Aortic Stenosis / Hypoplastic Left Heart Syndrome at 20 weeks - any experiences?

[LittleMy34]

Hello all,

Have just had a foetal heart scan at 20 weeks following a high nuchal translucency measurement, and unfortunately they found that the baby has severe aortic stenosis - a narrowing of at least one and possibly two of the main arteries. They seem to think that it's fairly likely to lead to Hypoplastic Left Heart Syndrome by the time the baby is born, which is very severe and the baby would die within a few days without major surgery.
If we go ahead, the baby would need surgery within a day or two of birth, followed by two more ops before he reaches school age. the fist op carries a 30% mortality risk, the second two are less severe.

So now we're trying to decide what to do - which is proving a bit difficult, to say the least..... Neither option seems like the obvious thing to do.

anyone had this experience? how on earth did you go about making a decision?

any thoughts welcomed.

LittleMy

I'm a sonographer. I scanned a friend. At the time I did not have the training to pick up this condition, and I didn't see it. My friend lives somewhere they do not offer anatomy checks, so I was doing a 'favour' letting her see baby and get pics. Her baby was fine for 1 week and then was rushed to hospital as he was floppy and blue, and had surgery at 9 days old. He then got enterising (sorry, sp) colitis and was in SCBU for 6 weeks. He is very lucky and is FINE now- he goes for annual check ups now he's two, but ATM is unlikely to require further surgery. His Dad actually had the same condition ( I didn't know until afterwards, he's fine too),and the surgery when a few weeks older but they were told at booking with this pg it was not a genetic condition, and it wasn't considered until he was ill.
The only side effect from the bowel problems is my friends little boy does get constipated, but not all the time and does depend on his diet (so it could be unrelated but his parents don't think so).
It makes me go cold that if I had picked it up at the scan he might not be here had his parents been directed/pushed towards termination. (I'm still having major guilts about not seeing it, despite it not being something I was trained to look for).
I completely agree with whats been said above- you're in such a difficult position with the curse of modern technology- so often it can reassure but the rest of the time it raises dilemmas we wouldn't have a hope in contemplating 10, 20 30 yrs ago.
I hope the information and helplines and websites everyone else have provided can help you, and hugs and support from me with whatever you decide.
mumofk

I don't know how helpful this will be but I think it is good to hear the positive outcomes too.

Friends of good friends of ours were given a similar diagnosis at their 20wk scan. Could have been the same, all I know is that he had major heart problems, the parents were told he would not grow properly, may not survive to full term and if he did would diefinitely need heart surgery in the first few days and probably wouldn't live much longer than that.

What actually happened was that he continued to grow strong and healthy and was born at full term weighing over 9lbs. He was doing so well they have postponed any thought of surgery till he is at least a year, and in the meantime he is a thriving, happy 9mth old.

I know this isn't always the case and should you decide not to terminate you may have a very different experience. However, things can turn out better than expected.

I am sorry that you have had this news about your baby and my thoughts are with you as you and your family make this difficult decision. xxxx

Thanks everyone for your stories and support. We've decided to have a pizza and go to bed, hoping that sleeping on it will give us some perspective. Feeling completely exhausted!

Thanks again - will post back tomorrow

littlemy x

hi littlemy hope you enjoyed the pizza

I have looked after many children having open heart surgery and on the whole they do very well. Infants and children recover much more quickly than adults do from things as they do not have the idea that they are ill iyswim. tbh I think it is more traumatic for the parents to see the babies like that than it is for the babies. We make sure that they are given pain killers and they are often sedated after the surgery but if all goes well they start to feed quickly and get back to normal quickly. With HLHS as they said there is a high death rate for the intial surgery and you do have to think that they will have 2 more ops but the outcomes from surgery are much much better than they were and because research is being done all the time and treatments improving then we don't know what the next 5 years might bring in terms of developments.
I am not saying this to try and persuade you to go ahead with the pregnancy as the flip side of all of this is that the baby may die before the first op after the first op or subsequent to that. They may also have a long recovery period as there is always the risk of complications.
You need to consider the effect of having a very very sick sibling on your other child and I know this is something you have thought about.

All I can say again is that you need to make the decision that is right for you not for anyone else. I honestly don't know what I would do if I were in your shoes and I have worked in the area for 16 years and am now very senior. Please contact the hospital again and ask to speak to a surgeon who does HLHS surgery (although bear in mind that they often are pro surgery as they do it iyswim) or to speak to a cardiac liaison nurse every children's heart unit in the country has at least one and they will be able to give you up to date info about their unit. The other thing to bear in mind if you choose to carry on with the pregnancy is that not all children's cardiac units do lots of HLHS surgery. Ther are some that do a lot more than others and depending where you live you should ask to go to one that does a lot.

I hope this helps please ask if you need more info.

LittleMy - been thinking of you. Hope you got a decent night.

LittleMy

Been thinking of you. I'm amazed and impressed by all the thoughtful and helpful messages you received on this thread. I know whatever decision you make will be hard. I have no first hand experience of this situation (although I had a friend who had heart ops as a baby and recently one as an adult) but I would only say that whenever I've had to make a tough decision in my life, going with my first instinct has never left me with regret.

I hope you find the answer you're looking for.

Sending hugs

China

LittleMy34, I am so sorry you have to make this decision. You and dh have the strength to do this. You know that whatever you do, it will be the best course for your ds.

My dd was born with a congenital heart condition which was surgically corrected at Guys' Hospital, now the spanking new Evelina Children's Hospital. Although hers was mild (just a big VSD), we did come into contact with many families whose dcs were there for multiple surgeries.

I am sure you would have been given leaflets from about ECHO, the support group for families of heart children treated at Evelina. They are very helpful and can put you in contact with a parent whose child has the same condition, to answer your questions from a holistic, rather than medical, perspective.

Also, if you are wondering about quality of life issues, I know of this other association, Grown Up Congenital Heart Patients AssociationGUCH, which supports young people and adults who have grown up with this condition. Perhaps you can give them a call to see how they can help you.

Let me know if you need anything else.

I worked in operating theatres until having dd as a scrub nurse. I can only echo what andiemustlosehalfastonemore has said. That you have to make the right decision for your family, but that children bounce back and are far more resilient than adults having similar surgery. They don't have the fear because they don't understand enough to fear the surgery. Parents have fear for them. Also, they don't know they are very ill iyswim and have no preconcieved ideas about how they should be so as soon as physically able they bounce back. At such a young age I have not encountered anyone who can remember being operated on, including myself at age 12 years. The anaesthetic drugs have sedative and amnesic effects (make you forget).
Good luck in whatever you decide x

Thank you all for your continued support and really useful information. Have been wading through more information today and spoken to a lovely lady at ARC (Antenatal Results and something or other) who was very helpful in listening to my stupidest thoughts.

I have some more questions for the cardiologist who did the scan, so am waiting for her to call me. Looking back on it, she was quite brusque with us at the time and i think we need more information than we got then. Having said that, there's only so much you can take in at that particular moment!

One thing the lady at ARC said that stands out was that sometimes it's impossible to make a decision until enough time has passed to take it all in and also to come to terms with what your gut instinct is telling you. That was really helpful, I now feel we can take some time without having made a decision just to let it all sink in. And as neither option is very attractive, I think what she said about needing time to come to terms with the decision is spot on.

So that's what we're trying to do! Work have been lovely and told me to take as much time as I need, which is good. It's DH's birthday on Thursday, not a good time for him but I'm determined to bake him a cake at least so I can do that tomorrow.

Thanks again all - we'll get to a decision and I know we'll learn to live with whatever it is in the long run. It's just going to take a bit of time.

LittleMy - I am so impressed with the amount of knowledge and support on this thread. There is so much help out there to support you into coming to the right decision for you.
I also just wanted to thank blueshoes for posting the link to GUCH, which DH hadn't known about and which I think will be very helpful for him. He has had a lot of conflicting information from different consultants about his condition over the years and sometimes it can be hard to find out what the most up-to-date thinking is, but Mumsnetters as usual are up to speed with everything!

littlemy I think I can guess where you had your scan hope dhs birthday is ok and I will be thinking about you and checking the thread
if you need anymore info please post

Do you know Bristol then andie? If you do there's only really one option in terms of where it was - sounds like you have personal experience of the people there.....

LittleMy - glad that you have had a chat with someone today. Enjoy baking and eating your cake.

You asked before about how siblings managed. I just wanted to ask if you have a good family network? My parents were extremely helpful when dd was in hospital for long periods. They looked after my other children at our home, kept them in a routine and generally smoothed over the cracks. Kept me sane TBH!

I hope the cardiologist can give you a clearer picture when she calls back. She can probably send on your scan tape for another opinion if you are not completely happy with her. I have friends whose children are looked after at Guys and are more than happy. We are looked after at Birmingham and the team there are very experienced with HLHS.

Take care and take the time to come to a decision that's right for you.

Thanks Saggarmakers - we only have my mum in Bristol - she looks after DS1 for half a day a week but she's nearly 70 so can't do much more, and she's a psychotherapist so has clients most days, which means it's tricky. Three sisters, but they're all in different parts of the country and with children of their own, so a bit tricky for them to be much help.

Have got some lovely friends though, including a few with children the same age and some without kids but who love DS1 to bits, so I'm sure we'll muddle through no matter what happens.

no littlemy was thinking of london as I know 2 of the cons are very brusque what does that say about their bedside manner they must all be brusque!

Has to be said, they could do with some bedside manner training! Our antenatal consultant is lovely though, really sympathetic and able to listen, such a rare quality in a consultant....

I know they do amazing work, but I think sometimes they get so wrapped up in the procedures and the statistics it's hard to remember it's a baby's life involved.

Andie, I would say they are all like that - our consultant (Alder Hey) is a classic example. When dh last took ds1 for a check up he overheard one of the nurses say to another (in reference to our lovely consultant), "Oh look, here comes God..." It made dh .

Littlemy, was just coming back to this thread to see how you are doing. You have some excellent advice here. DH and I were discussing it earlier and he said that he agrees with what andie said further down the thread wrt finding out about your unit's record for HLHS surgery.

LittleMy - I do have a link to a site which has unit stats - probably andie knows which one I mean. I will post it if you'd like. Or not. Just say. Stats like these are difficult to inteprete though in isolation and are really not a like for like comparison. For example a unit which has a poor mortality rate isn't necessarily one to avoid - it may be that it takes the severe cases that other units feel unable to manage.

Re consultants - most call a spade a spade I find and aren't in the business of giving false hope. Often it can be interpreted as being unsympathetic or harsh. Some do just think they're God though

Apparently we would have to go to Guy's as the Bristol Children's Hospital doesn't have much experience of HLHS - I looked up Guy's stats already and they're pretty good, but at the moment it's less about survival rates and risks etc, and more about whether we can decide to put our child through all that, and whether even the best possible outcome is worth the pain and trauma that getting there would take.

sorry, not nice things to have to think about

I know I'm sorry that you even have to be thinking about it LittleMy.

Be kind to yourself.

Littlemy - just wanted you to know I will be praying for you and your dh as you face this terribly hard situation.

littlemy my friend was at Guy's with her ds - she was at first a bit disappointed that they hadn't gone to GOSH (there was no bed available) but then realised how good the team is at Guy's, the surgeons did everything they could and they couldn't speak highly enough of the whole team there, x

littlemy guys is definitely one of the places to go I know the senior surgeon there very well and he is excellent brusque but very very good they have a well developed hlhs service and good results
but as you said this is about whether you want to make that decision or not so I think I will stop posting about surgery unless you ask and leave you to make up your mind about the way forward for your family

I will be thinking about you over the next few days if you need anymore info just ask I know how difficult this must be for you but we are always here to talk to through cyberspace and lots of us are thinking of you

Thanks andie, and northernlurker and saggarmakers, and bundle. We both feel a bit calmer than we were - the shock seems to have worn off, but we're still not sure of a decision. I'm leaning towards termination (sorry if I upset anyone with this, I know it's a difficult subject), but although DH is also leaning that way, I think he's worried that he only wants that option because he's scared of how hard it would be if we went ahead.

and I'm worried that he might make a decision based on what I want, rather than what he wants......

Littlemy, I was so sorry to hear about your scan. I've worked in Paeds ITU at Guy's and have spent a lot of time with parents who have been in the same position as you but chosen to carry on with the pregnancy. All I can say is that there is no right decision. The surgeries are stressful for all the family and you have to think of not just your unborn baby but your other child and yourselves too. Outcomes are very variable too.

I am not anti-termination at all and HLHS is one of the conditions I would seriously consider it for. I don't think that until you have been in the really difficult position you find yourselves in can you really judge what you would (or should) do.

Good luck. I am sure that you will make the right decision. I will be thinking of you. xx