The baby had a fatal pericardial teratoma and required fetal heart surgery

After a routine 24-week ultrasound, Braley and Louis Valenzuela received unexpected news about their baby Arlie—she had an olive-sized tumor on her heart, known as a pericardial teratoma.

“We were terrified,” Braley Valenzuela, 32, of Maryville, Tennessee, told TODAY.com. “We have never heard of this diagnosis. We had no idea it existed, and unfortunately, the OB and cardiologist here in Tennessee knew about the condition, but there was nothing they could do.”

The couple were stunned to learn that without treatment, the tumor would continue to grow until it crushed Arlie’s heart.

“We were basically waiting for her to die,” Louis Valenzuela, 35, told TODAY.com.

But then their local cardiologist sent the case to doctors at the hospital. Children’s Hospital of Philadelphia (CHOP) in hopes that they can perform fetal heart surgery to save the baby’s life.

“It was one of those situations where you want to do as much as possible. You want to protect yourself and your baby as much as possible,” says Braley Valenzuela. “But we literally had no idea what to do because it was such a rare diagnosis.”

“Easy pregnancy”

When Braley Valenzuela became pregnant with their second child, the couple felt happy. Everything started well.

“The pregnancy was very easy,” she says.

It wasn’t until the 24-week ultrasound that they even realized there was something wrong with the baby.

“They called it mass,” says Braley Valenzuela. “It was Friday and my obstetrician wanted me to come back on Monday for a follow-up ultrasound to see if they could try to determine what was a little better.”

A second ultrasound confirmed the presence of the mass, and doctors sent Braley Valenzuela to a nearby children’s hospital to see a pediatric cardiologist for another ultrasound. It was then that they learned that their child had pericardial teratoma.

“We’ve been told repeatedly that the outlook is grim and if you don’t do something soon—within days—the baby could die,” says Braley Valenzuela. pregnant. But only a few children’s hospitals in the country can perform such complex operations. Luckily, a pediatric cardiologist understood the importance of quick action and sent the case to CHOP, who agreed to perform surgery on Braylee Valenzuela’s child.

“It was so fast,” Braley Valenzuela said. “They said, ‘The doctors have looked at your file and think they can help you.’ It was a huge relief.”

However, the Valenzuelas did not realize how quickly they needed to act. When they arrived on a Friday in December 2023, Braley Valenzuela underwent “a ton of tests,” including an MRI so doctors could see what was going on with the baby’s heart.

“They explained, ‘Look, your baby’s heart function is already deteriorating, and in a few days the functioning will deteriorate even more,'” she says. “I remember asking, ‘Can we wait?’ Can we think about it over the weekend? And they said, “Well, sure, you can think about it.” But if you wait until Monday, we are afraid it will be too late.”

Valenzuela decided to have surgery, which took place the next day, Saturday.

“We have surgery in less than 12 hours,” says Braley Valenzuela. “It’s overwhelming.”

Pericardial teratoma

Fetal heart tumors “are quite rare,” said Dr. Jack Ryczyk, one of the doctors involved in Braley Valenzuela’s surgery. Pericardial teratoma is a tumor but is different from cancer. And yet it is deadly.

“This is a tumor that is growing rapidly because the very young cells… are misbehaving,” Ryczyk, director of the fetal heart program at CHOP and the Robert and Dolores Harrington Chair in Pediatric Cardiology, tells TODAY.com.

In a pericardial teratoma, he says, the germ cells—the young cells that develop into organs—have not received the right message about what they should be. Instead, they become masses.

“(They) could develop into other organs but remain immature and decide to grow on their own,” he says. “The concern about pericardial teratoma is that it grows quickly and uncontrollably in a very confined space.”

This teratoma grows on the lining of the heart, preventing it from properly lubricating the heart. This causes the heart to struggle to expand and contract normally. As the tumor grows in size, it increases pressure on the heart.

“These tumors can grow so large that they can reach two, three, four times the size of the heart, compressing the heart, (which) prevents the heart from filling,” Ryczyk says. “It is impossible to survive if the heart cannot be filled. That’s what makes these tumors deadly.”

Often, pericardial teratomas “begin and become apparent at approximately 20 to 24 weeks of gestation.” This is also the time when doctors need to act before the tumor grows too large and becomes fatal. Although Ryczyk and his colleagues consulted on dozens of cases, Arley was only the fourth patient whose condition had not progressed enough that surgery was an option. The other three children are doing well.

“The window of opportunity for work is very narrow,” Ryczyk says. “If you come too early, you won’t see it… But once it’s picked up and identified, it’s almost as if the house is on fire. You’ll have to act very, very quickly.”

Fetal heart surgery

To remove a tumor from a baby’s heart, doctors remove the baby’s breasts and arms from the uterus, essentially “working their way in without the placenta and uterus knowing we’re there,” explains Dr. Holly Headrick, the doctor involved in Brayley Valenzuela’s surgery. In one hand there is an IV for medicine, and in the other there is a device to monitor the baby’s heart rate.

“The strategy is really to be minimalistic in terms of disruption,” Hedrick, an attending surgeon in the department of pediatric, general thoracic and fetal surgery at CHOP, tells TODAY.com.

During the operation, doctors try to leave as much of the baby inside the mother as possible.

Doctors use special devices to maintain the volume of the uterus and “maintain amniotic fluid,” as well as anesthesia techniques to keep the uterus and placenta unaware that anything unusual is happening, Hedrick says. Cardiac surgeons then remove the tumor. At the time, Arlie only weighed about 1.8 pounds. Doctors immediately noticed that the pericardial teratoma was “lightly attached” to the aorta, Ryczyk says.

“When (the tumor) was carefully separated from the aorta, there was a small tear in the aorta and it started bleeding. Not just bleed, but pump, and you could see drops of blood,” adds Rychik. “The cardiac surgeons saw this and immediately went to work and, using microsurgical techniques, were able to correct it very quickly.”

After removing the tumor and fixing the tear, surgeons closed Arlee’s breast, returned it to the uterus, and then closed Braylee Valenzuela’s abdomen. In total, the operation lasted about an hour.

“You need to act quickly. Again, this is part of deceiving the whole system,” Hedrick says. “The faster you… the fewer complications.”

High risk pregnancy and childbirth

When Braley Valenzuela woke up, she immediately pointed to her stomach, worried that Arlie wouldn’t make it.

“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.”

Braley Valenzuela spent five days recovering in the hospital and then moved into the Ronald McDonald House for 91 days on “strict bed rest.”

“I could walk for five to 10 minutes at a time,” she says. “They didn’t want me to go into labor.”

Braley Valenzuela’s pregnancy was high risk and required close monitoring. Childbirth can cause serious complications.

“Having a baby is the thing we fear most,” Hedrick says. “She really shouldn’t be in labor without a high risk of uterine rupture, so we should always be prepared to intervene if she goes into labor.”

Louis Valenzuela flew back and forth between their home in Philadelphia to work and care for their oldest daughter, Lona Ann. His mom stayed with Braley Valenzuela, but it was still hard for her.

“I was very, very sad to be away from my family, especially my daughter,” she says. “It was extremely difficult.”

She also had weekly ultrasounds to make sure the baby was developing well.

“They monitored her heart and made sure it was returning to its normal size,” Braley Valenzuela said. “The tumor crushed him. And very quickly, within a few days, he began to function fully and normally.”

At 35 weeks pregnant, Braley Valenzuela felt pain and an ultrasound showed that the lining of her uterus was very thin. Concerned about the rupture, doctors performed a caesarean section on February 17, 2024. Arlie spent her first few days in the neonatal intensive care unit to receive extra oxygen and help her learn to feed. But her mom was delighted with how well Arlie was doing.

“She had a big heart,” Braley Valenzuela said. “He functions like any other child.”

After this good news, the doctors shared their wishes with the family.

“The doctors said, ‘We just hope she continues to be medically boring,'” Louis Valenzuela says. “We don’t pray for a lot. We just pray we don’t get bored.”

After about three weeks in Philadelphia, the family returned to Tennessee. Returning home, Braley Valenzuela felt great, but she was also worried.

“You expect things to go wrong,” she says. “Once we got over that initial scary feeling, we went back to our normal routine.”

Arlie recently visited her local cardiologist and is doing well, not having to return for a year.

“She’s doing great,” Braley Valenzuela. “I hope people have hope when they read Arlie’s story.”