Girl born with heart outside her body survives surgery
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.
Baby Vanellope Hope’s birth day was supposed to be Christmas Eve. But an incredibly rare condition which had her heart growing on the outside of her body meant she needed to be brought into the world early. On Wednesday 22 November a team of around 50 clinical staff at the world famous Glenfield Hospital in Leicester, worked together to bring her into the world safely and give her a fighting start in life.
This incredibly rare condition - Ectopia cordis – was confirmed at another scan carried out by Glenfield’s fetal cardiology consultant Frances Bu’Lock. She said: “When Naomi was 13 weeks pregnant I received a call about the potential complications that had been identified by a sonographer in Nottingham. I scanned Naomi at 16 weeks when her baby was a little bit bigger and by this time it seemed that the bowel was back where it should be, but that the heart was still beating away outside the front of baby’s chest. Other than that, baby appeared essentially normal.”
Naomi and Dean were given counselling and support so they could understand the very major risks to their baby. It was explained to them that there may be chromosomal abnormalities, or other problems that had not yet been detected through scans. These along with the risks the baby faced with potential damage to the heart and circulation in the womb, could lead to the pregnancy ending before she was born.
Undeterred in their fight for their baby, Naomi and Dean paid for a special blood test to check the risk levels of the three most serious chromosomal problems. Dean said: “When the results of that test came back as low risk of any abnormalities we jumped up and down in the living room and cried. At that point we decided to fight to give our daughter the best chance of surviving.”
Consultant in Fetal Cardiology Frances Bu’Lock said: “Naomi and Dean understood that for their baby to have any chance of survival outside the womb, Naomi would need to get to as close to her due date as possible, and baby would need to grow well and not to develop any other problems.”
“We explained to them that baby would need surgery as soon as she was born where we would work to keep the heart safe so it could go back into the chest cavity and be covered with skin. However, chances of successful surgery and long term survival were very poor; they understood that and wanted to continue with the pregnancy.”
However the pregnancy didn’t go smoothly. At 25 weeks baby’s heart rate was quite a lot slower than would be normal at this stage (around 90 – 100 beats per minute). This was of course a worrying time for everyone involved, but baby continued to be very active and grow quite normally. Naomi had regular scans to check baby’s heart rate and growth, which were all reassuring.
Consultant Frances Bu’Lock added: “At 32 weeks we began to plan for the delivery and baby’s surgery. 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.”
“We talked through this plan with Naomi and Dean so that they understood what we needed to do, and how we would all work together to keep Naomi and her baby safe.”
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.
The teams began to assemble from around 8am bringing all their specialised equipment, making checks and getting prepared, before a theatre briefing at 08.30am. 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.
On remembering how it felt to go into theatre that morning with Naomi, Dean said: “Both Naomi and I were holding our breath waiting to take her take her first breath; we didn’t dare breathe until she took her first breath. When she cried, we cried. I felt hopeless and just held onto Naomi and was staring into her eyes praying that it was all going to be ok.”
At 9:50am baby Vanellope Hope was born by Caesarian section. She was immediately wrapped in a sterile plastic bag, said a quick ‘hello’ to mum and dad and was whisked into the adjoining anaesthetic room where our senior neonatal specialists inserted a breathing tube, drips, and anaesthetised her. Special care was taken to protect her tiny heart and keep it sterile and moist.
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 her heart outside of her chest to help create more space within, 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.”
Dean said: “The moment she was born I realised that we had made the right decision. We know this is going to be a rollercoaster and have started to prepare ourselves for the difficult times ahead, but we needed to give her a chance, and the team here have done that. People always knock the NHS, but all we have seen from the team at Glenfield is kindness and a desire to keep Naomi and Vanellope safe and I can’t begin to thank them for what they have done for my girls.”
“Regardless of what the future holds we wanted to share our story and how the team at Glenfield stood by us and fought to give her a chance of life.”
Mum Naomi added: “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. 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, but the staff at Glenfield have been amazing. They’ve supported us, explained everything that might happen and involved us in the plans to keep my baby safe. I can’t put in words how grateful I am for everything they have done. They are amazing!”
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