What is ectopia cordis? Baby born with heart outside her body first to survive in the UK

After undergoing three operations after birth, three-weeks-old Vanellope Hope Wilkins, who was born with heart outside her body, has become the first baby in the UK to survive such a rare condition.

Medical experts, including the consultant cardiologist who carried out the operation, said they do not know of another case in Britain where a baby has survived after being born with a heart outside the body.

Vanellope was due on Christmas Eve but was delivered by caesarean section on 22 November, five weeks early to reduce the chances of infection and damage to the heart. A team of around 50 medical staff including obstetricians, heart surgeons, anaesthetists, neonatologists and midwives handled the delivery at Glenfield Hospital in Leicester, England.

Within an hour of her birth, she underwent her first operation followed by two more to place her heart back in her chest.

Doctors at the hospital had told her parents – Naomi Findlay and Dean Wilkins – to terminate the pregnancy after a nine-week scan discovered that the baby has ectopia cordis, with her heart and part of her stomach growing externally.

"It was a real shock when the ultrasound showed that her heart was outside her chest and scary because we didn't know what would happen. We were advised to have a termination and that the chances of survival were next to none - no-one believed she was going to make it except us," Naomi told the BBC.

The proud parents of Vanellope, who has been named after a character in the Disney film "Wreck-It Ralph", said that they were told that the first ten minutes after birth were crucial as her ability to breathe would be essential.

"But when she came out and she came out crying, that was it. The relief fell out of me. Twenty minutes went by and she was still shouting her head off – it made us so joyful and teary," the mother said, who believes her daughter is a real fighter.

Minutes after her birth, the little girl was wrapped in a sterile plastic bag to protect against infection. Also, special lines were inserted into the umbilical cord to support her heart when she was still attached to her mother.

Vanellope went for her first surgery 50 minutes after coming into the world. A second operation followed a week after where her chest was opened a bit more to create space to allow her heart to fit back in.

In the most recent surgery, the toddler's skin from under her arms were taken to cover the middle of her body. Surgeons have also created a mesh to protect her heart as she did not have ribs or a sternum. Vanellope is still on a ventilator as her organs fight for space inside her chest, but doctors believe she will soon go on to lead a normal and healthy life.

Expressing his happiness, her father Dean said, "She is doing really well. She's a fighter." He also said that Vanellope's story will surely bring hope to others.

"Some mums still terminate. If we can get out there that there is a hope and it can be done then it's giving all those mums out there a chance," he added.

Frances Bu'Lock, consultant paediatric cardiologist told the BBC: "Before she was born things looked very bleak but now they are quite a lot better - Vanellope is doing really well and has proved very resilient.

"In the future we may be able to put in some internal bony protection for her heart - perhaps using 3D printing or something organic that would grow with her."

According to the Guardian, babies born with ectopia cordis, estimated to be one in five to eight per million, have less than a 10% chance of survival.

In the US, there are few examples where babies with this condition have survived. One of the examples include Kieran Veitz of North Dakota, who was treated in 2015. She is now three and is living a normal life.

In another case, an eight-year-old Russian girl, named Virsaviya Borun, is also suffering from the Pentalogy of Cantrell, a less severe case of ectopia cordis and is currently in the US to seek treatment.

Unlike Vanellope, Virsaviya's heart is protected by a thin layer of skin. A part of her chest bones, a diaphragm and abdominal muscles are also missing.

What is ectopia cordis?

Ectopia cordis is a rare genetic defect that develops before birth in which the heart is abnormally located either partially or totally outside of the thorax or chest. In this condition, the heart can be found along a spectrum of anatomical locations, including the neck, chest, or abdomen.

Health Line website states that the condition affects about one in 126,000 births and treatment options are limited. Complications due to ectopia cordis are difficult breathing, low blood pressure, poor circulation and electrolyte imbalance (dyselectrolytemia).