In-Utero Surgery Offers Hope
Va. Baby Healthy After Procedure

By Leef Smith
Washington Post Staff Writer
Saturday, January 28, 2006

The operation was both delicate and complex, requiring the expertise of 16 specialists who worked together in November to thread a lifesaving stent into a malformed human heart. The procedure might have been routine were it not for the patient: a fetus nestled in her mother's womb.

After the surgery, on a heart the size of a grape, Grace VanDerwerken became the world's first fetus to have a cardiac device implanted, doctors said. Yesterday, Grace, just 17 days old, was discharged from Children's Hospital Boston, and she will fly home today to Loudoun County with her parents.

"It's been a miracle," Grace's mother, Angela VanDerwerken, 33, said yesterday in a phone interview shortly before a news conference in Boston to announce the "first of its kind" procedure. "She has an amazing outcome now."

Doctors who treated Grace weren't making hard-and-fast predictions about her long-term prognosis, but they said all signs point to a promising future, one that probably would not have been possible without creative thinking.

It was shortly after a routine sonogram that doctors told Angela and Jay VanDerwerken, 36, of Ashburn that their unborn child had hypoplastic left-heart syndrome, a congenital defect in which the left ventricle -- the heart's main pumping chamber -- fails to develop.

The condition requires three surgeries within the first several years of the child's life to rebuild the heart.

Making Grace's condition even more precarious was the presence of an intact wall, or atrial septum, between the two upper chambers of the heart, causing blood to back up into her lungs and damaging the tiny air sacs and delicate pulmonary vessels.

Even with open-heart surgery immediately after birth, doctors warned the couple that the infant had a 20 percent chance of survival.

"Things looked so grim," Angela VanDerwerken said. "We had to face the reality that she might not survive."

Although doctors at Children's Hospital Boston and Brigham and Women's Hospital have performed more than 60 in-utero procedures on the heart -- some of them to try to correct the condition Grace has by opening up the atrial septum -- the outcomes have not been as good as doctors had hoped. In most cases, the septum closed, and the baby was born with signs of lung damage.

A possible solution, doctors agreed, was to use a stent to prop open the septum to prevent permanent lung damage in the final weeks of gestation.

Grace, her parents agreed, would be the test case.

On Nov. 7, a team of specialists -- cardiologists from Children's and high-risk obstetrical specialists from Brigham and Women's Hospital -- used ultrasound imaging to guide a catheter through Angela VanDerwerken's abdomen and uterus into the heart of the 30-week-old fetus, a 1 1/2 -hour operation.

A fine needle, followed by a wire with a preloaded balloon, was pushed into the left atrium and then through the atrial septum to reduce the pressure that was causing the atrium to expand with blocked blood. The balloon was inflated until the atrial wall widened. Then the same process was used to create a second hole in the septum, only this time, a tiny stent was put in place.

Cheers rang through the operating room when heart monitors showed blood flowing through the stent. But doctors would not know how successful they'd been until Grace was born by vaginal delivery Jan. 10.

At birth, Grace weighed a healthy 8 pounds 2 ounces. And unlike babies born with similar heart defects, she was pink and without any signs of breathing problems.

"It was very exciting," said James Lock, chief cardiologist at Children's Hospital Boston. "After the baby was born, most of the cardiologists in the hospital walked by her bedside just to see if it was really true" that she was rosy and healthy. "I'm very optimistic that she'll do well."

Hypoplastic left-heart syndrome is serious enough that many babies found to have the malformation are not carried to term. Doctors at Children's treat five to 10 cases a year.

Grace is the fourth child for the VanDerwerkens, who said they believed they were on track for another healthy pregnancy until a second-trimester ultrasound.

Excited to learn the baby's sex, they brought their three young children with them to the obstetrician's office to be there for the news.

But in addition to learning that their baby would be a girl, they were told doctors couldn't quite make out the baby's heart. More tests were done before doctors delivered the devastating news, suggesting they consult with specialists.

Even after the surgery to insert the stent, Angela VanDerwerken said she couldn't let go of the fear that her child might not be healthy enough to have the surgeries necessary to thrive. It was Grace's birth and appearance of health that gave her hope.

"That was the point when I let myself realize she's here and she's going to stay, instead of thinking she might die all the time," VanDerwerken said. "When we saw she was doing so well, we knew she had a fighting chance."

After her birth, Grace spent a few precious moments with her parents, who took pictures and introduced her to her siblings: Hannah, 7, Jackson, 6, and Ethan, 3.

She spent the next eight days in intensive care preparing for, and then recovering from, the first of three open-heart surgeries. She'll have the second when she's about 6 months old and the last when she's 2 or 3.

"It's been a miracle," said Angela VanDerwerken, who is flying home today to Hidden Pond Place with her husband and daughter. "I'm very grateful for all of this."