Hypo Plastic Left Heart

[cloudedyellow]

My ds and ddil found out at the 20 week scan that their second baby has HPHS, a rare congenital heart syndrome for which there is no cure.
This is devastatingly sad news for all the family, but particularly for the poor parents who will have to make unbearable choices about their baby.
Added to which they are living in NZ at present and so far from the comfort of wider family support.
I am so concerned for them and my 4 yr old dgs.
Does anyone have any knowledge of this syndrome? I would be so grateful experienceany information or personal

I mean 'grateful for any information or personal experience'.

hi I was pregnant with a baby who had hypoplastic right heart which is the same but it is the right heart which is too small. the baby also had transposed great vessels and an enlarged pulmonary vein and constricted aorta. I was told that with a single ventricle heart any surgery and treatment would be palliative and there is no cure. There is surgery call norton (I think). Three seperate open heart surgery 0ne in the first week and then I believe at a year and another by 4. Chances of getting to 5 about 50%. Often the babies have other complications such as bronchitis and lungs become damaged. During their teens the hearts tend to fail (and sometimes lungs) and they require heart and lung transplants. I was told that the baby would be put at the bottom of the list as they are not considered good transplant risks. Those at the top when I was in this position (2007 in the uk) would often die before they get a new heart/lungs so those at the bottom (i reasoned) would be extremely unlikely to get a transplant.

WE looked at this website www.lhm.org.uk/ which was fantastic at giving us an idea of quality of life. at that point the family stories were open to the public and frankly, the children spent a lot of time in hospital and often had feeding tubes. It will not surprise you to learn that we chose a termination which I felt was the best option for my littel girl and my family. I am pro-choice and I have never regretted my decision and still believe it was the best option - so I may be giving a negative point of view on here. The other option i would have considered would be comfort care. This involves having the baby and letting it die naturally in the first day or week.

It is a stonger person than me who could deal with the trauma of having a baby with a single ventricle heart. However it may be better that they are in nz. in the us they do a transplant on birth and they have far better outcomes. If we lived thre I may have considered haivng my baby.

I am sorry to be so frank and so negative but I hope it helps you. I will try and come back on later and see if you ahve any questions. I hope it is not too distressing for you as you are so far away from them. x

norwood fontan procedure
see wikipedia - en.wikipedia.org/wiki/Hypoplastic_left_heart_syndrome

I was initially seen by john simpson at st thomas evelina and I had read papers by him before seeing him. He believes in not sugar-coating everything and I really felt he gave extremely good and honest advice. I then (for various pct reasons) had to be seen by someone at queen charlottes. She was more positive (life span into their twenties until needing transplant etc) she didn't fill me with confidence. However transplant and treatment could have got a lot better in 5 years. and obviously wikipedia issn't nec always right...

Thank , thank you, Manitz! I'm so sorry you had such a painful experience. It is very helpful to hear what you say.
I would've almost certainly gone for a termination myself as I don't think I would've coped with all the hospitalisation and risk and stress for the baby/child, but the parents may come to a different decision and I have to prepare for that.
It does seem that the States are much more proactive on the surgery front than the UK and NZ may take it's lead from them. So far my family have been offered all three options.....poor, poor things. Who can choose?
It is true that techniques could have improved and probably will go on improving...but..but...
Interesting to hear that a baby would be bottom of the list for a transplant - although maybe not in the states.
You don't say whether you have another child or children. If you do I hope all is well with them.
I really appreciate you replying and for what it is worth I too think it was the right decision.

hi clouded yellow glad it was of use, was not a very balanced view and meant to come back and say so. sorry.

your db and dsis are in between a rock and a hard place, you can only be there for them whatever they decide. During our decision making process I knitted and watched box sets (the wire) and cried - I didn't really ahve the energy to talk to people but appreciated texts/emails from people asking if i was ok to which i could respond if i wanted. After the termination (which was a birth at 26 weeks) I didn't have the energy except for my kids and husband. i felt an immense loss and I needed to shut down to repair myself. It took about 6 months for me to feel ok and each day was better than the last. I was pregnant by that point and that really helped me feel better.

It depends what your db and sil are like but I wanted people to acknowledge the existenc eof my child and not just the sad bit. I wanted people to ask about my labour and to deconstruct it as with my other births. I really appreciated having flowers from my cousin and visits as with my live children. I think it's a lonely place to be when you come home from hospital without a child and you think how different from the happy ocassion it could have been.

I have four living kids - although my 8 month old is trying to pull the ironing board down onto him as I write! So I don't feel at all hard done by, I have never been happier or tireder.

btw to clarify - when I said the baby would be bottom of the list that was because by then they would have been in their teens having been pretty ill all their lives and lots of operations with norwood and fontans etc so would not be a good candidate compared to someone who has a more immediate heart problem who may have been otherwise well and might be a better candidate.

Hope that makes sense. I think the us version of operating on a baby would mean those babies would be top of the list.

I don't think there's such a thing as a balanced view!
Pleased to know you have other healthy children. I feel concern for my grandson if the family are dealing with a sick baby/child. With the best will in the world, he is bound to be affected and sometimes neglected perhaps.
If they choose a termination (think this is unlikely) I will bear in mind what you say about needing people to acknowledge the loss of the whole child.
I'm really grateful for your posts. Thank you again.

sorry, I thought it was your brother, misread. if it's your son I can see that you must feel quite helpless as you are so far away. If you think they will continue the pregnancy I would definitely suggest you contact LHM as there will probably be parents and grandparents in similar situations to you.

i had a sister who died in childhood after an illness which meant my parents were very preoccupied with her health. It did inform my view but I would say that if it's handled carefully your grandson will be fine and will (sort of) benefit in some ways with the right support.

I hope they are able to make a decision they are comfortable with and that you are all ok over the coming months. x

Hi,

Hi. My daughter was diagnosed with complex congenital heart defects at her 20 week scan. She also had a undersized right ventricle with transposition of the great arteries, plus an undeveloped pulmonary artery, double outlet right ventricle, a VSD and additionally a 'criss cross' heart. It's a pretty unique set of defects but still essentially a single ventricle condition.

We were given the same options as Manitz, i.e the 3 stage repair, which would take 3 years to complete and would hopefully see her through to 10 but she'd then have heart failure in her teens and we could expect to lose her at that point. But that was the best case scenario. We could have lost her at any point, the consultant described her as a 'time bomb'. Heart transplant wasn't really mentioned to us, I think for the reasons Manitz said.

We made the devastating decision to terminate my pregnancy. I didn't think it was fair on my beautiful girl to live a short, restricted life or for her sister to lose her little sibling. This was 3 weeks ago and we are absolutely devastated. It's the ultimate lose lose situation, I'm sad to say. In my head I know I made the right decision for my little one but my heart aches for her.

I'm sorry this isn't a more balanced view; there are lots of children out there doing well with single ventricle circulations and hopefully there will be someone along shortly who'll be able to share a more positive experience. I suppose though the cruel fact is that it's a life limiting condition.

Please do call and speak to www.lhm.org.uk/ They were an amazing source of support and information for me. This is so tough for you as a mother and a grandma. My husband is from NZ and I know how hard the distance is.

Thinking of you all.

hi prettyfly. I'm so sorry to hear about your situation. It must all be so raw for you. I hope you are bearing up. x

Hi prettyfly, Thank you so much for responding.
Only three weeks ago? How kind of you to post when you must be feeling so sad and bereft.
As I said to Manitz, I believe you made the right decision, but everyone is different and will make different choices.
Thank you both for the lhm link. It looks very helpful.
Yes, NZ is an added complication, but the other Granny is travelling out at the end of August so that is some comfort.
Best wishes to you and your family. Thanks again.

There is a docu about this on BBC2 right now. It's part of a series about Great Ormond Street and this week it's about ccongenital heart defects. They are currently showing an op on a 24 hour old baby with a single chamber heart. It should be on iPlayer for the next couple of weeks, there is a lot of info about the choices the parents face.

You're welcome cloudedyellow. These conditions are so rare that I felt I should share my story, I just wish it was more positive. We were able to donate my baby's heart and I hope that this will make a tiny difference to other children in the future. What I didn't make clear in my previous post was that the doctors had never seen a heart like my daughter's before so although the single ventricle surgery was offered, the outcome would have been more uncertain than a 'straightforward' HLHS patient. I'm shaking my head now at the irony of writing the word 'straightforward' in relation to any complex congenital heart defect...I hope you see what I mean.

I couldn't bear to watch the Great Ormond Street programme. But I imagine it would have been very informative. My NZ relatives also found this website very useful: www.heartnz.org.nz/

All the best x

How typical, I missed the programme! Hopefully will pick it up on iplayer.
Thank you all for your help and support.
Prettyfly - it does sound as though your daughter had a very complicated heart defect (horrible word). Thanks for the NZ link too.
Manitz, thankyou for your helpful words about your sister.
Off now, but I expect I'll be back!

Dear clouded yellow. I am so so sorry to hear about the result of your son and daughter in law's 20 week scan. My baby was diagnosed with the same condition last August during the 20 week scan as well. It was a really really hard time as until then I thought I had the perfect pregnancy (no sickness, no real tiredness, etc). It was my first pregnancy and the first and only grand child expected in the family (both DH and I don't have any siblings). We live in London and both our families live abroad. Fortunately, our home town isn't as far as NZ so my parents came over to be with us during this difficult period.

As Manitz and Prettyflyforawifi, we also chose to terminate the pregnancy and although it was a horrible time, I never regretted our decision. I was first given a pill to stop the pregnancy hormones and then had an amnio to have a genetic testing (which came out all normal). Two days later I was admitted to hospital for the delivery. In my case it was 7 hours of labour and although it was quite painful, the midwifes have been very very supportive. We asked for a full post-mortem which confirmed the diagnosis.

All the doctors (private and NHS) that I have spoken to afterwards told me that it was a very unlucky event and the recurrence chance was very low. There are a lot of women who had health babies following HLHS. Unfortunately my bad luck has continued as my next pregnancy ended up in an early miscarriage and my current one is diagnosed with T21, so I will have another termination (albeit an earlier one) in the next few days. I am still very hopeful that I will have a health baby at some point in the future. I am sorry I didn't have a very positive answer but wanted to share my personal experience.

Please feel free to ask any questions you might have. I know it is not an easy time and being far makes it even more difficult (as I can see how my mom feels at the moment). Good luck and remember no decision in such a situation is a bad decision. Best

prettyflyforawifi, my baby also had a complex set of problems with her heart in addition to the hrhs which they hadn't seen before. Maybe once a heart starts to develop wrong all sorts of things happen and maybe there are few 'straightforward' single ventricle hearts. I was told similar to daphne, just one of those things.

Manitz that's interesting to know. I am so sorry you lost your baby. I got the impression that my baby's heart was so complex that it was difficult to predict how she would do whereas for the more 'straighforward' single ventricle cases they could predict more confidently. But also I might have heard/understood that wrong. There was so much to take in at the time.

Daphne my heart breaks for you. That's just terrible. You are very much in my thoughts.

Daphne I'm so sorry. How awful for you.
Need to get to bed now, but will write more tomorrow. Thank you so much for posting. It is enormously helpful to hear your experience.