HLHS Advice

Hypoplastic Left Heart Syndrome (HLHS) is a known killer. It is 100% fatal if left untreated, and before 1981 there was no treatment. For several years after that there were very few survivors as doctors learned that a surgical correction could not be done in one step, but rather took two (and eventually three) separate operations. Infant Heart Transplantation was a dream until 1984.

So doctors have only been able to combat HLHS for 30 years, and there is still a lot that is unknown. The hows and the whys of the defect are still only being guessed at. The operations are delicate: not only are they demanding on a newborn’s Circulatory System, but so much depends on blood flow and pressures in the heart. All of the blood now flows through the Tricuspid Valve, which wasn’t designed to move that much blood. The proper “balance” often depends on the patient and there is no “standard” series of operations for HLHS. Each one must be customized to the individual.

Which leads to this interesting report from the March 2010 issue of the  medical journal Pediatrics. 749 Pediatric Cardiologists were surveyed concerning their management recommendations when giving a diagnosis of Hypoplastic Left Heart Syndrome. 99.7% of all Pediatric Cardiologists recommended the three surgery approach. 67% of the doctors discussed a heart transplant with the new parents, and 62.2% discussed Compassionate Care without a surgical intervention. (The high numbers in each category shows that the doctors were not limited to one response.) However, only a small percentage – 14.9% discussed all three options.

Also, the doctors were asked what they would recommend when confronted with a prenatal diagnosis of HLHS. Almost all of them (98.8%) recommended continuation of the pregnancy with surgery after birth. 53.5% argued for continuation of the pregnancy with Compassionate Care/no surgery; and a very high 74.3% discussed termination of the pregnancy. Only 36.5% discussed all three options.

Interesting numbers, and much to think about. The bottom line is that most Cardiologists would tell you to fight for your child. But why would so many of them recommend ending the pregnancy when HLHS is diagnosed prenatally?

UPDATE: A reader has brought to my attention (See comments) that the research report states that the Cardiologists/Surgeons discuss these options rather than recommend them. A discussion, of course, is not a recommendation. Some patients may treat it as such (“The doctor will probably know best…”) but it does answer my closing question much better – and reflect the experiences of my readers who have chosen to comment.

I have stated before that I am not immune to mistakes, that is why I try to include a link to anything that is not in my personal experience – so you can check my work for yourself.

And I would like to publicly thank the commenter for bringing this error to my attention.

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13 Responses to “HLHS Advice”

  1. Margareta Barrs Says:

    This is really interesting, I have a HLHS son called Kian.

    I found out he was HLHS in-utero and the doctor told me about all 3 options but also stressed that the operations aren’t a cure but are classed as ‘palliative’ – meaning that they give the child time before transplantation is needed.
    We were told about transplant for babies but they only recommended them if the child wouldn’t be able to get through the first stage of the op (Norwood) as there aren’t many baby hearts and more than likely the child would probably die before they get the much-needed transplant.

  2. McCanless & Mary Clare Pennington Says:

    […] reading from Funky Heart about a prenatal diagnosis of […]

  3. McCanless & Mary Clare Pennington Says:

    […] reading from Funky Heart about a prenatal diagnosis of […]

  4. Kerri Pennington Says:

    I linked to you on this one…interesting! Thanks!

  5. Teresa Okeson-Prater Says:

    My son was born in 1990 with HLHS, we were fortunate they caught it hours after birth and were able to transport him to a facility that treated HLHS, Minneapolis Children’s Medical Center. Our doctor there gave us all 3 options. My decision was based on my son’s blood type A- and the fact that the Univ of MN at the time was involved in a scandal with their heart transplantation anti-rejection drugs. I chose the 3 stage surgery. It has always been understood by me that it was not a permanent fix, but a method to hold him until a better solution could be developed. Look at how far we have come in 20 years, imagine how far we will come in another 20 years.

  6. Shannon Carter Says:

    Very interesting post. We were given all three options when Derrick was diagnosed in-utero, but only really discussed one because to us, there was only one option. It surprises me that so many cardiologists recommended terminating the pregnancy. That seems almost hypocritical…isn’t their job to take care of people with heart defects?

    It’s amazing how far they’ve come…Here’s to hoping they make even bigger gains in the next 20 years!

  7. Kathryn Bonnett Says:

    Our youngest son, Seth, was diagnosed in utero with Hypoplastic Left Heart Syndrome. I don’t recall the cardiologists ever suggesting termnating pregnancy but I know the maternal/fetal specialist did.

    Our cardiologists mentioned all 3 options, compassionate care, transpant, 3 staged surgeries. Compassionate care was never an option. Transplant would have required a trip several states away (and we have 3 older kids at home). We opted for the surgeries. However, and I don’t know if this was ME or what happened, I always felt a little like the message I recieved was “This is very serious but we’ll do these 3 surgeries and he’ll be just fine.”

    Sadly, a week after the Glenn, while still intubated, Seth coded and suffered severe neurological damage. Ultimately we did end up choosing comfort care at THAT point. And we kept Seth as comfortable as we could for his remaining 10 days…

    Interesting post, thank you for sharing.

  8. Susan Dones Says:

    My son was born in 93 with HLHS, diagnosed after birth. He had dones so well no one dreamed he had a CHD. The doctor discussed all three treatments, we were also told that his heart was so bad that the 3 phase surgery would only be short term and he would have to have a transplant to survive and that he may not be a candidate because of his severity. We chose compassionate because then they knew very little and he was so bad. They barely got him stable for transfer to the NICU. Despite wanting him to live, I couldn’t ask him to go through something I wouldn’t choose for myself, knowing his condition. I have never regretted my decision, just that it had to be made. I am so glad they’re learning so much, especially prenatal, which may be the biggest help for our special babies.

  9. Sugar Says:

    Read the abstract of the article referenced above. The cardiologists and cardiac surgeons surveyed did NOT recommend termination. The number reported is how many discussed it, in the context of discussing all the options. The summary presented here is a misreading of the actual survey results.

    • Steve Says:

      You are correct, it was a very bad choice of words on my part.

      I’m not immune to mistakes, which is a big reason why I always try to have a link to the original source material. Thank you for catching that!

  10. heather Says:

    my son asher was diagnosed at 8 days, and at first i was told that he was going to die and i needed to say my good-byes (he was in multi-organ shut-down and almost dead at the time). then doc talked to sick kids and they would “see if they could try,” because of the severity of his HLHS and of his current condition. when we were sent to toronto that night, we were given 4 options:
    1) Norwood-Glenn-Fontan,
    2) hybrid-Norwood/Glenn-Fontan
    3) transplant,
    4) comfort care.
    i refused to even consider comfort care, and transplant seemed to risky at the time. in the end, we went with the 2nd option, and i’m glad we did. sure, it’s been rough, but asher is now post-hybrid, post-ASD stent, post-Norwood/Glenn, post-Coles, post-Fontan, post-pacemaker… and he’s doing remarkably well.
    but the cardios at sick kids told us that some parents choose CC because it’s such a rough road with HLHS, and i’ll be completely honest here, there are days i think maybe i should have given it more thought. not necessarily chosen it, but at least considered it. asher’s been through more than i like to think about. but he’s happy (other than PTSD now), so i still would have chosen the hybrid. but there are days…

  11. Tom Says:

    Our youngest was also born with HLHS. We did not catch it in-utero. With my history, we tried a few times to get a look at the heart, but she was always turned at an angle that never gave the dr a good view. So we found out 48 hours after birth. This was in 2002. We were given all 3 options. Frankly, I can’t imagine an honest doctor not giving, or at least explaining, all 3 choices, no matter what the doctor’s personal preference is. Anyway, we chose the transplant route because we decided the overall quality of life potential was worth the risk. She has done remarkably well since the transplant although it was a shaky ride up to that day. The amazing thing is that, because of our insurance company fighting with the hospital over specifically heart transplantation, we were sent to St. Louis for the transplant which she got in almost exactly 7 weeks from being listed. We found out a few weeks after we got back to Colorado that if we had stayed here, she probably would not have received a heart in time. So everything worked out very well for her!

  12. mother2 Says:

    I have two lovely boys that born witu HLHS, they got all three stages of the surgery. They are very clever, good pupils and they functions quite well,almost like any ather child in the same age.They drive bike, swimm and almost every thing that I never dreamed they do. All thanks to god.

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