BOSTON (AP) – Angela VanDerwerken’s first surgery could be a lifesaver. But it wasn’t her life that was at risk. It was her baby daughter’s.
Last November, she traveled from Ashburn, Va., to Children’s Hospital Boston for in-utero heart surgery on her 7-month-old fetus, who was found to have an often-fatal developmental heart defect.
On Jan. 10, VanDerwerken gave birth to Grace, her fourth child. Just 13 days later, Angela, her husband Jay and their daughter are going home.
At a news conference Friday, 2-week-old Grace drifted in and out of sleep in her mother’s lap as doctors explained the novel circumstances that led to her birth.
Grace is the first baby to have had a heart stent implanted while in the uterus, said the hospital’s cardiologist-in-chief, Dr. James Lock.
Her condition, hypoplastic left heart syndrome, is the most lethal of the infant heart disorders. The left side of the heart – including the body’s largest artery, the aorta, and the heart’s main pump, the left ventricle – is severely underdeveloped and in many cases, useless. Grace also didn’t have a passage from the left to right chambers in her heart. Her blood was barely pumping and it had nowhere to go.
Until recently, Lock said, there were “essentially no survivors of this condition.” Although increasingly earlier intervention has lowered mortality rates, most of the babies that lived faced life-long health problems, and the survival rate was still only between 20 and 50 percent.
In previous cases, doctors had learned that the essential step in saving the lives of babies with HLHS and the missing passage was to poke a hole in the wall that separates the left and right chambers of the heart and keep it open as long as possible before birth. This allowed blood to reach other essential organs, particularly the lungs, which also have a tendancy to be severely underdeveloped in babies like Grace.
Experience had shown, however, that the holes had a tendancy to close, leaving the babies back in a critical state.
The team of doctors that worked with Grace decided to both cut the hole and implant a stent, or small tube to keep the hole open, 29 weeks into Angela VanDerwerken’s pregnancy.
The VanDerwerkens watched a video of the procedure for the first time at Friday’s press conference. Footage of the procedure is blurry, but there is no mistaking the distinct movements of a beating heart. A shadow passes across the screen and then a slight jolt – the hole is cut, the stent is inserted and blood begins to flow.
A diagnosis of HLHS coupled with the missing passage “strikes fear into the heart of every surgeon,” Grace’s cardiovascular surgeon, Emile Bacha said. But Grace and her mother exceeded his expectations.
“When this baby was born, she was pink,” Lock said, not blue from oxygen deprivation like other babies with her type of heart defect.
Lock, hesitant to make any absolute predictions, said Grace will be followed very carefully.
“I expect her to be a taxpayer and a productive citizen in the future,” he added – a normal life despite a singular birth.
Grace had a second operation when she was four days old, and will need two more in the next two years to streamline her blood flow.
The VanDerwerkens learned their baby had a problem when a 20-week ultrasound didn’t show a four-chambered heart.
The couple had several options: a series of operations; a heart transplant; or “compassionate care, which basically meant do nothing,” Angela VanDerwerken said. That option would have given the couple a few weeks at most with their baby before she died.
VanDerwerken said she never had any reservations about choosing surgery, “It was easy to agree to because we knew that Grace had everything to gain and the risks were minimal.”
Angela VanDerwerken said she hopes the in-utero stent will become routine for other babies like Grace.
“We picked her name after we found out she was a girl, which was when we first found out she had a heart defect. We knew she would be a blessing to our family no matter what. And now she is to the world over. She’s our amazing Grace.”
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On the Net:
Children’s Hospital Boston: http://www.childrenshospital.org
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