… but there is still a lot to be done

In my last post, I wrote about the amazing advances in Congenital Cardiac Surgery and how Heart Defect mortality rates have dropped 38% from 1979 to 1997. But there is still one defect we don’t have a good answer for yet: Hypoplastic Left Heart Syndrome, or HLHS.

Hypoplastic Left Heart Syndrome is not a singular defect, and could have any number of variations. But all of them feature a small (or nonexistent) Left Ventricle and a small Ascending Aorta.

If you look at a cutaway view of a normal heart, you will notice right away that while they are roughly the same size, the Right Ventricle has a larger volume than the Left Ventricle. The Right Ventricle has a smaller pumping muscle: a larger one isn’t necessary because the Right Ventricle only pumps blood to the lungs and back. But the Left Ventricle features a large, thick pumping muscle. When it contracts, the blood is really going places: out into the Aorta, and from there all over the body.

So if the Right Ventricle is content to just drive around the block, the Left Ventricle is at the airport boarding a flight to London. But in a heart with HLHS, the left Ventricle and its pumping muscle are tiny and the Aorta is barely functional. After all, the  root word for hypoplastic means “underdeveloped”. Because of this, a right sided heart defect (like Tricuspid Atresia, which is what I have) is more survivable than a left sided heart defect. 95% of children with HLHS who receive no treatment die within one week.

Even with surgery,  in the mid 1980’s only 28% of HLHS patients survived. (See the 5th Paragraph of the above link.) Until the late 1980’s an HLHS repair involved only two surgeries – The Children’s Hospital of Philadelphia (CHOP) didn’t begin to use the intermediate operation until 1989. (The entire link is informative, but page down to the section labeled “Discussion” for a look at how the three surgery procedure developed.) Current survival rates for the three stage surgical procedure are roughly 75%, with almost no data for long term survival.

This is completely unacceptable.

So what can we do about it?

1) Pass the Congenital Heart Futures Act. The Congenital Heart Futures Act, currently under review by two Congressional committees, will authorize more National Institutes of Health funding for Congenital Heart Defect (CHD) Research. Research is already going on – this January 2008 report from the National Institutes of Health (NIH) states that families with a Bicuspid Aortic Valve in their medical background are more likely to have an infant born with HLHS – but more funding means more and better tools, and more people trying to find a solution.

The Act will also create a CHD Patient Registry, maintained in one location and accessible to physicians. A properly administered registry will assemble a massive amount of data for study. The Centers for Disease Control will also develop educational programs concerning Congenital Heart Defects and their effects on patients and their families.

2) Identify Major Surgical Hubs. You aren’t going to allow a 200 bed community hospital to attempt the three surgery repair needed for HLHS. And it’s not that they are not careful, caring people… they do not have the experience. Identify the large national centers that perform many difficult medical procedures and create regional pipelines that move patients to these hospitals as quickly as possible.

3) Test new surgical theories. Gone are the days when new surgical procedures were developed through the “try it and see” method. With today’s faster computers, surgery can be simulated. A surgeon can “practice” on a computer before performing the actual operation, which give him the chance to anticipate any problems that may occur. By creating a computer simulation of an HLHS heart (multiple variations of HLHS can be programmed in, as can other defects) and using it to test new surgical theories, surgeons can explore “what if…?” theories without actually harming a patient.

4) Create a HLHS-only Registry. As a subset of the National CHD Registry, create a registry dedicated to gathering data only from patients with Hypoplastic Left Heart Syndrome. Small databases already exist and have been valuable in research: for example, researchers have used a HLHS database to  analyze the surgical approaches to HLHS to determine which ones work better. Research and better surgical procedures reduced HLHS deaths in California nearly 50% between 1990 and 2004. But such studies draw on limited databases for their information. Create a national database, and you open even more avenues for study.

5) Determine if HLHS has a genetic or an environmental origin. As noted in the January 2008 NIH report, families with an occurrence of a certain heart defect are more likely to have HLHS occur in the family later. That points to a genetic cause. But there is also evidence of an environmental factor – a “cluster” of twice as many HLHS cases than would be normally expected in a certain section of  Baltimore, Maryland. We need to devote the time and resources needed to determine what exactly causes HLHS: Is it a genetic predisposition?  Or is the environment the trigger? If it is genetic, can we learn how to prevent it? If it is environmental, what is the cause, and can we eliminate it? Or perhaps certain environmental conditions cause the genetic changes that eventually lead to HLHS.

These are just some of the things that we could do to improve Congenital Heart Defect survivability in general, and HLHS survival in particular. Quite often, we have to “think outside the box” to see the problem from an entirely different angle, and then perhaps we could find the answer.

Because every heart deserves to live a lifetime.

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7 Responses to “… but there is still a lot to be done”

  1. Teresa Prater Says:

    Very good article. My son is an 18 year old HLHS survivor, he had the Norwood on Mother’s Day May 13, 1990.

  2. Lisa Says:


    I think about HLHS often.
    Even though both of my boys were labeled as HRHS, Eli’s L-TGA makes me feel more unsure about his future due to his left-sided ventricle making those London trips with the structure of the neighborhood cruiser right vent!

    No one ever said that CHD is easy…

  3. Bernie Says:

    I get Google Alerts for HLHS and am often brought to your blog. I really appreciate your advocating for those with CHDs. Our son, Luke, passed away from HLHS on April 18th of last year. He was diagnosed shortly after birth and underwent the Norwood at 7 days old. His passing was sudden and quite scary, and I have met many other HLHS families who have endured the same tragedy. Thank you for fighting for those with CHDs and for proving that those with CHDs can grow to live full lives. I also wanted to let you know that I have used the same analogy when asked about HLHS vs. HRHS and why the children with HRHS seem to do much better than HLHS kids. I think it is a very fitting analogy. Thank you, once again, for your thoughts. You are doing great things for those with CHDs.

  4. Laurel Nelson Says:

    What I find interesting about HLHS (and my daughter does not have it, she has the right sided defects) is that HLHS is MORE common than the HRHS ones, yet it is way more severe. I find that interesting because usually in things medically related you would expect that something that crops up more often to be less severe than something that is rarer.

  5. Nakia Lay Says:

    Thank you for your posting. My son is 3 1/2 and has HLHS and has also had all 3 surgeries. He is doing relativly well. With no major problems. Its people like you who educate those less informed to help spread the word that so many need to hear. Thank you so much.


  6. Kindra Says:

    I will pray for the children who have lost their battle,along with the ones who are still fighting it. I am 22 weeks pregnant and my baby has just been diagnosed with HLHS. It is websites like these that are preparing me for what myself and my son will go through. I believe in God,and that we aren’t given more than we can handle-so no matter what the outcome I plan to bring awareness to the people in my community of HLHS. Thankyou to everyone else who has told bits and pieces of their stories….it does bring hope!

  7. Anna Jaworski Says:

    Steve, another great post! My son was born with HLHS in 1994 and is 14 years old. I agree with you about the need for more information. Fourteen years ago, there was almost nothing. “The Heart of a Child” had stories, but the HLHS infants all died in infancy. The AHA had a small booklet about heart defects available for parents but its reference to HLHS said all of the babies died young. There was nothing hopeful out there for parents. That’s why I self-published “Hypoplastic Left Heart Syndrome: A Handbook for Parents.” I knew there were some successful stories and I wanted my son’s story to be one of the stories to inspire others. I’m getting ready tomorrow to send “The Heart of a Father” to the printer — over half of the stories are by men whose lives have been affected by HLHS. One of your readers wrote about how prevalent HLHS is. She’s right. It’s far too prevalent (4th most common heart defect) for us to ignore it any longer. We need to do something about it. The Congenital Heart Futures Act is a great beginning. Thanks for being such a strong advocate for this important piece of legislation.

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