Posts Tagged ‘Hypoplastic Left Heart Syndrome’

Better Surgery!

June 1, 2009

Here’s an interesting report from the March 2006 issue of Heart: A look at the outcomes of surgical correction for Hypoplastic Left Heart Syndrome, or HLHS. What makes this report so unusual is that it covers twelve years at the same hospital: While surgeons, doctors, and nurses come and go, the hospital policies and standards of care stay in place and hopefully improve. So this can be considered an unbiased study. When results from more than one facility is used, the results can be affected by a hospital that has more (or less) successful results than average.

Beginning in 1992, 333 patients underwent the Stage I surgical correction for HLHS, also known as the Norwood procedure. 203 patients underwent the Stage II operation (the Bidirectional Glenn), and 81 had the Fontan Procedure (Stage III). While HLHS is difficult to overcome, the report does not elaborate on the difference in numbers. It is quite possible that patients had their Stage II or Stage III operations at different facilities.

Over the course of the study, mortality after the Stage I operation dropped from 46% during the first year (1992) to just 16% in the last year (2004). The survival rate jumped dramatically in 2002, when the Stage I was altered: the Modified Blalock-Taussig Shunt that was part of the operation was replaced by a Right Ventricle to Pulmonary Artery Conduit.

Medical research is a long, tedious assignment – there are very few “bolt of lightning” moments and it often requires gathering massive amounts of data. But it works, and is often the only way to learn if a new theory is both effective and safe.

2009 Bolder Boulder Photos (With Song Lyrics!)

May 29, 2009

Hold your mouse pointer over each photo!The ACHAmobile!

On the road again, just can’t wait to get on the road again; The life I love is making music with my friends, and I can’t wait to get on the road again!On the Road Again, Willie Nelson



Shy girl, afraid of the water; now you’re leaving them in your wake. So you fell down seven times but you got up eight!Look at You Now!, Mark O’Shea

The Funky Heart & Stephanie (HLHS)I’m just glad to be here, happy to be alive!End of the Line, The Traveling Wilburys

Get on your feet and get ready to great the 2009 ACHA Boulder Boulder Team!People you’ve got the power over what we do, you can sit around and wait, or you can bring us through. So come along, sing our song, you know that you can’t go wrong.The Load Out, Jackson Browne


Heather (Tricuspid Atresia) and Steve (Tricuspid Atresia)

Look at your life like a diamond, look at you shine from inside out!
Maybe you took some time to find it oh but look at you now!
– Look at You Now!, Mark O’Shea


George - CHD and Cancer Survivor!


Well it’s all right, even if you’re old and gray; yeah it’s all right, you’ve still got something to say. Well it’s all right, remember to live and let live; yeah it’s all right, you’ve still got plenty to give. – End of the Line, The Traveling Wilburys


Homeward Bound

Oh won’t you stay… just a little bit longer?Stay, Maurice Williams and the Zodiacs

No, a lifetime’s not too long…. to live as friends. Friends, Michael W. Smith

… but there is still a lot to be done

April 13, 2009

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.

A possible link

April 1, 2009

UPDATE #1: Shepherd’s blog has been updated: He’s out of surgery and it appears that everything went well! Keep him close in your thoughts as he begins to recover!

UPDATE #2: Anthony is no longer on Dialysis, and he’s a bit irritable with having to stay in the ICU. He’s getting better!

There are two new reports out concerning the relationship between Congenital Heart Defects (CHDs) and environmental factors.  This research from Cincinnati Children’s Hospital Medical Center presented at the American College of Cardiology (ACC) meeting today shows that Hypoplastic Left Heart Syndrome (HLHS) seems to be seasonal – most of the occurrences occur with children born from April to July. January births seem to have a very low incidence of HLHS. The researchers contend that obviously, other factors are at work, and contend that there is a possibility that a woman suffering from Strep Throat during her pregnancy could trigger HLHS.

The possible Strep Throat/HLHS connection is an important find – it is a finalist for the ACC’s Best Poster Awards Competition this week.  This is not the kind of poster competition you probably participated in in grade school. The goal of a scientific poster is to communicate new research quickly, efficiently, and accurately. You have to submit a legitimate research paper with your poster and there are guidelines the poster must conform to.

The second report comes from the Indiana Scool of Medicine, where researchers studied all birth defects that occurred between 1996 and 2002. Their findings show that there seems to be a correlation between time of conception and the occurrence of birth defects – and they pin the blame on pesticide use.

The two reports seem to contradict each other – More HLHS cases occur in children born from April to July, while more birth defects occur in children conceived during April to July. I think that’s the difference – the Indiana research is looking at all recorded birth defects – the Centers for Disease Control (CDC) recognizes 22 different categories of birth defects – while the Cincinnati researchers looked only at heart defects. But no matter what set of data you study, it seems to suggest that something is going on.

And if smart people can figure out what it is, perhaps they can stop it.

It doesn’t have to be this way

March 30, 2009

There is a new report being presented at the American College of Cardiology meeting in Orlando today. You could conclude that this report is disturbing, but I want to dispute it. No, I am not saying that the report is incorrect, but that you don’t have to be “trapped” by the results.

This report contends that even though Congenital Heart Surgery techniques save a child’s life, their quality of life may not improve. The full text of the press release is here. Since the full report is not available at the moment, let’s look at a few points in the press release:

“Advanced treatments often result in unintended complications, particularly when combined with the hemodynamic impact of the heart defect itself,” says Bradley Marino, MD, a cardiologist from the Heart Institute at Cincinnati Children’s Hospital Medical Center and the study’s lead author. “The child’s neurodevelopmental, psychosocial, and physical functioning are all impacted by these complications, and they may adversely affect the child’s quality of life. If we know the predictors, we can put interventions in place to change the outcome for them.”

I actually agree with and support what they are trying to do. As we all know, even though two children may have the same heart defect, the physical effects can be vastly different based on the severity of the defect. Two people, for example, can have a Ventricular Septal Defect (VSD), yet one can be affected by it much more than the other due to how big the VSD is and where in the heart wall it is located.

To measure how much of an effect these factors have on children, Dr. Marino and his team developed the Pediatric Cardiac Quality of Life Inventory (PCQLI), a self-administered questionnaire that quantitatively assesses health-related quality of life in children and adolescents age 8-18 years, and their parents. The questions are designed so that anyone with a third grade reading level can understand and answer them in fewer than 10 minutes.

Dr. Marino and his colleagues recruited 759 pairs of patients and their parents. The patients were between the ages of 8 and 18 and had at least one prior cardiac surgical procedure. The researchers evaluated the effect of income, socioeconomic status, recent hospitalization, Fontan palliation (a surgical approach to treating hearts with one ventricle), and a greater number of doctor visits in the past year.

The Quality of Life Inventory seems to be a list of simple questions that 759 patients and their families answered. “Fontan palliation” means that the patient only has one ventricle, so this would put patients with Hypoplastic Left Heart Syndrome (HLHS) and defects that affect the right ventricle – like Tricuspid Atresia – on the study list. So I would be considered for this study. Also, it seems that there are very few factors being studied that the patient can affect. Number of doctor visits could be affected by both income and socioeconomic status, as a family with fewer resources could be more likely to skip a doctor’s appointment.

Modifiable factors associated with lower quality of life scores among patients after surgery included recent hospitalizations and greater number of doctor visits. Non-modifiable factors included Fontan palliation and both lower income and socioeconomic status.

The study takes this into account – you can get more or fewer “points” based on hospitalizations and doctors visits. But a lower income, a lower socioeconomic status, AND having only one operational ventricle are automatic deductions.

So I get points off for only having one working ventricle. The study is right, this IS a limiting factor. But the study is also wrong… I have met a lot of Tricuspids and HLHS survivors who are full of life. Two women I met at the Adult Congenital Heart Association (ACHA) convention in Philadelphia both have Tricuspid Atresia, both of them are full of love for life and have a full schedule. One of them is engaged! I love them both like sisters – I’ve said before that my cousin is the sister I never had, but now I have THREE sisters!

I also met Rose, a young lady with HLHS who was looking into an uncertain future, because she is among the oldest HLHS survivors alive and no one can predict what might happen to her in the future. So Rose had brought her heart-healthy fiance to the convention: If he was going to put a ring on her finger, he needed to know what the score was! (You go, Rose!)

Do not get me wrong – living with a heart defect, especially one that leaves the patient with one ventricle, can be difficult. You have to adapt an attitude that you are going to take care of yourself, and be disciplined enough to know that there are things you can’t do and things you shouldn’t eat, and stay away from them. It is difficult to find a child with this attitude – that kind of self control only comes with age and experience. Even now, I “misbehave” occasionally.

So don’t let this study turn you into a “woe is me!” person, or make you into an overprotective parent. Your quality of life is determined by what you do, and how much effort you put into it.

Two guys, three and half hearts, and twenty holes of Golf

March 9, 2009

“Golf is a good walk spoiled,” according to Mark Twain. And to be totally honest, it’s not my game. I can make neither heads nor tails of it, and can find no good reason to watch it on TV.

But on Friday afternoon at the Honda Classic, a young man named Erik Compton was playing. And John Paul George was by his side.

Compton was born with Cardiomyopathy and had his first heart transplant at the age of 12. Things were going well for him until last year, when he suddenly had a heart attack and wound up in the hospital again. His attack resulted in a second heart transplant, and now here he is, ten months after getting his (second) new heart, playing in a Professional Golfer’s Association (PGA) level golf tournament. Compton hasn’t yet qualified through “Q-School” (the PGA’s testing program: do well in Q-School and you can get your PGA card, which allows you to enter tournaments as a professional) and had to get a sponsor’s exemption to play.

Enter John Paul George.

John Paul (named after the Pope, not the Beatles) is a 14 year old Cardiac Kid from Juno Beach, Florida living with Hypoplastic Left Heart Syndrome (HLHS). He’s about half the size of a “normal” 14 year old teenager and looks like a child, but John Paul is a rock. His dad spoke with Erik Compton after a practice on Wednesday, and John Paul was invited to hang out with Erik during the tournament on Friday.

There was one catch: Compton had been granted the right to use a golf cart, something rarely done on the PGA Tour. He planned to turn it down and walk the course, to prove that transplant patients could live their lives to the fullest. John Paul could use a cart if he wished; after all, he wasn’t competing. But John Paul refused. If Erik was walking, he was walking too.

Darkness had stopped the match the day before, so Erik had to finish two holes before “officially” starting Friday’s round, but John Paul was good to go. Together, the guy on his third heart and the boy with only half a heart took on The Honda Classic. Erik was shooting the lights out and John Paul was right there with him, every step of the way. Compton shot a 69 and for a moment, was tied for second place.

In the perfect world Erik would have swept into first place with John Paul by his side, outplayed everyone on Saturday and Sunday, and won the whole bloomin’ thing. But that only happens in bad fiction. Y.E. Yang won the tournament; Erik finished tied for 44th and won $16,000.

But it’s not about the money. It’s not about the game.

It’s about overcoming the obstacles in your path and living your life, no matter what. And in that contest, Erik Compton and John Paul George tied for first place.

In it to Win it

November 9, 2008

In case you haven’t heard, Katie passed away earlier today. Hypoplasic Left Heart Syndrome (HLHS) is one of the most difficult Congenital Heart Defects to overcome: The three surgery combination that can allow the heart to function wasn’t even developed until the 1980’s. Before that, HLHS was almost 100% fatal. And Katie is proof that we are still a long way from completely beating HLHS.

Hear this and hear it well: Adventures of a Funky Heart is declaring war on Congenital Heart Defects. There shouldn’t be any Heart Moms, Heart Dads, Cardiac Kids, or Heart Warriors. That’s why I’m going to Washington, DC, to lobby the United States Congress this coming February. As of last week, 78 have signed up to participate – a few more than participated in Lobby Day 2007. There is still time to register, CLICK HERE.

“Go west, Funky Heart!”, and that’s where I’m going, to participate in an Adult Congenital Heart Association (ACHA) event in May 2009. Details aren’t finalized yet, but I’m planning on letting you know all about it. I can tell you that it’s big, it’s bold, and Funky Heart and the ACHA are gonna be right smack dab in the middle of it!

It would be wonderful if you could travel to DC and work with us during Lobby Day. If you can’t, I plan to show you exactly how to lobby your Congressional Representative through e-mail, telephone, and fax. I’ll let you know more about May 2009 when everything is finalized, and perhaps you can join us there, too!

And if there is any other opportunity to get the word out about Congenital Heart Defects, those of us living with them, and the need to beat this illness, I’ll be involved. Will you?

On the Street Where You Live

November 6, 2008

This past week I’ve spoken a lot about Katie, sometimes I wonder if I’ve said a little too much. I’ve been asked, both online and in real life, if maybe I’m paying just a little too much attention? It’s not really a surprise, as I thought about it myself.

I’ve never met Katie or her parents. All I have learned about them, I’ve read on their website. It’s been a hard thing to try to follow the recent news about her, because it hits so close to home. Katie is five months old – the same age I was when I had my first heart surgery.  She’s had the Bi-Directional Glenn; I’ve had the original version of that operation. Katie is at Yale New Haven Hospital, where Dr. William Glenn himself lived and worked. I’ve read that Dr. Glenn, despite being a world famous heart surgeon, would leave his home on Saturday mornings and walk to Yale’s football field to cheer on the home team. He sounds like my kinda guy.

Katie has Hypoplastic Left Heart Syndrome (HLHS). I have Tricuspid Atresia, which is occasionally referred to as one of the defects that make up Hypoplastic RIGHT Heart Syndrome. (HRHS) The term HRHS wasn’t even invented when I was born (I don’t think anyone had thought of HLHS, either) so I’ve always just referred to myself as having Tricuspid Atresia. But when you think about it, Katie’s heart and mine are nearly mirror images of each other.

Spooky. As the title of this post notes, it hits you on the street where you live.

After my surgery at Johns Hopkins in 1967, one of the doctors mentioned in passing that we were one of only two families who had made it in that night. The other child had been born premature, and despite being healthy in every other respect, hadn’t made it. The doctor shook his head. “Some children…they just aren’t fighters.”

There’s no doubt about it, little Katie is a fighter. She’s hanging on for all she’s worth, clawing and spitting and digging her nails in. Keep fighting, Katie! I’m pulling for you!


October 19, 2008

Holding a copy of Helen Taussig’s book Congenital Malformations of the Heart is almost like holding a copy of scripture. This is where it all began: before publication of this book in 1947, there was almost no understanding of Congenital Heart Defects (CHD). No way to diagnose them. No field of Pediatric Cardiology, barely any heart surgery to speak of. Very few survivors. No groups like The Congenital Heart Information Network (TCHIN), Mended Hearts or the Adult Congenital Heart Association (ACHA). Hardly anything. And certainly no Adventures of a Funky Heart.

I have heard stories of doctors from the late 1940’s and early 1950’s examining their patients with a stethoscope in one hand and Taussig’s book in the other. And finally, instead of telling the worried parents that the situation was hopeless, being able to offer at least a small chance: “They’re making amazing progress with surgical solutions at Johns Hopkins, maybe if we contact them…”

She was years ahead of her time. Taussig wrote an entire chapter concerning a defect she described as “Atresia or marked hypoplasia of the aortic orifice prevents the expulsion of blood from the left ventricle in the normal manner. In such abnormalities the development of the left ventricle is also usually defective.” This defect is almost certainly what we know today as Hypoplastic Left Heart Syndrome (HLHS).

Other records exist that actually predate Congenital Malformations. Alfred Blalock’s surgical notes from the first Congenital Heart Surgery (later known as the Blalock-Taussig Shunt) have been saved and are online. You can read them here. (Page 1 Page 2) And here is a photo of the clamp that Vivien Thomas designed for the surgery.

The first operation occurred on November 29, 1944. Blalock and Taussig wrote a paper about the shunt that was published in the Journal of the American Medical Association on May 19, 1945, that brought the news to the world. (Here’s a drawing from that article). Normally works in medical journals are not noted in the public press, but a medical reporter who was actually a doctor (and not a well informed amateur, as many of them are) read the article. He wrote a short blurb about it that was printed in his paper and picked up by the wire services.

And the people came. At first they went to their local doctors, to find out all they could about this new operation. And then they came to Johns Hopkins Hospital…on the train, in cars, and some of them actually walked, bringing their sick children to the one place that might could help them. Blalock and his team actually performed an estimated 300 heart operations in a single year. The American Weekly printed an article about Taussig, Blalock, and the B-T Shunt in its February 17, 1947 issue and still more people came.

And in late 1947 Congenital Malformations of the Heart was published. The life changing surgery was only designed for Tetralogy of Fallot (ToF) patients, but now there  was a way to figure out exactly what was going on in a defective heart, and with knowledge came a chance.

There is a small section titled “The Plan of the Book” in which Taussig notes how her book is organized. In the second paragraph is this chilling sentence, which was true in 1947: “Although occasionally a patient with one of these malformations may live to adult life, in most instances death occurs before eighteen months of age.*” At the bottom of the page, the footnote reads “*The recent advances in vascular surgery may alter the prognosis in this group of malformations.”

I think Dr Taussig would be thrilled with the results of recent advances in vascular surgery.

My Glenn Shunt is worth more on eBay!

October 13, 2008

Yeah, you read that right. My Glenn Shunt would bring a higher price on eBay! Yours? Not so much. I have a classic vintage model, so the price would be higher!

I’m kidding with you, obviously. If you happen to need a Glenn Shunt (or any other heart operation) then the true cost is out of your reach; it’s priceless.

The Glenn Shunt is one of the oldest heart operations around. It was first described in 1951, and Dr. William Glenn of Yale University first reported performing the procedure successfully in 1958. Since he was the first person to routinely have success, the operation bears his name. (If you or someone you know has a Glenn shunt, please click THIS LINK and download and read the PDF file. There is a lot of important information here that you need to know!)

When I tell people I have a Glenn Shunt, the ones who know what I’m talking about will nod their heads knowingly. Most of the time, though, they are still wrong. My Glenn was done in 1967, and I am a proud owner of a Classic Glenn Shunt. Most of the Glenns done today are the Bidirectional Glenn Shunt.

So what’s the difference? Before you describe the Glenn, it helps to have a diagram to help you visualize it. Click HERE for a useful diagram of the heart.

In the Classic Glenn, the Superior Vena Cava (The large vessel that leads into the Right Atrium) is closed near the Right Atrium (usually, it is not cut, but rather sewn closed.) The Pulmonary Artery (the “T” shaped blood vessel that runs under the “loop” formed by the Aorta) is also cut… the right branch of the Pulmonary Artery is disconnected. The hole left by cutting the right branch of the Artery is sewn closed, and then the right branch is connected to the side of the Superior Vena Cava.  By doing this, the Right Atrium is completely removed from the blood flow. Blood coming to the heart through the Superior Vena Cava now goes directly to the Right Lung, and flows back to the Left Atrium normally. Then it goes through the Left Ventricle and back out to the body.

The Bidirectional Glenn was invented, surprisingly, in 1966. While it was around when I had my Classic Glenn in 1967, my operation was the fifth Glenn Shunt (of any kind) that had been performed at Johns Hopkins; so it is a safe assumption that the surgeons weren’t prepared to try the new version just yet. In fact, the Bidirectional Glenn really came into its own in the 1980’s, when it became the second step in the three operation Norwood Procedure used to combat Hypoplastic Left Heart Syndrome (HLHS).  It’s also part of the Fontan Procedure, sometimes performed as a seperate operation as part of a Staged Fontan.  The biggest difference in the two operations is that in the Classic Glenn, the Superior Vena Cava is completely cut and sewn into the right branch of the Pulmonary Artery. In the Bidirectional Glenn the Pulmonary Artery is not cut, which allows blood flow to both lungs.

It’s important for someone with a Congenital Heart Defect (CHD) to know what “version” of an operation they have had. For years, I told doctors “I have a Glenn Shunt,” not knowing that the operation had been changed. After I had told a new doctor that I had a Glenn Shunt, he slapped my X-Ray on the lightboard, took a long pause, and finally said “I don’t know what the hell this is, but it ain’t no Glenn Shunt.” Only after the head of the Cardiology Department came in and said “I haven’t seen one of those in a while!” did I realize that simply saying “Glenn Shunt” wasn’t good enough. Thankfully that snafu occured during a routine office visit and not a crisis visit to an Emergency Department.