boring."
After about three weeks more in Philadelphia, the family returned to Tennessee. While coming home felt wonderful to Brailey Valenzuela, she also worried.
"You're anticipating like something is going to go wrong," she says. "Once we got over that initial scary feeling we settled into a normal routine."
Arley recently visited her local cardiologist and she's doing so well she doesn't have to return for a year.
"She's doing great," Brailey Valenzuela. "I hope people can have hope when they read Arley's story."