In world first, Philly surgeons remove fetal heart tumor during pregnancy

Katie Rice knew something was wrong when she got the results of her 20-week ultrasound.

The technician told her only that she was carrying a boy, then abruptly left the room.

In came a succession of doctors who said the fetus had some sort of mass on his heart and probably would not survive. Rice's own health was at risk, and she might have to terminate the pregnancy.

Instead, less than a month later, the Vermont woman was on an operating table at Children's Hospital of Philadelphia, where surgeons would try something that had never succeeded before. They would open the mother's uterus, remove the rare, fast-growing tumor that was pressing on the baby's heart, then sew her back up so the pregnancy could resume its normal course.

It worked. In a new study in the American Journal of Obstetrics and Gynecology, the surgeons reported that Rice gave birth to a healthy baby boy, Tucker Roussin, who is now 3.

But along with that success story, the team at CHOP reported the results of seven other heart-tumor cases from 2009 to 2015, in which fetal surgery was not attempted for one reason or another.

In some instances, the tumor was too far advanced for surgery, and the end result was a stillbirth. In two cases, tumors were surgically removed at or after delivery, and the babies survived.

In one case, the parents opted not to try prenatal surgery, and the fetus died. CHOP fetal cardiologist Jack Rychik stressed, though, that these parents made a "very legitimate choice."

"It's a big undertaking to subject yourself to an operation while being pregnant," he said. "At that point we couldn't make the statement that anybody had ever done this successfully."

Studying all eight cases helped doctors understand that with this rare type of tumor, they have a window of perhaps a week or two to take action.

"It pays respect to them to mention them," said surgeon Holly L. Hedrick, who led the team that removed Tucker's tumor. "They really made it possible for Tucker to happen."

For Tucker's mother, it all remains a blur.

'Just go for it

'

Midway through pregnancy, a developing fetus has a chest cavity smaller than a woman's fist.

There is room for the lungs, the heart - then about as big as a cherry tomato - and little else.

"If you have something else in there, it's going to push on something you don't want it to push on," Hedrick said.

The tumor on Tucker's heart was called a pericardial teratoma. It is so rare that Rychik, the cardiologist, was unable to put a number on it. Possibly one in hundreds of thousands?

"Incredibly rare," he said.

The heart is growing fast in this stage of pregnancy, but a teratoma can grow even faster. It is not cancer, but it represents a grave danger because it can squeeze the heart so much, it starts to leak. That, in turn, can cause a dangerous condition in the mother that resembles preeclampsia, called mirror syndrome - marked by impaired circulation and severe swelling.

In one of the cases reported in the CHOP study, the tumor grew from half the size of the heart to more than three times its size over the course of a month.

For Katie Rice, the news at the Vermont hospital seemed grim. But one of the doctors there - she does not know who - previously was a fellow at CHOP, and passed along the suggestion that its surgeons might be able to help.

She and her partner, Mike Roussin, drove to Philadelphia in late January 2013 for an initial appointment. Rychik and colleagues told them more about the tumor, but advised waiting a week to see whether it would grow.

They came back a week later, and it had.

The couple were invited into a room full of doctors, where they learned that the surgery could put her own health at risk. Maternal fetal medicine specialist Nahla Khalek and her colleagues ran down the list of possible complications: pulmonary edema, a need for blood transfusions, risks from anesthesia.

And the operation could jeopardize Rice's ability to have more children. She would be having two major abdominal surgeries in four months - one to remove Tucker's tumor, and one to deliver him by C-section. Her mother, who also had come down from Vermont for the meeting, was worried.

But Rice decided to go for it.

"With the surgery, at least the baby had a little bit of a chance," she said. "We thought that we would just go for it and hope for the best."

Two days later, she was on the table.

A whitish-pink mass

Since 1995, CHOP has performed more than 1,400 fetal operations, more than any other facility, officials say. They include procedures to treat spina bifida, among other conditions.

Removing a heart tumor was a new one.

It had been tried at least once before at the University of California San Francisco, reported in a 2002 paper. But that tumor was already so large that the fetal heart had started to leak, a condition called hydrops, and after the surgery the mother developed mirror syndrome. The baby was delivered early and did not survive.

For the attempt in Philadelphia, Hedrick was scheduled to take the lead. Along with her were fetal surgeon Alan W. Flake and a host of other physicians and nurses.

The team cut into Rice's uterus, then gingerly removed the fetus' arms from the placenta in order to lift his chest to the surface, leaving his head inside. They inserted an IV into one slender arm.

Standing by was cardiothoracic surgeon J. William Gaynor, who would remove the tumor.

"I'm just there going 'Wow, this is unbelievable,' " Gaynor said. "I'm just a spectator there watching all of this."

The tumor was whitish-pink and knobby-looking, so large that it obscured the heart. Gaynor took it out piece by piece, using a small cauterizing tool.

As the heart became liberated from this large mass, it started to contract irregularly in response. The surgeons quickly stopped and gave the fetus some blood. After a few minutes, the heart calmed down a bit, and the team went back to work.

The whole thing took just a few hours.

A typical boy

Word of the success is spreading in fetal surgery circles, said Michael R. Harrison, one of the surgeons on the team that performed the earlier surgery in San Francisco.

"It's a very important contribution to a rare problem," he said.

Harrison is widely considered the founder of the field of fetal surgery. Scott Adzick, director of the Center for Fetal Diagnosis and Treatment at CHOP, was a fellow under Harrison before coming to Philadelphia.

The message from the Philadelphia success story, he said, is this: If a fetal heart tumor appears to be growing, then fast action is needed.

Just this month, another East Coast woman in Katie Rice's condition got a plane to San Francisco, hoping for similar success, Harrison said.

But when she arrived hours later, the fetus had developed hydrops, and it was too late.

For Tucker Roussin, the timing was right. Now 3, he loves monster trucks, sandboxes, riding his bike, and water parks.

"Look at me now!" he says in a family video.

The right side of his heart is a bit smaller than average, a residual effect of the tumor, but the doctors say he is perfectly healthy.

"He is full of energy," his mother said. "He is a typical boy."

Except that he has a scar on his chest, which he will happily show anyone, on request.

tavril@phillynews.com

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@TomAvril1