LONDON — Surgeons in Britain have saved the life of a baby girl who was born with her heart outside her body as a result of a rare condition that usually leads to the termination of pregnancy or death, the hospital where she was treated said Wednesday.
The Glenfield Hospital in Leicester, England, said the infant, now named Vanellope for a Disney character, was born with a rare condition known as ectopia cordis and had been thought to have less than a 10 percent chance of survival. The surgery to relocate her heart was said to be the first successful procedure of its kind performed on a newborn child in Britain, although a few comparable cases have been reported in the United States.
Her parents, Naomi Findlay, 31, and Dean Wilkins, 43, of Nottingham, England, discovered in June that they were expecting their first child. A scan at nine weeks showed ectopia cordis, with the heart and part of the stomach growing externally, the hospital said in a news release.
The condition is extremely rare — estimated at five to eight cases per 1 million live births. “Because of the risk of infection, as well as the risks from the associated defects, pregnancies may be terminated or babies may die in the womb or soon after birth,” the hospital said.
“All the way through, it was, ‘The chances of survival are next to none. The only option is to terminate. We can offer counseling,’ and things like that,” Findlay, the mother, told The Leicester Mercury newspaper.
“In the end, I just said that termination is not an option for me,” she said. “If it was to happen naturally then so be it.”
Hospital officials in Leicester said the medical team knew that other physicians had tried to perform the same procedure at intervals of roughly two to three years, but none had been successful in Britain.
The baby was delivered by cesarean section on Nov. 22, about a month before her due date, the hospital said. She “was immediately wrapped in a sterile plastic bag” to keep her organs sterile and the tissue moist. A team of around 50 performed the procedure, the hospital said, including obstetricians, surgeons, anesthetists and midwives.
“At around 50 minutes of age, it was felt that Vanellope was stable enough” for the team to begin “the task of putting her entire heart back inside her chest,” the hospital quoted Dr. Jonathan Cusack, a consultant neonatologist, as saying.
Dr. Frances Bu’Lock, a consultant in fetal cardiology, told the BBC that the procedure to save the child was “touch and go.”
The baby was born without a breast bone, Bu’Lock said, and physicians are planning to create some form of artificial sternum at a later stage. After three operations, the infant’s heart, which had protruded through a hole in her chest, is now in her body and covered by her own skin.
In most cases, babies born with ectopia cordis also have heart problems in addition to the heart’s unusual placement; typically babies who survive are ones like Vanellope, who seemingly have no other heart issues.
Jack Rychik, director of the Fetal Heart Program at the Children’s Hospital of Philadelphia, said the prognosis hinges partly on the heart’s structure. In the heart, he said, there are four chambers - two on the top and two on the bottom - as well as vital vessels; but in most cases of ectopia cordis, the heart structure is affected - whether it be holes, blockages or other developmental problems.
Rychik said there are more anticipated favorable outcomes in cases without other heart problems.
“If this particular patient had no structural cardiac abnormalities - just ectopia cordis - and had a normal genetics and chromosomal makeup, and it was detected prenatally and managed in an anticipatory manner - that’s a threading of a needle that’s pretty wide,” he told The Washington Post.
In any case, Rychik said, the challenge with ectopia cordis is the heart needs to be put back into the chest, which is often filled with the lungs and other organs.
“So the way to think about this is, you sort of have to be a bit of an architect and reconstruct the chest around the heart and do so in a manner that does not compress the cardiac structures and allows for function and allows for growth of the chest,” he said.
The baby was named Vanellope Hope Wilkins. The name was taken from a character called Vanellope von Schweetz in the animated film “Wreck-It Ralph.”
Explaining the name, Findlay told The Leicester Mercury: “Vanellope in the film is so stubborn, and she turns into a princess at the end, so it was so fitting. The Hope part is the fact that it has brought us hope.”
Now 3 weeks old, the baby has “more strength than you could ever imagine,” Wilkins, her father, said, according to BBC News.
“She’s fighting it all the way and she’s defying everything, isn’t she?” he added, addressing the baby’s mother. “What they’re saying she can’t be doing, she’s doing it.”
The Washington Post contributed.