Paediatric Cardiologist from our University part of team that helped to deliver miracle baby with heart growing outside of her body

Academic Champion in Paediatric Cardiology Dr Frances Bu’Lock, from our University’s College of Life Sciences, has worked as part of a team of around 50 clinical staff at Glenfield Hospital to bring Vanellope Hope, a baby with an incredibly rare condition which had her heart growing on the outside of her body, into the world safely and give her a fighting start in life.

Naomi Findlay and Dean Wilkins from Bulwell, Nottingham found out they were expecting their first child together in June, but a scan at nine weeks brought them some scary news that they were not expecting.  Scans showed that their baby’s heart and some of her stomach had begun to grow on the outside of her body.

This incredibly rare condition - Ectopia cordis – was confirmed at another scan carried out by Glenfield’s Fetal Cardiology consultant and Leicester’s Academic Champion in Paediatric Cardiology Dr Frances Bu’Lock. 

Dr Bu’Lock said: “After 32 weeks we could begin plan for the delivery and baby’s surgeries. An MRI scan of Naomi’s abdomen was done to check the bony structures of her baby’s chest wall, as well as her lungs and brain.  We came together as a team of fetal medicine doctors, obstetricians, anaesthetists, cardiac and abdominal surgeons and cardiologists, to review all of the available information and discuss how best to plan for a delivery, surgery and subsequent care.

“It was decided that delivery by caesarean section would be best to reduce the risks of infection, risks of trauma or squashing of the heart during delivery, and that surgery to provide some sort of covering to the heart would be needed immediately after baby was delivered.”

On Tuesday 21 November at 35½ weeks pregnant, Naomi and Dean came to Glenfield Hospital to prepare to deliver their baby in one of the cardiac theatres at Glenfield the following morning. Naomi was wheeled into theatre just after 9am and was met by four main teams of doctors, midwives, nurses and allied health professionals, numbering almost 50 people in all, who were all going to play a part in delivering baby and keeping her and mum safe.

At 9:50am baby Vanellope Hope was born by Caesarian section.

Consultant Neonatologist Jonathan Cusack said: “At birth, the baby was placed in a sterile plastic bag - we do this with other babies that have organs such, as the bowel outside of the body.  The bag keeps the organs sterile but also keeps the tissues moist.  We inserted a breathing tube into her mouth and gave medications to sedate her and stop her moving.  We then inserted special lines into the blood vessels in her umbilical cord, to allow us to give fluids, medications to support her heart, and to monitor her blood pressure accurately.

“Vanellope was born in good condition. She cried at birth and coped well with the early stabilisation and her heart continued to beat effectively.  During the stabilisation, we continually dripped warmed saline solution onto her heart to stop the tissues drying whilst she was under a warmer.

“At around 50 minutes of age, it was felt that Vanellope was stable enough to be transferred back to the main theatre where she had been born to the waiting anaesthetists, congenital heart disease and paediatric surgical teams who began the task of putting her entire heart back inside her chest.”

The actual defect in baby Vanellope’s chest wall was quite small.  The main concern with re-positioning her heart was that the arteries and veins which bring blood to and from the heart were extremely elongated, and might become kinked and blocked when the heart was placed inside the chest wall. 

To ensure this didn’t happen the plan was to use a special splint to support the edges of the larger hole that had been created in the front of her chest, attached to its own plastic tube. 

This meant it was possible to hang pull the chest wall forwards so the hear could be allowed to fall back into the chest under its own weight as far as possible, and allow a plastic sheet to be stitched around it to seal the heart away from the outside air.

Babies born with this condition generally have less than a 10 per cent chance of survival, depending on the risks of other conditions as well as the ability to place the baby’s heart safely in the chest.  Cases such as Vanellope’s, where everything else appears essentially normal, are even rarer, and whilst therefore it would seem more hopeful she will do well, it is therefore almost impossible to be confident of this.

Consultant Anaesthetist Dr Nick Moore added: “We transferred baby Vanellope to our Paediatric Intensive Care Unit at 1.35pm on the 22 November where she will need to stay for at least several weeks, with her family by her side, while she hopefully gets strong enough, and big enough, for her heart to be placed fully within her chest and covered in her own skin. She has a long way to go but so far at least she now has a chance at a future.”