The Dirty Little Secret of HLHS

She didn’t even have a name for it.

When Dr. Helen Taussig wrote Congenital Malformations of the Heart in 1947, she described one malformation as “Atresia or marked hypoplasia of the aortic orifice prevents the expulsion of blood from the left ventricle in the normal manner.” Taussig described several variations of the defect – actually different defects, later grouped together under the same deadly name – but could offer no treatment suggestions. Her Tetralogy of Fallot (ToF) patients could at least squat and get some temporary relief; children cursed with this malady died in less than one week. The left side of the heart wasn’t damaged as much as it just wasn’t there.

It wasn’t until 1958 that Dr. Jacqueline Noonan and Dr. Alexander Nadas named the group of defects Hypoplastic Left Heart Syndrome (HLHS). Hypoplastic comes from the root word hypoplasia, which means “small”.

But having a name for it didn’t make it any less deadly as HLHS continued to claim 100% of its victims. This sad story continued until 1985, when the first successful infant heart transplant took place.

At about the same time the Norwood Procedure was developed. Originally designed as one operation (which consistently failed) the procedure was soon split into two heart operations – and eventually three – which seemed to work. HLHS children now had a chance. Obviously long term survival rates aren’t known yet, but approximately 70% survive the three surgery protocol.

And thats when the dirty little secret of HLHS showed itself: While survival rates for the three operation procedure may be in the 70% range, getting from Stage I to Stage II is the hardest step.

The goal of the Stage I operation is to make the Right Ventricle do the job of the defective Left Ventricle – getting the blood to the body. With all of the blood flowing through the right side of the heart, that side is subject to higher flow pressures than it usually receives. A lot higher. In a defect in which the entire left side of the heart is damaged, a successful outcome usually depends on the Tricuspid Valve – located on the right side of the heart.

The inital results were confusing. The Norwood worked – then it didn’t. There didn’t seem to be any rhyme or reason to it, no way to predict outcomes. The surgery itself worked, but too often the results  seemed to be as random as a roll of the dice. The answer was the Sano Shunt, invented by Japanese surgeon Shunji Sano – a direct connection from the Right Ventricle to the Pulmonary Artery through a Gortex conduit. (this sounds redundant, but the Pulmonary Artery is normally disconnected from the ventricle in the Stage I operation). Studies showed that survival chances with the Modified Norwood was 11 times greater than with the Classic Norwood.

And now it is time to turn the tables – we’ve got a nasty little trick of our own. Heart defects have to start somewhere… at some point in fetal development, there must be one isolated problem that seems to “snowball” into something bigger as time passes. If we could find that one little problem and repair it then, maybe we can stop the snowball before it gets too big. A pretty cool idea, and maybe one day…

Someday…

A study released Monday (September 28, 2009) revealed that for seven years, doctors at several hospitals in Boston and Harvard Medical School have been detecting Aortic Stenosis in fetuses. Fetal Aortic Stenosis is a snowball; it usually becomes HLHS as the fetus develops.

70 future HLHS patients underwent surgery while still in the womb – a needle was inserted into the mother’s abdomen, passed into the fetus, and into the heart of the unborn child. A small balloon was used to enlarge the abnormally small Aortic Valve. 51 of the procedures were considered successful… and 30% (17 children of the 51) were born with two functional ventricles. That’s 17 children who won’t have HLHS.

Mark September 28  on your calendar… that was the day that the CHD world changed forever.

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16 Responses to “The Dirty Little Secret of HLHS”

  1. Andrea Himmelberger Says:

    Wow! Absolutely amazing!

  2. Anon Says:

    Just finished watching series 2, episode 17 of star trek, next gen. Picard has a cardiac replacement as a routine operation. They show us the surgery of tomorow! Quick recovery! Ahh so its possible!

  3. McCanless & Mary Clare Pennington » Post #808 deserves a WOW. Here it is… Says:

    […] to Adventures of a Funky Heart, whose author just so happens to be an adult CHDer living in SC, and […]

  4. Wendy Says:

    Hi Steve

    Check this out – I think it’s similar to the surgeries you were talking about – we followed this story in the local paper.

    http://www.sickkids.ca/AboutSickKids/News-Room/Past-News/2009/first-in-canada.html

    Amazing eh? So much hope for the future

    Wendy

  5. Alli Says:

    Holy Smokes. Thank you so much for publishing this and the links!! My son is 6 months old with HLHS and I have often wondered how things might be different if there was an in utero procedure for just this thing. This is absolutely incredible news and truly made my day!!!

    • Lisa Hatton Says:

      Sadly- I am bitter. I know I shouldn’t be but it hurts so much since our daughter passed away from HLHS on September 28, 2009. She was born on September 25, 2009 & I had asked about in utero procedures. A year later may have made all the difference in the world…

      • BErnadette Says:

        I understand your pain Lisa my precious granddaughter had her 1st surgery 5 days after birth (Children’s Boston)last year Feb 5 she left us 99 days later…

        Still missing her , not angry but sad that the hole left in our lives especially her parents will never be filled… Keep thinking good thoughts

      • Lisa Hatton Says:

        Rereading that I suppose I should have said bittersweet. Jasea has only been gone 3 1/2 months & I have never experienced pain like this. I am happy that there are so many others that will never have to experience the sadness & loss that my husband & I are feeling right now. I am happy that so many children will have a second chance. It truly is amazing.

  6. Laurel Nelson Says:

    Wow! That is amazing! Those families must be so grateful to have dodged a bullet like HLHS before their baby was even born. Amazing.

  7. Shelly Says:

    Wow…that is fantastic! There is so much hope for the children of the future! 🙂

  8. Zoe Says:

    Amazing! When I look at the progress that has been made since my son was born with Shone’s Complex 25 years ago, I am filled with awe, and thankfulness for his presence in our lives.

  9. Suzanne Tarini Says:

    Great news. Two of my son’s cardiologists (he’s a four year old with HLHS) are on that pioneering team at Children’s Hospital Boston. Amazing! What a promise for the future.

  10. angie stuart Says:

    wow!!!!!! my little girl is 13 months. hlhs was the worst news i had ever gotten but shes a huge blessing. im so glad to hear that things will be different for future little ones!!!

  11. Julie Says:

    My daughter just got through the Norwood and the Glenn. She could not leave the hospital until she got the Glenn at 3 months old! I have known many other children with HLHS and they did not survive after the Norwood. It is nice to hear that things will be different in the near future. God Bless the doctors and researchers doing their best!

  12. Jill Gambon Says:

    The In-utero procedure was created by and still is done by my childs cardiologist Dr. Wayne Tworetzky, he is amazing. also Amazing is her surgeon Dr. Emile Bacha he took my daughters hlhs heart and she is now a bi-ventricular repair, thats right i said she has a “normal” circulation. she is dure for a valve replacement next month however she is still doing very well. There is harldy any info becuase this is so knew just know that she was the 26th baby to have the bivent done on an hlhser and that they are working and its yet another chance for our kids. i wanted to add that she did have a left ventricle just very very small a very deformed mitral valve and aortic stenosis. she was a hlhs child but did have some lv. as you know there are many variations of hlhs. (just wanted to state that as ive been told by many that no way could my child be a real hlhser..lol)

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