Archive for the ‘Atrial Septal Defect’ Category

Rebuilding a damaged heart

June 29, 2010

I’ve written about the Wake Forest Institute of Regenerative Medicine and the Bioprinter, both attempts to create organs in the lab. But instead of creating entire organs from scratch, doesn’t it also make sense (or sometimes make more sense) to just rebuild the damaged section?

CorMatrix has just received approval to market its bioscaffold material in the European Union. It’s been available over here since 2006, which makes it unusual. Medical devices almost always come into use in Europe before they are introduced over here.

Bioscaffold means “living framework” and that is just what the new material is. If you were to take a piece and have it analyzed, you’d learn that it is made mainly from pig intestines. But when you use it to patch an Atrial Septal Defect (ASD) for example, it changes. Oh it is still the pig innards, but instead of truly repairing the ASD, it just covers it. The material encourages cell growth, and before too long the patch will be completely covered by heart tissue.  As time passes the patch material will dissolve, leaving… nothing. No ASD, no patch, only cardiac cells. And since the cells are the patient’s own body, they’ll grow right along with the patient and never be rejected.

Personally, I’m looking forward to the day when scientists can build an entire heart in the lab! But it is a complex organ, and before they get that far they’ll do things like this – figure out what parts of the heart are working and rebuild the damaged sections. It just makes sense.

The Gerbode Defect

May 20, 2010

Here’s an echocardiogram of one of the most rare Congenital Heart Defects (CHDs) that exist. Look closely or you won’t see what is known as The Gerbode Defect.

The Gerbode Defect isn’t an Atrial Septal Defect, though it acts like one. It isn’t a Ventricular Septal Defect either, but it acts like one of those, too. The Gerbode Defect is a connection between the Right Atrium and the Left Ventricle. (CLICK HERE for a simple diagram.)

And again, that’s not really the problem. The defect lies in the Tricuspid Valve – there is either a small “gap” in the base of the leaflet closest to the Septum or there is a small hole in the septum just at the base of the Valve. (Here’s a diagram of the Tricuspid Valve showing the possible location of the defect.)

The Gerbode Defect is hard to find, hard to diagnose, and easy to miss. The defect is so rare that researchers at Children’s Memorial Hospital in Chicago report that they observed six cases of Gerbode Defect over a nineteen year period. And while most of the time it is a Congenital Defect – you are born with it – Gerbode can also be an acquired defect.

Wha…? How is that possible? Endocarditis can cause the valve or valve wall to deteriorate. Here’s yet another reason to avoid Endocarditis if you are a CHD Survivor!

Five CHD Questions: When?

May 12, 2010

When are Congenital Heart Defect (CHD) survivors at their most vulnerable?

There are two schools of thinking in the CHD world. For lack of a better term, we’ll call these two philosophies “Old School” and “New School”.

The Old School is what Cardiologists used to tell CHDers and their parents: Now that the child has his/her surgical correction(s), they are “fixed”! Certainly, they may be a step slower, not be able to do as much, can’t play as hard as the other kids, but their defective heart has been repaired and they are set for life. All is well that ends well!

New School is the reality: As time passed as the early survivors began to grow into adults, they started having problems related to their deformed hearts. Finally it was realized that just maybe that original repair wasn’t permanent, and some of the old problems were coming back. So now, New School Cardiologists tell their patients that while they are repaired, a CHDer needs lifelong care by a specialized Cardiologist to make sure that those problems stay under control.

Be warned, the Old School of thought still exists, despite all the evidence to the contrary. Old School thinking exists on several levels – not only does it include the fallacy of “once repaired, totally repaired”, there is also a segment of the population who think Heart Defect = Death Sentence. Such thinking is toxic and you do not need this in your life: The best thing you can do if you should run into someone with an Old School mindset is to RUN!

The most dangerous time for a CHDer is when he/she begins to transition away from their Pediatric Cardiologist to an Adult Cardiologist. Most Adult Cardiologist deal with Acquired Heart Disease, not Adult Congenital Heart Defects (ACHD). Acquired Heart Disease are heart problems caused by diet, clogged arteries, hardening of the arteries, high cholesterol, and the natural aging of the heart. Someone with a Congenital Heart Defect will not have these problems, or they will manifest themselves differently in our bodies. So we need someone experienced with caring for Adult Congenital Cardiac patients – an ACHD doctor.

The problem is, there are currently very few ACHD doctors available. In an interview recently posted on Adventures of a Funky Heart!, Dr. Michael McConnell gave an example: There are about fifty Pediatric Cardiology practicing in the city of Atlanta, Georgia, but only two part time ACHD Doctors.

ACHD doctors can be found, though it may take some searching. The Adult Congenital Heart Association (ACHA) offers an ACHD Clinic directory for the United States and Canada on their website. (ACHA Membership is free). ACHA is also pursuing Vision 2020, a proposed blueprint for Adult Cardiological Care in the 21st Century.

Vision 2020 builds on the Bethesda 32 Report produced by the American College of Cardiology. Bethesda 32 was produced in 2000 and addresses the issue of Adult Cardiological Care. (The name is derived from the 32nd meeting of the American College of Cardiology’s yearly focus group held in Bethesda, Maryland.  The group studies a different issue each year.)

The Bethesda 32 Report divides the known heart defects into three groups – “Mild”, “Moderate”, and “Severe” and recommends the proper level of care needed for each group. While someone with a “mild” heart defect – a very small unrepaired Atrial Septal Defect (ASD), for example – can usually be cared for by the general medical community with yearly ACHD checkups, someone with a “Severe” defect (my Tricuspid Atresia, for example) needs to be seen in at an ACHD Clinic regularly. Severe CHD patients should also consult with their home clinic before undergoing most medical procedures, getting pregnant, etc.

There is a good outline of the Bethesda 32 Report HERE; you can read the report online by clicking THIS LINK. Click “Print a PDF” from that page to print out a copy of the Report. Be prepared; it is over 60 pages long.

Having a copy (or several) of Bethesda 32 can come in handy when/if your insurance company balks at providing coverage for an out-of-network doctor. The report can be used to show established medical guidelines saying that you need to be seen by a specialist.

The profile of the “average” Heart Defect Survivor is changing, as there are currently more adult survivors than there are children. Congenital Cardiac Care is slowly changing to reflect this.

Did you cause your child’s Heart Defect?

April 9, 2010

There has been some recent news coverage that contends that you just might have, if you were obese when you became pregnant.  But just in case you need to be reminded, News organizations make money by selling their product. There are lots of ways to do this, but two of them are really effective: 1) Make something so appealing that you just have to know more; and 2) Scare you so bad that you tune in/buy a copy to learn how to protect yourself.

How many Heart Moms look at their child in an ICU unit and ask themselves “What if…?” I’d wager that the answer to that question is all of them.

So you need to take health news on TV and in magazines with a grain of salt. A big grain of salt, really. The first thing I would recommend is that you drop by Gary Schwitzer’s Health News Review Blog. Gary has been in the Health Reporting field for 30 years, and he’ll tell you if the latest news report is wrong, and how it is wrong. This is a great resource to check out all those “We’re doomed!” news stories for yourself. Gary can teach you how to look at those news stories with a critical eye and not be scared to death.

Don’t take everything you read or hear at face value; if possible, try to find the original research. Read the information for yourself; then make an informed decision.

Did you cause your child’s Heart Defect? No one can tell you for sure. The new report that is stirring so much concern says that there is a better chance of having a child with a Heart Defect, but obviously it is not a certainty.

The heart begins as a straight tube, much like a drinking straw. As the heart develops it actually starts to beat in this configuration! Later, it will twist and fold over onto itself, forming a loop that will begin to grow together and form the organ that we are all familiar with.

All this happens before you even think that you might be pregnant. By the time you see the doctor to confirm it, the heart has formed. If you are destined to be a Heart Mom, it has already been decided.

Now this doesn’t mean that you can ignore all of your doctor’s advice! But think about it – millions of women have been like my mom – she did everything right, tried to follow their  doctor’s instructions to the letter, and still  I was born with a Heart Defect. Other women had every vice known to man, and their children were born healthy. So what causes Heart Defects?

I think that the main cause is a combination of genetics and environment. Research funded by the March of Dimes has found several genes that interact to help the heart form. Of special interest is the GATA4 gene – something it does helps the heart split into the left and right sides and form the Septum.  Figure out what is happening there, and maybe you can stop ASDs and VSDs!

The National Heart Lung and Blood Institute (NHLBI) has also determined that genetics play a part in the development of Hypoplastic Left Heart Syndrome (HLHS). A study of the family medical history of HLHS patients found that their ancestors also had a specific heart problem: a Bicuspid Aortic Valve. So apparently there is a genetic cause for HLHS – now the challenge is to find it.

Environment also contributes to heart defects. A section of Baltimore, Maryland contains a “cluster” where the incidence of HLHS is twice as high as the national average. What is going on there? What makes that area so different that it could trigger a Heart Defect?

Nationally, the incidence of Heart Defects is 1 out of every 125 newborns. But in the state of Wisconsin, the rate is 1 out of every 74 newborns. Wisconsin isn’t taking this lying down – they’ve created the Wisconsin Pediatric Cardiac Registry and are studying the data that it has generated, looking for clusters and what might be in the area that could create a Heart Defect hot spot.

What is the interaction between genetic factors and environment? What happens that triggers the formation of a heart defect? No one is certain, and the scary answer is that different factors contribute to different heart defects.  But if one day we could answer those questions, then maybe we could slam the door on Congenital Heart Defects.

Because every heart deserves to live a lifetime.

Devil in the Slot

March 2, 2010

BONUS: 100 Karma Points to the first person who can leave a comment telling me where the title of this post originated!

You have a heart defect.

No, I’m not talking about the 1 in 125 of us who live with a Congenital Heart Defect, I’m talking about YOU. Mr. or Mrs. Average. You were born with a heart defect. Two of them, actually.

Obviously, you don’t need to breathe while you are still in your mother’s womb. You can’t breathe, unless you can somehow magically breathe fluid. So your lungs are “turned off” and you have two small defects that allow the heart to function but blood to bypass the lungs and pick up its oxygen from your mother. When you are born and you take your first breath, the body sends a signal to those two defects to shut down and for the lungs to take over.

(Pretty cool, huh?)

One of these small defects is called the foramen ovale and it is really a very small Atrial Septal Defect. In fact, it isn’t really a hole in the septum, it is two overlapping flaps. When you begin to breathe and blood begins to flow properly, these two flaps will eventually seal closed and everything will be great. In about 30% of people, however, it doesn’t close. When that happens, it is called a patent foramen ovale, sometimes called a PFO (“patent” means “stuck open”).

Many times this doesn’t cause much of a problem; sometimes it is even undetectable unless the patient coughs while having an ultrasound.

Now here is the problem: when a person has a PFO, or when it is slow to close, occasionally a small “pouch” forms in the wall of the Left Atrium. Doctors at the University of California at Irvine recently found that pouch while doing autopsy research, and it could be the source of several problems.

That little pouch is nice and quiet; any blood that gets in there settles down – it’s no longer part of the normal blood flow, and it is pretty calm in there. While the heart is pumping and blood is churning all around it, it’s a nice quiet little neighborhood… almost a gated community. (This Link has a good explanation and has a nice drawing of the pouch about halfway through the article. )

That’s not good. Blood can settle in the pouch and form a clot. And since it is on the left side of the heart, the clot skips the lungs (which not only add oxygen to the blood, but they also act as scrub brushes, too!) and then it is off on a tour of the body. Round and round she goes, where she stops nobody knows. But if it stops in the wrong place….

STROKE!

So if the doc tells us we have a PFO, we should get it closed, right? Perhaps, and perhaps not. The medical evidence isn’t in yet. We know what might happen, what could happen – but how many times is the pouch really the cause of a stroke? We don’t know yet. A clinical study (the RESPECT trial) is being conducted right now to determine if a PFO causes more strokes. At least one doctor isn’t waiting to find out – she’s full steam ahead. The Food and Drug Administration is saying wait a minute, slow down, catch your breath… let’s see what the evidence says. Then we can decide. (You really need to read what Isis has to say about PFOs, she gets the point across quite well, in simple English and with a lot of humor, too!)

So, is there really a devil hiding in that “slot” that could be in your heart? And if there is, what are the chances of him coming out? No one knows… yet.

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.


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