Micropreemie Survives Lifesaving Heart Surgery for Rare Fetal Heart Tumor

Side-by-side operating rooms for mom and premature baby

It was a made-for-TV moment: a mother in a delivery room having a C-section to rescue her extremely premature daughter, who has a heart tumor that’s larger than the heart itself. The mother is flanked by dozens of doctors, her partner, and a chaplain who is ready to quickly baptize the baby in case she doesn’t survive. You may assume there is shouting, chaos, fear. Yet instead, there’s calm.

“What struck me the most was how calm everyone was. No one was panicking; they were just quietly doing their jobs,” says Michael Ma, MD, pediatric heart surgeon with the Betty Irene Moore Children’s Heart Center at Stanford Medicine Children’s Health. “I was in my operating room waiting for the baby to arrive, and when they wheeled her in, I thought she was stable. You couldn’t tell her life was in danger.”

At 18 weeks along, Amanda Valencia learned from her obstetrician in Santa Clara, California, that her ultrasound showed a bright spot on her baby’s heart.

“She told me that it was unlikely that my baby would make it,” Amanda says. “I started crying right there in the scan room.”

Amanda was referred to her nearby hospital, where more images were taken of her baby’s heart. A large heart tumor was identified, and Amanda was immediately referred to Stanford Medicine Children’s Health’s Fetal Cardiology Program.

“Her baby had an extremely rare, extremely large heart tumor called an intrapericardial teratoma. They start in the womb and grow rapidly. It was only the second time in my career that I’ve seen this type of tumor,” says Michelle Kaplinski, MD, fetal cardiologist.

When Amanda came to Stanford Children’s at 25 weeks along, the fetal cardiology care team started with diagnostic scans and tests to determine the size of the tumor and the effect it was having on her baby’s heart. They also counseled Amanda and her partner, Carlos, helping them understand the condition and what it meant for their growing baby, and helped them come to a decision that was right for them.

“Even though the outlook was poor, due to the rapid progression of the tumor and the baby’s prematurity, the family wanted us to do everything that we could to save the baby. We quickly mobilized all of the necessary teams,” Dr. Kaplinski says.

“When we got to Stanford, wow. They made me feel so comfortable,” Amanda says. “They told me the good and the bad, and they comforted me and helped me understand what to expect.”

The fetal cardiology team rallied multiple experts, starting with the Fetal and Pregnancy Health care team, which focuses on caring for fetuses with complex anomalies. From there, various Stanford Children’s specialties were called upon to help Amanda achieve a successful delivery and give her baby a great start. These additional specialists included doctors from high-risk obstetrics, neonatology, aerodigestive, heart surgery, cardiac intensive care, cardiac anesthesiology, oncology, and obstetric anesthesiology.

“The heart tumor was twice the size of the baby’s heart, and it occupied at least 50% of her chest,” says Yair Blumenfeld, MD, maternal-fetal medicine doctor and director of the Fetal Therapy service, offered in the Stanford Children’s Fetal and Pregnancy Health Program. “It was compressing her heart, forcing it into an abnormal position.”

The decision that the doctors made and remade several times a day was whether the baby was safer in the womb or outside of it. On the one hand, the heart tumor was growing (this can happen even when a tumor is noncancerous). On the other hand, the team wanted the baby to remain in the womb for as long as possible so that her lungs and other vital organs could develop.

“The greatest determining factor of how well a premature baby does is their gestational age. The longer a baby can develop in the womb, the better. We did everything we could to delay her delivery for as long as possible,” says Danielle Panelli, MD, a maternal-fetal medicine (high-risk obstetrics) specialist at Stanford Medicine Children’s Health and the doctor who would eventually perform the cesarean delivery.

Besides delivering her early, another option was an uncommon, highly experimental fetal heart surgery where doctors would remove the fetus, perform open-heart surgery, and put the fetus back into the womb.

“We went back and forth on whether we should deliver her early or try the highly experimental fetal surgery. Because our pediatric heart surgeons are highly skilled at performing heart surgery on small babies, and our neonatology care team is exceptional at helping very premature babies survive and do well, we decided it was safer to deliver her,” Dr. Blumenfeld says. “We also weighed what the surgery might mean for Amanda’s future fertility, which she wanted to preserve.”

A crucial condition that the multidisciplinary team watched for was cardiogenic hydrops—a buildup of fluid in the heart. They expected hydrops to develop because the tumor was weighing so heavily on the heart, which was struggling to pump blood to her body. As fluid began to build up, the fetal cardiology team noticed a subtle change in the amount of blood that flowed out of the baby’s heart. That prompted them to move forward with delivery.

A cesarean section birth was set for the next morning, just nine days after Amanda’s arrival. A second operating room was booked right next door so that Dr. Ma could perform immediate heart surgery on the baby.

“That night and the next morning, everyone on our large team came together to recap our delivery plan. We had several contingency plans for various challenges that might arise, like what we would do if the baby’s airway was blocked, or if the baby’s heart rate dropped and an emergency C section was needed,” Dr. Panelli says.

Angelina Anna Torres-Valencia was born at 26 weeks and three days, weighing around 2 pounds. On the operating table, Amanda was surrounded by a large team of doctors, ready to act quickly to support her and to save her baby’s life. The cesarean birth went without a hitch, but Angelina needed immediate life support. The airway team and neonatal critical care team acted fast to perform CPR to save her life and transfer her to Dr. Ma, who was waiting in the operating room next door.

“Angelina didn’t have much time. We immediately opened her chest; the mass was clearly taking up the majority of the space inside. The tumor was growing from the front of her heart muscle through the pericardial sac, or lining, of the heart. We very carefully removed the mass without damaging the fragile muscle underneath,” Dr. Ma says. “Once the tumor was removed, her heart immediately sprang back to life.”

In all, Angelina’s heart surgery took just six minutes. She is the youngest preemie that Dr. Ma has ever operated on.

“I was still opened up on the table when I was told that they got the whole tumor out from the root. I started crying with relief,” Amanda says. “I couldn’t believe how fast it all happened.”

In no time at all, Angelina’s blood pressure improved and her body turned pink. The multispecialty team’s intensive planning, expertise, and sound forethought made for a calm, methodical, and successful outcome for both Amanda and Angelina, despite how sick baby Angelina was.

“It was heroic. This is an example of our highest level of advanced care we offer, and it’s something we are skilled at doing here at Lucile Packard Children’s Hospital Stanford,” says Sonia Bonifacio, MD, neonatologist.

“This case pushed so many of our limits. It’s a testament to our institution’s strengths,” Dr. Blumenfeld adds.

Angelina was brought for immediate recovery to the Cardiovascular Intensive Care Unit, where the staff has expertise in caring for babies who have undergone heart surgery. The neonatology team worked closely with the cardiac team to ensure that Angelina’s critical prematurity was expertly addressed. After her health was stable, she was transferred to the Neonatal Intensive Care Unit (NICU). Angelina had a standard, uneventful NICU course of treatment for her prematurity. After a few weeks, she was weaned off oxygen support, and soon after she started taking food by mouth to supplement her tube feedings. Her heart continued to have a strong beat.

“What’s good about this heart tumor is that when you remove it, the heart is normal, and the tumor doesn’t grow back,” Dr. Kaplinski says.

The team consulted Allison Pribnow, MD, a pediatric oncologist at Stanford Children’s, and fortunately Angelina did not need chemotherapy (medicine to treat cancer) because the tumor was completely removed. The pediatric oncology team continues to monitor Angelina, and she is doing well.

Angelina spent a little over four months at Stanford Children’s, with her mom nearby at the Ronald McDonald House at Stanford. Amanda spent six hours every day at her bedside.

“I felt so much relief taking her home. She has family who already absolutely love her,” Amanda says.

Angelina just turned 6 months old. She is blowing bubbles, kicking her feet, and interacting with her parents. It melts Amanda’s heart when she takes Angelina for a walk in the stroller and she asks, “What are you looking at?” and Angelina catches her eye and laughs.

“Amanda and her family were rightfully scared in the beginning, but they always had hope,” Dr. Bonifacio says. “Having a baby in the NICU is like being on a roller coaster because you don’t always know what’s around the next bend, but Angelina’s parents managed it with a lot of grace.”

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