A hole in the heart...
Updated: Apr 19, 2022
Hello. Thanks for stopping by. This post is not a particularly eloquent (or interesting!) piece of writing but I wanted to share Noa’s cardiology journey on the off chance it helps someone in the future and also to spread awareness of Congenital Heart Disease. I know that in the future Noa will have a lot of questions and so I also wanted a record of how it all went down. At the bottom of this blog you’ll find some more general information about CHD, useful links and resources.
Here's our story...
PART 1 - Diagnosis
PDU (29th January 2021)
In January 2021, when Noa was 9 months old, she had a fever for 10 days. She’d been on antibiotics for 5 of those but her temperature continued to spike so after speaking to our GP we were sent to the Paediatric Decisions Unit (PDU) at Stoke Mandeville Hospital to get her checked out.
At Stoke the usual checks were performed - temperature, blood pressure, a little device round the toe to measure blood oxygen saturation and a quick listen to her heart and lungs. The doctor who listened to Noa’s heart and lungs with a stethoscope made a little “hmm” and said she’d be right back with her colleague. A few minutes later she returned and doctor number 2, a paediatric consultant, who also had a listen and said, “We can hear a loud murmur, a very loud one”.
He explained that sometimes babies get murmurs when they have infections because their hearts can swell, but that this was not that, this was a Grade 4 murmur, which meant something structural was causing it. They did an ECG and at around midnight we were sent to get a chest x-ray before being admitted to the ward overnight so we could see the Cardiologist, the next day.
Dr Dey (30th January 2021)
After a very noisy and sleepless night on the ward we met Dr Dey (a Paediatric Consultant with a special interest in cardiology). Incredibly he diagnosed Noa’s defect with just a stethoscope and then confirmed his findings with an Echocardiogram. We were told Noa had a form of Congenital Heart Disease, in her case an Atrial Septal Defect or ASD (a hole in the septum between her two atria) and a Pulmonary Stenosis (a narrowing of the artery that takes blood to the lungs). Dr Dey said that both things were mild, very common, that there was a chance that Noa would need surgery (but also a chance she may not) and if she did, that it would be when she was around 4/5 years old and that often they could close the hole with a little device and via a catheter inserted into her groin, rather than requiring open heart surgery.
The next step was for her be assigned to a Paediatric Cardiologist who would do some more scans and give us more information in a few weeks time.
Dr Adwani (26th February 2021)
A month later we met Noa’s Cardiologist, Dr Adwani. After doing another Echo he said something along the lines of: “So as you know, Noa’s hole is very big, she will need to be operated on soon and because of the size and location of the hole we cannot do the operation via a catheter so it will be an open chest surgery”.
No, no I did not know that.
And of course because of Covid I had to digest this news on my own (Rhodri hadn’t been allowed into PDU with us previously or to this appointment) and try to wrangle a very distressed baby who hated having the echo’s done whilst trying to vocalise the hundreds of questions suddenly screaming in my head.
When? Where? What will happen? What are the risks? How big will the scar be? How long will we be in hospital for? Will she need further operations? Why has this happened to her? Was it something I did? How does it make her feel now? Is this why she was always so sad as a baby? Why she gets so cold? Why she’s been poorly a lot?
He said that it was “pretty urgent”, but not so urgent that we couldn’t wait for things to calm down a bit with Covid. Noa weighed just over 9kgs and ideally she would weigh 10kg before they operate. He said he’d see us again in June and aim for surgery (which would be down in Southampton and performed by someone else) after the summer holidays.
Back to the Ward (18th March 2021)
We ended up back in PDU in March and were admitted to the ward for 5 days as Noa was very poorly with an infection they couldn’t diagnose (a horrible week, we couldn’t leave our room and Noa had to undergo lots of tests including a lumbar puncture, which was pretty horrific) and then again in April when, fortunately, we weren’t kept in overnight. It’s hard to say whether Noa’s heart condition makes her more prone to upper respiratory illness. Some doctors have said yes and others no. She’s had 4 bad bouts in 18 months plus other less significant coughs and colds but her circulation is definitely affected, she get very cold and often gets blue lips when she’s cold, tired, expending a lot of energy or after meals, when her bloodflow is focussed on her stomach. Her heart is also enlarged on one side where it’s been working really hard.
From about March/April Noa stopped gaining any weight. When we saw Dr Adwani again in June she hadn’t gained any weight in 3 months. We were referred to a dietician who was really helpful and gave us lots of helpful advice on how to add more good fats to her diet and Noa finally hit the 10kg mark recently. She’s 18 months now and has just started fitting her 12-18 month clothes.
Cardiology follow-up (15th June 2021)
In June we went back to see Dr Adwani at Stoke Mandeville. Rhodri wasn’t technically allowed in but came along anyway and we put our foot down a little bit when questioned by a nurse. Heart surgery is serious and we had lots of questions but when Noa is distressed (as I knew should we be after another Echo) my brain just can’t focus on anything except her. Fortunately, the nurse was quick to agree and let him in.
Dr Adwani attempted to do another echo but after Noa’s last couple of hospital visits she had clearly developed a huge fear of hospitals and doctors (who could blame her) and she was very upset during the scan (what he lovingly referred to as “uncooperative”). This echo was not just for having another quick look, Dr Adwani needed images and measurements to take to a multidisciplinary team meeting where they would discuss Noa’s case and decide on a plan of action. Because he couldn’t get what he needed we would have to go in again (to the John Radcliff in Oxford this time) for Noa to be sedated for a scan.
Sedated Scan (9th July)
We had to wait about 3 weeks for that appointment. I was dreading it, I didn’t really know how they were going to sedate her, how long she’d be out for and where I would be when it happened.
We got to Oxford and a lovely nurse settled us into a room. The usual checks were done and then we were told we were just waiting for Dr Adwani to start making his rounds on the ward. A nice play nurse brought us some toys and there were some workmen outside the window who Noa enjoyed watching. She’d had to be nil by mouth since 4am, her appointment was at 10am but Dr Adwani didn’t arrive until 11.15. He checked her over and a nurse asked if I could administer the sedation (which was in liquid form in a syringe to be taken orally). She said it can be quite bitter… understatement of the century. When I put some in Noa’s mouth she LOST HER SHIT. So then I had to battle to get the rest in whilst feeling awful and then trying to comfort her, when I was the one who’d just force fed her the purple poison. She cried for about half an hour non-stop, I was a total wreck by the time she finally conked out. So stressful!
Dr Adwani and another colleague brought the Echo machine into the room and proceeded to scan Noa for a full hour, taking over 100 images. It was fascinating watching but pretty eye-opening, seeing the size of the hole, which is nearly 2cm and basically the whole of the length of the septum between the two atria. I said it looked more serious than I had imagined and asked how she was coping so well, he said babies are very resilient but that we would start to see more serious symptoms if we left it much longer.
When Noa woke it was 1pm, she must have been starving. She fed from me and then ate lots of the packed lunch I had brough while the nurses observed her to make sure she was ok and not too drowsy. She was amazing though, bounced back really quickly and chatted all the way home.
Southampton (25th August)
We were finally referred to Southampton and given a date to meet Noa’s surgeon, Dr Antonio Ravaglioli. We travelled down on a Wednesday and waited in the Paediatric Cardiology Outpatients area. There were quite a few families there with the cardiac kids. Everyone was supposed to be social distancing but one little boy, Enzo, was drawn to Noa’s digger collection so we let them play.
Dr Ravaglioli was late and clearly very very busy, he rushed into a room and took a few moments to chat to a nurse but when we were called in, he sat back and smiled and acted like he had all the time in the world for us. I told him Noa was very shy and not a fan of doctors, so he didn’t force anything, he just put a toy train near her and let her slowly warm up to him. He talked us through what he planned to do, which is to use a piece of pericardial tissue (tissue from the outside of her heart) to plug the hole. He told us he wasn’t sure about the pulmonary stenosis diagnosis and that the reduced output through the pulmonary artery might be down to the heart struggling to push the blood through because of the hole, so he said he’ll have a look when he’s in there but fixing the first problem might fix the second. He said he wanted to operate at the end of October/beginning of November and that we’d get a call probably 2 weeks before.
While we were down there Noa had a few other checks including a swab for MRSA, which came back positive. Apparently 1 in 30 people have MRSA naturally occurring on their skin and it is completely harmless until you have open wounds where the infection can get in. We treated Noa with a body wash and nose cream in the run up to surgery.
A little more information about the operation
Dr R will make an incision nearly the full length of her chest, top to bottom. He will saw through her sternum and open her ribcage. Noa will need to be put on a heart and lung bypass machine, that will pump blood round her body so her heart can be opened and operated on. The whole thing should take 2-3 hours. Afterwards she will be in the PICU (Paediatric Intensive Care Unit) for 24-48 hours, then high dependency and then onto Ocean Ward, which is a ward specifically for ‘Cardiac Kids’. Southampton is a specialist centre in the UK and Ocean ward has the most amazing reputation. I’ve met lots of amazing parents who’ve been down there with their children and they’ve all praised the staff and set up.
PART 2 - Surgery
I wrote all of the above before we had a date for surgery. They told us we’d get 2-3 weeks warning and we were given just less than 2 weeks. We isolated from the Monday, did covid tests on Tuesday, were due to travel to Southampton on Thursday with surgery scheduled for Friday, but when we called on Wednesday afternoon as instructed we were told not to set off the following day because there were no beds available and the following morning they called to say surgery was cancelled. We really struggled with this news, we had prepared ourselves mentally and suddenly didn’t know how long we’d have to wait, plus it was frustrating after a week of isolating. We spent all day on the phone and eventually managed to get a new date only 9 days later. So we had a lovely weekend out of isolation and then started again the following week. Surgery was set for Monday 1st Nov, we called on Saturday and they told us there was a bed but to call again before setting off the following morning. Sunday morning (Halloween), we were given the green light. It was happening…
The day before (Sunday 31st October)
We arrived at Ocean Ward, a ward dedicated to the care of kids with Cardiac problems, at 11am on Sunday morning. We were shown to a private room (the one advantage to Noa’s MRSA diagnosis) and told to settle in. She had a day of tests and at some point we were told we’d been bumped from 1st on the list to 2nd, so we knew she’d be operated on sometime the following afternoon. She had an echo, xray, ecg and a cannula put into her hand.
Rhodri couldn’t stay in the room overnight and we weren’t told until 8.30pm that evening that he wouldn’t be able to stay in the hospital’s Ronald McDonald accommodation because of Noa’s MRSA diagnosis, so he had to find a hotel last minute. Noa slept through that night, much to my surprise. I didn’t sleep a wink.
Surgery Day (Monday 1st November)
It was a long wait until surgery on the Monday, Noa was nil by mouth from 5am. I woke at 4.45am to give her a cup of milk and try to give her some food. It was too early and she wasn’t really interested in either but she had a little. I had no idea how she was going to cope until 1pm with no food and just sips of water but she did amazingly well with lots of distractions and Thomas on the tv.
In the end she waited until 2.30pm. When they finally called us she was still attached to a little machine slowly administering antibiotics into her cannula. As we walked to theatre Rhodri held her whilst I held the machine. We walked into the very warm anaesthetic room and she instantly started crying, there were so many people in there, all wearing masks. I was trying to comfort her but was holding back my own tears and could barely speak. They asked us a few questions and started adding drugs to her cannula, so I sat her down on the gurney and cuddled her while she got sleepy and then lay her down. They said we could both give her a kiss goodbye, which caused us to both totally break down. A doctor who was holding a mask to her mouth and nose moved it away and we both kissed her before being lead out and back to our room on the ward.
The next few hours were easily the worst and longest of our lives. We went for a little walk outside but the closest green space to the hospital is a massive cemetery, so we went the other way. We were just walking aimlessly and we felt too far away from her so we quickly turned back and returned to our room. We had been told the surgery should take 2-3 hours but it ended up taking 4.5. The first 3 hours was difficult but that last hour and a half was agonising knowing something had not been straightforward. We asked for news a few times but there was nothing. Our room was on one side of an atrium and the window was directly opposite a corridor with a door that lead to the anaesthetic room we had left her in. Every time the door opened we held our breath waiting to see if it was Dr Ravaglioli leaving.
We eventually saw him leave theatre, it was just before 7pm, 10 minutes later her came into our room. He took one look at me and the first thing he said was “it’s OK, you can breathe”. I burst into tears. He explained that the operation had been much more complicated that expected but that it had gone well.
Dr R explained that at the beginning of the surgery Noa had a couple of episodes of desaturation, which is unusual for this kind of surgery. Then when he was putting her on bypass he couldn’t drain her heart. He realised that half of her pulmonary veins were draining to the right side of her heart instead of the left, this is a rare condition known as PAPVD or Partial Anomalous Pulmonary Venous Drainage. He also found she had PDA or Patent Ductus Arteriosus which means that the little opening that exists between the aorta and the pulmonary artery in foetuses (that bypasses the lungs because they’re not needed yet) hadn’t closed in the few days after birth like it should have. He said Noa’s right atria was receiving 80% more blood volume that it should have been and if they had just plugged the hole and not noticed the other issues, the pressure would have killed her. So he not only had to patch the hole with her pericardial tissue, but also close the duct and re-route two of her veins.
Dr Ravaglioli said the surgery had gone really well despite the surprises and that her heart was pumping normally again and not requiring any medication to do so. He said she would be settled into PICU and then we would be called to see her. As you can imagine we couldn’t find the words to convey the gratitude we felt towards him. What a day job.
The relief we felt was unreal but we knew she wasn’t out of the woods yet. She’d be kept sedated and ventilated overnight at least and we just had to hope she didn’t get any kind of infection or have any problems when they removed the breathing tube.
Looking back at the photos and videos now I can’t believe it’s been a week already. Noa was in PICU for three days but they removed her breathing tube, chest drains and woke her up a bit that first morning. She didn’t cope particularly well to start with so they increased her sedation again and let her rest for longer but I got to have a cuddle and it was brilliant to see her breathing on her own with only a little oxygen though a nose tube.
The next day
On Tuesday she started to wake up a bit more. She made us all laugh by asking for a massage before promptly passing out again. On Wednesday morning we were told she’d pulled out her own feeding tube overnight, so she was able to drink oat milk through a straw and she had a fruit pouch. Her catheter had also been removed. Her throat was clearly very sore, and she had ulcers on her tongue from the breathing tube, but she was very thirsty asking for water, so we gave her as much as we were allowed. She also had a lot of congestion on her lungs and was too weak to cough properly but everyone told us that she would in a few days time.
On Wednesday evening she was moved back to the ward, Noa vomited a lot just after we got there so they decided to see how she did on just paracetamol and ibuprofen (because morphine can make you feel nauseous) and they wanted her to start eating more. That went really well. Her temp was up and down but the paracetamol kept it mostly at bay. We were encouraged to get her walking. She was very wobbly at first but improved so quickly and the exercise helped her to take deeper breaths and cough up the mucus in her lungs.
On Thursday Noa had an echo, which all looked good, and they changed her dressing for the first time from a solid one to a honeycomb one. It was the first time we’d seen her scar. Before the surgery it was very hard not to get fixated on the fact that she’d have this huge scar for the rest of her life down the middle of her chest so I thought this would be an awful moment, but we were both just really impressed with how neat it was. If anything, I was grateful for it. Now that we knew she had been dealing with two other defects and we had been through the terror of leaving our baby in that anaesthetic room, it just didn’t matter anymore.
In order to change her dressing and remove her pacing wires (wires that are attached to the outside of the heart then brought through the skin for use in the event that she arrested or her heart rhythm was off) they gave her some sedation. Unfortunately for us, it had the opposite affect to usual and Noa spent all afternoon high as a kite, acting like she’d been down the pub all morning on the Stella! She was off her rocker. Throwing herself around, arching her back, running into things. If anyone had been measuring my blood pressure they’d have called for a consultant! It was very scary trying to stop her from hurting herself only a few days after surgery and bedtime was not fun!
On Friday Noa was sent for an x-ray which looked fine and there was talk of us leaving the next day and then on Saturday morning we were given the green light. She had the last of her stitches and dressings removed, including the main one but the little wounds where her drains had been but weren’t quite healed, so she had some Steri Strips applied to those. At 2.45pm on Saturday, exactly 5 days after Noa’s surgery began, she was discharged.
Her recovery has just been astounding. A week later she’s only having pain relief 3 times a day, diuretics in the morning and apart from some bruises where her central line was, when she’s clothed, she just looks completely normal. We keep calling her Noa V2.0.
Her scar will take some time to heal but will be very thin when it does. We’ll have a check-up at the John Radcliffe in Oxford in a week and then a few regular ones before they drop down to 6 monthly and then annually for the rest of her life. I asked one of the doctors if she’d ever need another surgery “no”, if she can live a completely normal life “yes” and if she could be an Olympian “if she wants to be”.
For the next six weeks we can’t pick her up under her arms, we need to avoid illness because excessive coughing, sneezing or vomiting wouldn’t be good for healing. Her sternum will be pretty solid after 10 days (we’re already on day 7!) and we need to keep her scar covered in the sun. But other than that, she should be good.
The cheesy bit…
If you’ve got this far I’m pretty impressed but I can’t end this without saying some massive thank yous.
Firstly to Dr Antonio Ravaglioli, her amazing Paediatric Cardiothoracic Surgeon and his surgical team and secondly to all the incredible doctors and nurses in Southampton Paediatric Intensive Care and on Ocean Ward, especially to our lovely friend Toni, who I usually only ever see when we’re drunk at parties so seeing her in her professional capacity as a PICU consultant was impressive (and weird!) but mostly hugely reassuring.
I want to say a huge thank you to the other ‘cardiac mums’ I’ve been introduced to. Carys, Caz, Claire and Lucy who have all been through a similar thing and have supported me with hundreds of Whatsapp messages over the last few months. As with everything, it’s important to find your tribe so as not to feel alone and these four women have been there for me every step of the way.
To all of our family and friends who have been so supportive – thank you.
Congenital heart disease is a general term for a range of birth defects that affect the normal way the heart works.
The term "congenital" means the condition is present from birth.
Congenital heart disease is one of the most common types of birth defect, affecting almost 1 in 100 babies born in the UK.
Little Heart Warriors