Which HLHS surgery works better?

Update: My friend is out of surgery and doing well!

The three operation Norwood Procedure was developed in the 1980′s as an answer to Hypoplastic Left Heart Syndrome (HLHS). Since infant heart transplant was developed at about the same time, HLHS was 100% fatal before the invention of the Norwood.

But the results from those early Norwood Procedures varied wildly, especially in the early days when the Norwood consisted of only two surgeries. When the Sano Shunt – a direct Right Ventricle to Pulmonary Artery connection, using a “tube” made of Goretex – was introduced in early 2003, matters improved greatly. The Sano Shunt provided the heart with much more stability and increased survivability rates.

But while we know that the Sano works better, there hasn’t been much research into how much better it works…. until now.

The Pediatric Heart Network studied the cases of 555 infants with HLHS. Through random selection, roughly half of the infants received the Stage I Norwood with a Sano Shunt, the other half received the Stage I Norwood with a Modified Blalock-Taussig Shunt (MBTS).

Followup with the children showed that 26% of the Sano Shunt children required a heart transplant before the age of 1; 36% of the children with the MBTS version needed a transplant. But after two years, the number of children who needed a heart transplant was within four percentage points of each other. Good Pulmonary Artery growth helps in the success of Stage II and Stage III of the Norwood, and the MBTS seems to encourage Pulmonary Artery growth.

Meanwhile, the Sano Shunt had more complications that required a balloon or a stent to keep the shunt open. True, this is not as serious procedure as open heart surgery, but the fewer, the better. After all, minor surgery is any surgery that happens to someone I don’t know – when it is YOU, your child, or a family member, things quickly become very serious!

Overall, it seems that the Sano Shunt gives an HLHS patient better results over the first two years – after that, the odds even out. These children will have to be followed for years to determine the long term success rate of either the Sano Shunt or the Modified Blalock-Taussig.

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.

Gracie’s Theme

Gracie Gledhill had to fight from the moment she got here. Born with Hypoplastic Left Heart Syndrome (HLHS), Gracie had the first stage of the Norwood Procedure at four days old. She never received the second operation, the Bi-Directional Glenn Shunt, because her doctors felt that she was too sick for the operation. Finding a transplantable heart became her only option. (For those who would like to know, all three surgeries to repair an HLHS heart are very well described in the HLHS link)

Gracie  fought and scratched, tooth and nail, never giving up. And finally it looked like she was about to win: the call came, a donor heart had been found, and Gracie was the lucky recipient.

Not so lucky… the new heart was itself damaged, the left ventricle barely functional. Gracie fought, her doctors fought, people worldwide (including Funky Heart readers) prayed, but in the end, Gracie slipped away – not quite one year old.

Fellow Heart Warrior Paul Cardall had become friends with the Gledhill family and introduced his new instrumental Gracie’s Theme at her funeral. Paul has honored Gracie again, by assembling a short video of her life with Gracie’s Theme as the background music.

“I dedicate this video to every family who has lost a child.” – Paul Cardall

My Glenn Shunt is worth more on eBay!

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.

Stopping the Unstoppable

It’s hopeless.

We can’t do anything.

That is what parents of babies diagnosed with Hypoplastic Left Heart Syndrome (HLHS) were told not many years ago. The word Hypoplastic means “underdeveloped”, and that is exactly what has happened: the heart’s Left Ventricle is much too small to pump blood to the body. The hypoplasia extends much further than the Ventricle; the Aortic and Mitral valves are tiny (or nonexistent), and the Aorta itself is tiny. The child is alive only because of a Patent ductus arteriosus, a “built in” heart defect everyone is born with.

The Ductus Arteriosus is a small connection between the Pulmonary Artery and the Aorta that allows the heart to function while still in the womb. Once the child is born, the connection will close by itself, usually in less than two weeks. A connection that is “stuck open” — a Patent ductus arteriosus — is easily corrected. But in this case, when the ductus closes, blood flow drops and the child goes into shock.

HLHS was the defect the doctors had no answer for. Obviously, a surgical repair was the only option, but how? How do you fix a heart that is, for all intents and purposes, missing the left half? A heart transplant was an option, but rarely was an infant’s heart available, and there was almost no time to find one.

But in the early 1980′s the answer came. Rather than try to fix everything at once, make the repair in stages. Three operations, (known collectively as the Norwood Procedure) performed in sequence, allowed the child to survive. Certainly it was understood that this wasn’t a cure, but the child could live if he or she received lifelong medical care.

Fast forward to 2008. The first generation of HLHS survivors are now in their early to mid 20′s. And with no older survivors, no one has any idea what future health problems may affect them. One of the goals of the Adult Congenital Heart Association is a national registry of patients with heart defects. If data is gathered about those of us living with a Congenital Heart Defect right now, the data can be analyzed and used to predict what might happen next.

Or it can be used to make things a little easier for the next generation of CHD patients. As an example, I take 11 different medications. Yes that is a lot, but some CHD’ers take more, and some take less. If the knowledge gained from a national registry could eliminate one medication that each of us has to take, the savings would be phenomenal. The ACHA also supports training for cardiologists in Adult Congenital Heart Defect care. We support ongoing care for adults with a CHD, especially those of us with a complex defect.

Much too often you read in your local newspaper about the child who needs surgery for a major heart defect (Infant Survives Heart Surgery, reads the title of the one and only article about me in the local Daily Planet) but what happens after that? We grow up, get jobs, pay our taxes, fall in love… in other words, we live our lives.

And that’s the most important thing we do.