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Oscar’s wonky heart

The last time I spoke about this on the blog was before Oscar was earth-side. I brain dumped (as I always do in this blog) everything that was going on at the time. But in the effort to cut a really long story short, here’s the low down:

At the 20 week scan when I was pregnant with Oscar, it was found that he had a rare anatomical heart variant – a Right Aortic Arch. This type of heart defect is associated with types of cardiac disease and some syndromes, specifically DiGeorge syndrome. They could see from scans that he didn’t have any obvious cardiac diseases and I had an amniocentesis to rule out genetic complications like syndromes so for the most part it was assumed that it was just a rare variant.

The aorta has only been studied on ultrasound for the last 5 or so years and a right sided aortic arch has been known to be a variant with no other complications. For instance people well into their adult lives have been for ultrasound scans for other reasons and they have coincidentally found a right aortic arch. They are completely healthy people as far as their heart is concerned. Likewise, post mortems have been done on 80yr olds and it has been found. So it really can be one of those rare quirky things that means absolutely nothing.

It has been explained to me that a heart with a right arching aorta is effectively a mirror image of a ‘normal’ heart. There are 3 vessels that branch off of the aorta, one of which immediately branches into a 4th vessel. With a right aortic arch it is important ensure that the vessels are complete and still go to where they are meant to but they can often take unusual routes to get to where they need to. A common complication of a right aortic arch is a vascular ring which forms around the trachea and oesophagus as a result of the vessel trying to get to where it needs to. This can cause children to have problems with their breathing,  gagging and swallowing, and poor weight gain.

As far as I am aware, it is impossible to diagnose a vascular ring antenatally.  Therefore for Oscar, he has simply had cardiac appointments at Brirtol Royal Hospital for Children to keep an eye on him since he was born. He should have had his first appointment when he was 8 weeks old but because of Covid that didn’t happen. His first appointment was in November 2020 (just before he was 9 months old). He absolutely hated having the tests done and they only got partial images of his heart under echo at that appointment. The rest of his tests were fine and he has none of the associated symptoms so they just gave him a follow up appointment for a year’s time. The only thing that did happen whilst we were there was that he completely lost it at the end of the echo and screamed blue murder. On this, his consultant consultant some other Dr’s came rushing into the room to hear him. He had a stridor. A stridor is a noise heard when there could be an obstructed airway. Once they pointed it out to me, I recognised that he had had it in the past but only when he was really really upset. I has just put it down to that. a stridor can be a sign of the presence of a vascular ring. However at this point because there were no other worrying features it was just a case of wait and see and review again in a year.

In the last year the stridor has disappeared, probably mainly due to the fact that it was only audible when he got really really upset, which doesn’t happen very often anymore. He doesn’t display any other the other symptoms in normal day to day life either. However, we have noticed that when he gets poorly, he gets really poorly, and very often develops a violent cough that hangs around for weeks and weeks, even though he’s only had a minor cold. He coughs so hard that he vomits, a lot. In the summer this lasted 6+ weeks and in the end the local hospital (urgent care centre) put him on antibiotics suspecting a tracheal infection – there was no obvious signs of a chest infection but frequent chest infections can be another symptom, not that we knew this at the time.

Anyway, his next follow up appointment was delayed as we moved house and I forgot to inform them. So he was probably sent an appointment in November but we never received it. Instead, after a particularly bad bout of him being ill, I called the cardiology nurses who got him sorted for an appointment fairly quickly.

He had that yesterday. We happened to go on a quiet day for them as they had a couple of cancelled appointments and a couple of no shows. I think this was was good thing as we were in there for what felt like forever. It was 2hrs, but he must have been under the ultrasound scanner for at least an hour, probably longer. That’s a LONG time for a (nearly) 22 month old kid. They started with simple observations and an ECG. Which he HATED, so I had very little expectation of them actually being able to get useful images this time too. With a bit of bribery with chocolate buttons then distracting him with Paw Patrol, the sonographer seemed to get what she needed to… but then half way through us talking with the Dr, the consultant came in and interrupted, saying that unfortunately they needed more images… so after already a LONG time, he had to sit again for another AGE of time, they were finally happy. He was an absolute superstar and just laid with me watching TV. He did SO SO amazing!

It was also quite obvious by this point that there was something specific they were investigating. This wasn’t straightforward. Finally the consultant sat down with me (dad wasn’t allowed on because of Covid…) and confirmed that he does indeed have a vascular ring. They don’t know for certain how exactly it affects him as that can only be seen on MRI but they were able to trace the offending vessel to a certain degree. That’s what took so long. They could also see that the 4 vessels of the aorta all brach off independently (remember I said they start as 3 vessels and then one branches again to create the 4th… I know, I told you that info was a long time ago now!). I don’t entirely know what that means for him but I think it’s the 4th vessel which has formed the vascular ring.

For now, this doesn’t mean an awful lot. They definitely think that the vascular ring is to blame for his struggles when ill, but as he doesn’t get any symptoms when he is well, there is no immediate reason to rush to do anything. He will have a specialist respiratory referral for an MRI (and another new test which will hopefully mean he won’t have to have an endoscopy) to look into how significant the vascular ring is and then they will decide from there if anything needs to be done. The NHS current waiting time for this specialism is currently around 18 months…. so he has his next specific cardiology review in 2yrs. He’s allowed to have this much of a wait because essentially he is a well kid but I am under strict instructions to get back in contact as soon as I suspect he is getting more symptoms. it’s one of those things that may well not cause him any more problems, but time will certainly tell that. It is likely that as he grows symptoms will get worse because the vascular ring will become tighter around the trachea and oesophagus, they grow, the vascular ring wont. Worst case he will have to have heart surgery to separate the ring.

But do you know what? If that is the worst of his problems and after that, he is a completely well kid, we can only be thankful that we have been so fantastically counselled and taken care of through this whole process. He is such a sweet natured boy, he deserves the best of everything and we want whatever is right for him. I think back to having the amniocentesis when I was pregnant and thinking about possibly having to consider the worst. We are so thankful that he is as well and he has every chance of a completely normal life. I am so so aware that there are so many kids out there with much worse diagnosis’ than his, with much scarier times ahead than us.

In view of the symptoms he gets now when he is poorly, I fully suspect that there will be more to come in the future, but he’s a tough cookie, and we can take this on with full force. And I am so thankful to Bristol Royal Hospital for Children for everything they have already done for us, not to mention what may come our way in the future xxx

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