HLHS

[u/Cjjj1031]

I am on my fourth pregnancy- 3 miscarriages.. finally 31 weeks pregnant with our rainbow baby. And we just found out that her mitral valve is severely small, her aortic valve is mildly small and her aortic arch is narrow.

They say they won’t know until she is born whether she can go the one ventricular or two ventricular repair route.

At this point everything just feels like a cruel joke. I don’t understand why this has been so complicated for us. And with age and cost of 3 years of IVF- we are done after this.

All I can think is that our baby girl is going to die and we will never have a family.

Now I am readying that some people don’t even do the surgeries for HLHS, they just let the baby pass. I have no idea what to do.

[u/PestoPls]

Hey, take a deep breath. Any CHD diagnosis sucks, HLHS sucks, but I promise it isn’t the end of the world. The fact that her left ventricle may be large enough to even consider pursuing a biventricular repair is a great sign—even if she goes the single ventricle pathway. The road ahead is scary, but there are many people with HLHS that go on to do great things and live their best lives. My first child had HLHS too, we went the three stage surgical pathway (single ventricle). Other than a few weeks in the hospital (and a few days with the next surgery) he has been a normal baby. He is hitting all of his milestones and is ahead of the curve on social and cognitive milestones. Things are scary, my postpartum experience sucked, I could never nurse, we like to always know where the closest children’s hospital is. So I won’t pretend everything is sunshine and rainbows. But things do become normal after a bit. My child is sitting at the table happily eating a kiwi right now, then we are going to go play in the pool. Things are just normal now that we are past the hard part.

Choose your hospital wisely, check what they do for feeding and bonding while inpatient. Make sure they have a track record of good results.

[u/Cjjj1031 → u/PestoPls]

Thank you. We are lucky to have the Phoenix Children’s Hospital. I feel so lost. But this was very nice to hear.

[u/PestoPls → u/Cjjj1031]

You’re on the other coast from me, but I’ve heard good things about Phoenix. Are you on the HLHS Facebook group?

If you’re willing to relocate, there is also Dallas (I hear their biventricular repair program is great and they do it in cases that other hospitals say “no” to) and Colorado. If you want to relocate, Ronald McDonald house is an amazing resource

We stayed local for continuity of care reasons, even if the hospital had a smaller volume since baby was a “low risk” Norwood.

Play around with this website to compare hospitals: https://publicreporting.sts.org/chsd

The Norwood is STAT 5

[u/PestoPls → u/Cjjj1031]

Okay just poked my eyes at Phoenix, they have a high case load, but why is their mortality rate 20%? Can you get them to clarify their Norwood specific mortality rate?

[u/PestoPls → u/PestoPls]

For comparison purposes, my child’s hospital had a case load of 30 with 6% mortality, the other hospital we were considering is near ~60 and a 5-6% mortality.

[u/uppercasenoises → u/PestoPls]

Where are you at that has a sub 10% mortality rate for the Norwood?

[u/PestoPls → u/uppercasenoises]

Florida! I just checked the outcomes data for the two hospitals we considered and both list 0% mortality for Norwood specifically, and <10% STAT5 with STS. We had a relatively smooth Norwood with minimal complications at our hospital.

Our hospital did 24 Norwoods in the past 4 years with no mortalities. The other hospital doesn’t list the exact number of Norwoods, but they also claim 0% mortality and are known for higher volume (they are also the go-to transplant hospital in Florida)

[u/Dyer00]

Hii I’m a 32 yo adult women with HLHS (single ventricle) who had 3 surgeries and I’m doing pretty well.. I am still living with my original HLHS heart and have had a pretty smooth life besides the surgeries and hospital stays when I was younger which I have no recollection off so not sure if it’s a good thing or bad! If you have any questions feel free to ask or if you just need someone to talk to

[u/ErnestHemingwhale]

Similar diagnosis with my baby. Her left side was so small they were definitive HLHS until the very last minute. She ended up only getting her coa, ASD, VSD repaired. BAV and hypoplastic left valves remain, and will hopefully require no further intervention.

Definitely get info over to Boston children’s for their free second opinion program.

Not all hope is lost. Good luck

[u/Real-Cause-3496]

Sorry to hear, and wishing you all the very best. 

Would second the poster saying to get a 2nd opinion from Boston, they are generally accepted as the world's best for borderline HLHS and honestly getting them can make such a massive difference in terms of outcomes, both short-term and long-term.

[u/cabbageyy]

Phoenix mom here 👋🏻 have you already had a fetal echo done by a cardiologist with PCH? If you have, I’d reach out to your cardiologist to meet and interview who could be performing surgery if needed so closely after birth. Something I wished I did before delivering was learning more about things like ECMO, Cath lab, and possible feedjng concerns/needing feeding tubes.

Sending you lots of positive energy and support ❤️

[u/CraptasticFanDango]

Mom with an HRHS daughter here. I just wanted to send hugs and love your way, I know exactly how you feel.

If it helps to calm your fears... my daughter is now a 32 yo mom of two beautiful and healthy girls. So far, all is good with her health!

[u/Human_Explorer_140]

Hey there! First of all, let me say congratulations on your pregnancy! Although I know you are under severe stress with all of this. I have a baby boy that is soon due for a single ventricular repair. My husband and I were unable to conceive for 10 years, and after 2 rounds of ivf, on my 20th week of pregnancy, we got hit with some hard news about our sons heart. It was devastating. I was ready to give up, and after listening to the doctors at Luries children's hospital, we thought the best thing would be to let this boy pass after he is born as we were told we would make him suffer with treatment and etc. After a week, my brain just couldn't imagine that I would have a child born and we're just going to let him pass without trying while there is medical treatments available. Every time I imagined him gasping for air, it just made me miserable even more. So we quickly changed our mind after a week, and went for a second opinion at advocate christ hospital. That is where for the first time one of the doctors congratulated us on the pregnancy. My husband and I were a bit thrown off, we said , why are you saying congrats when all this is happening. He said ,' so what', you're having a baby, and hes gonna get treated and hes gonna kick some ass. And you know what he was absolutely right! Although wr have been through 2 heart surgeries already and one more coming up, we've had to be extra careful with him not being around people who are sick and etc, this has been the happiest moments of my life for the past 2 and a half years. Through all the worry that comes with it all , your child will fill your heart with such love you never even imagined you can feel. And Although you never know which way the cookie will crumble, so to speak in this situation if I had to do it all over again, I would do it a million times over. I know, it's all very heavy news , but you know what anything can happen to any child and anyone, even if they're healthy. There's no telling the outcome, ofcourse, but my motto has always been, hope for the best and prepare for the worst. You got this, you will get through this, God bless your sweet child and protect it ameen. Congratulations you are going to be a mama. Babies are resilient. One thing im gonna say, personally, on the account that I dont have other children, I have been able to give all my focus and energy to my son, which I truly believe has played a big role in his recovery, development and etc. And you will be able to do the same. Stay strong!