What Is Ectopia Cordis? Baby Survives Being Born With Heart Outside Her Body

heart defect
Five-month old Ayush Nayek, who is recovering following surgery to treat an extremely rare heart condition, is carried by his mother Suparna Nayek during a press conference held at the M.S. Narayana Heart Centre in Bangalore on June 7, 2013. Manjunath Kiran/AFP/Getty Images

A child born with her heart outside of her chest is alive. Vanellope Hope Wilkins is thought to be the first baby born in Britain with a condition called ectopia cordis to survive, according to The Guardian. (The child's first name, according to the parents, was inspired by a character in the 2012 movie Wreck-It Ralph.)

Since her birth in late November, Wilkins has had three surgeries to put her heart back in her chest.

"I had prepared myself for the worst; that was my way of dealing with it. I had brought an outfit to hospital that she could wear if she died," Wilkins' mother, Naomi Findlay, said in a press release issued by the hospital. "I'm now confident she won't wear it so I'm going to donate it to the hospital. I genuinely didn't think my baby would survive."

Congenital heart defects are actually pretty common—about one percent of all children born in the United States have one, according to the Centers for Disease Control and Prevention. But this particular defect is extremely rare and usually fatal. In 1870, Dr. Henry Tuck, a physician based in Boston, wrote in the New England Journal of Medicine about a child who was born with this defect and survived for less than 90 minutes. He wasn't the first to see it; at least six other cases are referenced in the article.

The first case labeled as ectopia cordis—at least, the first case Tuck could find—was a child born in 1706 in Madrid, Spain. Another case study, published in BMJ Case Reports in 2012, indicated this particular defect may have first been seen thousands of years ago.

No one quite knows why this happens, according to that case report, but doctors do know that the prognosis is very, very bad. Less than five percent of babies born with the most common type of ectopia cordis survive for more than a month. The baby born in 2012 survived for less than a day.

"Newborns with this complex and life threatening deformity require intensive care right from birth," the authors of that 2012 case study wrote. "They require immediate resuscitation and coverage to the exposed heart and viscera with saline-soaked gauze pads wrapping to prevent desiccation and heat loss."

People have been trying to fix ectopia cordis with surgeries since at least 1925, but no one actually succeeded until 1975.

Since then, however, the picture for some children has gotten rosier. The condition is still serious and still usually fatal, but Vanellope is far from the first to survive worldwide. Doctors reported in Circulation that a boy born in the early 2000s had survived for at least three and a half years after surgery. A girl born in Texas in 2012 had surgery and lived, as did another born in 2014 in North Dakota.