Archive for the ‘Fontan Procedure’ Category

Back to School

November 12, 2010

“Trouble is, there’s not enough of us to go around – we’re spread thin, so sometimes, important things get ignored or don’t get said.” – Judge Tolliver (John Goodman), The Jack Bull (1999)

Yesterday I had the chance to go back to school – I went back to my Alma Mater to our School of Nursing!

I asked for directions just to be sure – when I graduated the School of Nursing didn’t exist. And I didn’t want to assume I could find it only to discover that it was in a back corner of the campus. But it wasn’t a problem; as soon as I turned off of the main highway and scanned the campus, there it was. Everything was exactly as described, which usually doesn’t happen – more often than not, I get twisted up and turned around but everything went perfectly.

After meeting the instructor for the first time, we went upstairs and into a classroom where I met the students (about 30 of them) and talked about myself and my heart defect. If you’re a reader of this blog, you know I usually post the printed text of my presentation. Not the case this time – as I told the students, when I make a presentation to Heart Families, I’ve got a plan and am pretty sure what I will say. With them, I wasn’t sure what they wanted or needed to know, so I’d just talk about myself. If they had a question, feel free to break in.

Someone had a really good question about did I need oxygen. Technically no, I don’t need oxygen, but I sleep with a flow of four liters per hour. It was originally prescribed to keep my Hemoglobin down, and I can skip it for several days without problem. When I take a weekend trip, I don’t take it with me. But I’m like a rechargeable battery and the O2 is like my charger – after about 4 days of sleeping without oxygen, I feel run down.

After I talked about myself and the Question and Answer session, we moved over a larger room set up as a hospital ward. When I walked in there was a bed to my right with a medical mannequin in the bed, tucked under the covers neatly. I saw him/it out of the corner of my eye and for a moment there I thought it was a real person!

We didn’t bother Earl (or whatever the mannequin’s name was), he looked comfortable. I took another bed, and in pairs and threes the students came in and examined me. My heart was listened to more times than I can could count, and everyone took a close look at my blue fingernails. My right hand is a little more blue than usual because of the swelling associated with my wrist, but my left hand is better suited to observe Capillary Refill (press down on the fingernail until it turns white, then release. Observe how long it takes for the blood to flow back.)

More than one student seemed to be very interested in the fact that you can’t read my pulse in my left arm – a side effect of the Blalock-Taussig Shunt, the surgery I had in 1977. In the Blalock-Taussig, the Left Subclavian Artery is cut and sewn into the Pulmonary Artery. The Left Subclavian normally passes near the shoulderblade (the Clavicle), and down the left arm. Because it has been disconnected, you can’t get a pulse in my left arm, can’t take an accurate blood pressure reading, and blood draws and vaccine injections should be done in the right arm.

(NOTE: If you have the Modified Blalock-Taussig Shunt, a small artificial connection is used to connect the Subclavian Artery to the Pulmonary Artery and the Subclavian is left intact. You usually can feel the pulse bilaterally on a patient with the Modified Blalock-Taussig!)

When the students weren’t listening to my heart, the instructor was – with an electronic recording stethoscope. I’ve had this done before, back in 1977 at the University of Alabama at Birmingham (recounted in this post.) But back then it was a Stethoscope head connected to wires that ran to a machine the size of a toolbox, that recorded my heart on a cassette tape. This one looked like a regular Stethoscope, perhaps a little thicker around the head. It would record (sensitive enough that it recorded a cough!) and then transmit the recording to a laptop computer via Bluetooth! My Geek side started getting the best of me, and I was developing a very, very bad case of STEVE WANT! But I knew that if I asked how much it cost, the instructor would inject me with 1000 cc’s of reality. Reality is a difficult drug to take – it’s good for you, but can make you feel pretty lousy.

I enjoyed my visit to the Nursing School and I’d like to thank everyone for making me feel so welcome. Even if none of the students chose to work in Congenital Cardiology, they’ll bump into other patients like me – it’s estimated that in the United States, there are slightly more adults living with a Congenital Heart Defect than there are children. Adults with Heart Defects are living longer and better, and we’ll have “normal” medical problems in addition to our bad hearts. And today’s Cardiac Kids are growing into tomorrow’s Heart Warriors.

So its important for those of us with a heart defect to “meet the public” – and not just to raise awareness, but to educate. To guide new Heart Families through this scary world we never expected to enter, but also to give the professionals who will be taking care of us a chance to learn from us. it doesn’t matter if someone is the best Heart Surgeon, the best Cardiologist, or the very best Cardiac Care Nurse… sometime in the past, these people had no idea that Heart Defects even existed.

Someone had to teach them.

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‘These wounds I had on Crispin’s day.’

October 24, 2010

Monday, October 25:  Saint Crispin’s Day


This day is called the feast of Crispian:
He that outlives this day, and comes safe home,
Will stand a tip-toe when the day is named,
And rouse him at the name of Crispian.
He that shall live this day, and see old age,
Will yearly on the vigil feast his neighbours,
And say ‘To-morrow is Saint Crispian:’
Then will he strip his sleeve and show his scars.
And say ‘These wounds I had on Crispin’s day.’
Old men forget: yet all shall be forgot,
But he’ll remember with advantages
What feats he did that day.

– from Henry V by William Shakespeare, 1599

…And say ‘To-morrow is Saint Crispian’

October 24, 2010

If you are a fellow heart surgery Survivor, a Heart Parent, or just want to help rid the world of Congenital Heart Defects, feel free to link to Monday’s post. It will appear very late on the night of Sunday, October 24.

A disturbing report

September 23, 2010

Funky Heart reader Cindy forwarded THIS .PDF FILE that you need to read. Titled Forty Years of the Fontan Operation: A Failed Strategy, this report contains information that you should consider. Download it, print it, and read it carefully. But I’ll warn you: this report will make your hair stand up.

Forty years ago a heart defect that eliminated the Right Ventricle was “uniformly lethal” but today that same patient is “not only likely – but expected – to survive.” One of the major weapons in our arsenal is the Fontan Operation, the the Fontan comes with its own set of difficulties. The author, Dr. Jack Rychik of the Children’s Hospital of Philadelphia, considers it “a failed strategy.”

Dr. Rychik lists detailed statistics concerning the decline of Fontan patients, then explains why they tend to deteriorate: elevated central venous pressure coupled with a reduction in cardiac output. He then explains three important issues for a Fontan patient: liver damage; Plastic Bronchitis, and Protein Losing Enteropathy (PLE).

This report may be disturbing to many readers, but please remember: outcomes aren’t pre-determined. Dr. Rychik states that 40 years ago single ventricle defects were “uniformly lethal” – but I just turned 44. Heart defects aren’t like math, the answers are not so cut and dried. 2 +2 =4; 4 x 4 = 16. That’s a constant. But each defect affects the patient slightly differently, and the “standard answers” may not apply. When you are talking about a defective heart, 2+2 may equal 66.4.

Just making a guess, I believe that I know 12 to 18 people who have had the Fontan. That includes close friends, people I have met just once or twice, and some I only know through email and blogs. These people are in all stages of health – a few are doing wonderfully, most of them are doing good with occasional Bad Days, and a few can barely go. When you rank test subjects based on general health, Fontan survivors won’t be grouped around one point. They’ll be all over the place. To quote almost every Cardiologist who has ever discussed future options with a patient: It’s certainly not perfect, but it is what we have to work with.

Rychik concludes that in the past, giving a single ventricle patient the ability to live 30+ years was a noble goal. Today, it is unacceptable. He contends that Cardiologists and surgeons need to think outside of the box, and come up with new options – either redesign the Fontan (again); scrap it in favor of something else; or develop a Right Ventricle Assist Device.

Either way, every heart deserves to live a lifetime.

What you need in a Surgeon

September 15, 2010

It had to happen. Consumer Reports is now rating Surgical groups that perform Bypass Surgery. Just pick up an issue; you’ll be able to find out who makes the best toasters, washing machines, and who can re-route your arteries.

If it were only that easy for Congenital Heart Surgery. There are 35 different heart defects – this is the “base” number, that doesn’t count any defect that may have elements of two or more defects – and a multitude of different surgical options. Consumer Reports only looks at Bypass Surgery, a Congenital Surgeon may repair an Atrial Septal Defect (ASD) in the morning and perform a Bidirectional Glenn Shunt in the afternoon. If he’s on call he may come to the hospital in the middle of the night not knowing what problem he’s facing.

So assuming you have time to pick a Congenital Heart Surgeon, what do you need to look for? Experience and skill are the two basic factors, with a little understanding of what the numbers mean.

Experience is easy – Doc, how many years have you been operating? How many heart surgeries have you done, total? The Cardiologist says my son needs to have the *** Procedure, how many of those have you done? Answers to these questions will help you decide.

Now comes the not so easy part – try to determine your surgeons skill level. Let’s invent a situation: You ask the surgeon how his patients do after the operation, and he tells you that 80% of them go on to lead full lives. Now we need to know something else – what kind of operations is he doing? If our fictitious surgeon is fixing ASDs and 80% his patients “go on to lead full lives”… RUN! That’s a pretty low number. But if he is an Hypoplastic Left Heart Syndrome specialist and he usually performs the Norwood Procedure, 80% is very successful.

This is where it helps to be familiar with the Aristotle Complexity Score, and like so much about heart defects, the general public doesn’t know it exists. Most of the CHD community is even unaware of its existence. The Aristotle Score is a “rating system” of congenital heart surgical procedures, based on the three factors. Each factor is assigned a score of 1 to 5 points, then the points are totaled to find the Aristotle Complexity Score.

The first factor considered is Mortality: Historically, what percentage of patients having this surgery perish during or immediately after the procedure? An operation with a low Mortality receives one point; the highest Mortality receives 5. The next concern is Morbidity. Morbidity is defined as “a diseased state or symptom”, but in this context it is a measure of how long the average patient spends in the ICU after the operation. Again, short stays earn one point and the longest earn a five. The third consideration is the perceived difficulty of the operation. All heart surgery is difficult and requires years of practice and training – if it wasn’t, there would be a surgical center on every corner. But closing an ASD is easier than performing the Arterial Switch.

So every heart surgery is rated, with the “easiest” (a straightforward ASD repair) being rated at 3.0 and the most difficult (A Biventricular repair) receiving a 15.0. For example, my 1967 Glenn Shunt would be scored a 7.0. The Blalock-Taussig Shunt is a 6.8, while the Modified Blalock-Taussig (which uses an artificial tube to make the Subclavian Artery to Pulmonary Artery connection) is considered not quite as difficult at 6.3. The Score does not exist as a “ranking system” of surgeons (almost like Consumer Reports)  but as information. If the OR calls Recovery and tells them that we’re sending down a patient with an Aristotle Score of 10, they know a very ill person is on the way.

So knowing the surgeon’s experience and skill level is really only half the question. If you were to be able to determine how difficult his work is, that could increase (or decrease) your confidence level.

The Fontan

August 10, 2010

In late 1987, my cardiologist recommended that I have the Fontan Procedure. Now I was doing pretty good, he said, but having the Fontan would bring me to as close to “normal” as I would ever get. I thought about it, and decided to have the operation – after I graduated college. I was one semester (three months) from graduation and thought that waiting long enough to officially finish school would be a good idea. So that is what I did, and my surgery was scheduled for May 1988.

If you are a regular Funky Heart! reader you know what happened. After heart surgery the heart develops a layer of scar tissue, and in my case there was a lot more than anyone had anticipated, and it had stuck to the back of my rib cage. When my ribs were cut open that scar tissue tore. I required 20 units of blood and nearly bled out on the operating room table. My surgeon, Dr. Albert Pacifico, managed to get the bleeding under control and then backed out – the risk of restarting the bleeding was just too great. And if it started again, this time Pacifico and his team may not have been able to stop it. (“I tried every trick I knew to stop the bleeding,” he said later. “And I even had to make up a few new tricks on the spot.”)

The difficult thing about the Fontan procedure for me is that while I am somewhat familiar with the history of the procedure, I can’t understand it or explain it. I read the books, I study diagrams, (The Illustrated Field Guide to Congenital Heart Disease and Repair has several good illustrations. They don’t seem to help me understand what I am looking at, though!) but I just can’t get into my head how it works. And apparently that is a common problem – the “standard” cardiac treatments don’t seem to work as well, or work differently. The heart itself doesn’t control cardiac output; that really depends on the lungs. Patients who live at higher altitudes can show marked improvement by relocating to a lower altitude – in some cases an operation that has never worked right can suddenly find a happy balance as the altitude decreases. But before we put all of our Fontan Survivors who live in the Rocky Mountains on a train bound for the coast, the altitude adjustment doesn’t always work.

The Fontan Procedure is named after Dr. Francios Fontan, a French surgeon, and was first described in 1971. (CLICK HERE to see the article that describes the Fontan in its original form.) Even the professionals seem to have difficulty understanding exactly why and how the Fontan works: A study of 476 Fontan operations were analyzed and a “Fontan Score” assigned to each individual procedure. Variables that could affect the Score included “surgical center, age, weight, fenestration, length of hospital stay at time of Fontan procedures, and post-Fontan surgeries or interventions.” When the Fontan Score was analyzed, only 18% of the variables could be explained; the rest (82%) of the factors were unknown. And any long-term study of the Fontan is going to be confusing, as the operation has been modified over the years. (Had my operation been successful I would have the second version of the Fontan, and it has been modified since then)

Fontan Survivors are also subject to arrhythmia, and people with stable Fontan circulations can and probably should have a mild to moderate exercise program. It is important that the patient is stable, so don’t just drive down to the gym one day and sign up for Jazzercise classes. Consult your Cardiologist first!

Another problem – a big problem – for Fontan Survivors is the possibility of developing Protein-Losing Enteropathy, or PLE. PLE is an unexplained loss of protein from the body, usually through the intestinal tract. PLE is not a side effect of Congenital Heart Disease, it only affects people who have had the Fontan. One large study done in 1996 found that ten years after having the Fontan, the cumulative chances of having PLE are 13.4%. A 2003 study concludes PLE could be triggered by an infection. This leads to the disturbing thought that maybe all single ventricle patients are predisposed to PLE, and the Fontan Procedure coupled with an infection is the “trigger” that sets it off.

Someone, somewhere, had the Fontan Procedure today… and despite the drawbacks of the operation, they will probably have a better life because of it. As a 2008 report concludes, “this imperfect circulation would still be the only surgical option for this difficult patient population.”

When the test doesn’t work

August 3, 2010

A recent clinical trial showed disappointing results: Enalapril, used to treat high blood pressure and heart failure, was being tested in infants born with a single ventricle heart. The basis of the trial was that since the drug works in adults, perhaps it will also work in children.

It didn’t work at all.  When the results were analyzed, the outcomes for Enalapril group and the Control group were  practically identical. The results of this randomized trial do not support the routine use of enalapril in this population, the researchers wrote.

Did the drug fail? Certainly, it did not produce the desired result. But the clinical trial shouldn’t be considered a failure – after all, now doctors know what not to do. They may not have any better answers, but now they know that prescribing Enalapril in these circumstances won’t work. And sometimes you have to learn what not to do before you figure out what you should do.

Cardiology is littered with the relics of operations, drugs, and procedures that were abandoned in favor of something better. As I mentioned in my presentation in Houston, I had the Blalock-Hanlon procedure during my first surgery in 1967. At that time it was done regularly – the only way to enlarge or create an Atrial Septal Defect was to open the Right Atrium and use a scalpel or a probe to literally poke a hole. Today the operation has practically been retired, since so much can be done with Catheters. Today’s Heart Parents have never heard of the Waterston Shunt or the Potts Shunt – both operations were dropped when someone thought of a better way.

When someone tells you they have had the Fontan, you should ask them when they had it… it has evolved over the years, and each version has good points and bad points. Protein-Losing Enteropathy (PLE) doesn’t seem to affect anyone except Fontan Survivors, and not all of them. And one day scientists will figure out PLE, or the Fontan will be altered again…. or a better procedure will replace it.

And who knows? Perhaps at a Congenital Heart Defect (CHD) Conference in the year 2041, someone will ask “You had the Fontan? What’s that?”

Three Ghosts

July 14, 2010

The Ghost of Heart Defects past….

I am beginning to feel a little better about him now, but he is still on the critical list….He will have to be operated on again, but unless it is a case of having to have the surgery as soon as possible, it should be several years. We have been here a long time and it seems like we have a long time to go. Keep writing when you get a chance because it does us good to get mail from home. (Copied from a letter to my grandparents from my parents, February, 1967)

The Ghost of Heart Defects present….

Things with Isaac’s recovery have gone very well. In April, his cardiologist told us that Isaac was doing great and for the first time he told us that Isaac had “no restrictions”. I couldn’t believe it… I could go on all night raving about how wonderful he is, but the real point of this is to let all of the other heart families out there know that there is hope for your child as well. Isaac only uses 1/2 of his heart since his Fontan and yet no one even knows he is sick. (Copied with permission from the blog of a Heart Mom, July 2010)

The Ghost of Heart Defects yet to come…

It was amazing, Dad. Little Freddy was so blue when he came out. They slapped him on the bottom and he didn’t really cry, he just gasped for breath. They strapped him down to this little board – strapped him down so tight that he couldn’t move – and the first thing they did was jab a needle right into his chest. That made him pretty mad, and it didn’t do me any good either. If that wasn’t bad enough, they followed that up with another injection, and they put Freddy’s umbilical cord in a sterile container and one doctor left the room with it. They told me later that the first injection was stem cells, and they injected them directly into the heart. The next injection was either genes or entire cells, I’m not sure which, but the doctor called it “NRG1”. They say that the stem cells will repair his heart very slowly, and the NRG1 will help speed things up. They say that Freddy is going to need a heart valve, so they took his umbilical cord. They will harvest the stem cells there and build one in the lab for him. And when they replace it, the doctors won’t cut him open – they will put the valve on the end of a little tube, and push that tube through his blood vessels to his heart. Then they will just pop it into place. (E-mail that will hopefully be sent at some time in the future; all of these technologies are being developed today.)

Late Isaac Update!

April 3, 2010

If all continues to go well, Isaac very well could come home tomorrow!

Hmm… since he’s a patient at Duke, I wonder if Coach K would let him play in the National Championship Game Monday night? (Assuming Duke wins tonight, of course!)

Probably a little soon for that!

Experience

January 14, 2010

All the families – including mine – were gathered in a small conference room just down the hall from Surgical Waiting. Surgeon Albert Pacifico flipped through a handful of index cards; consulting them to refresh him memory. Pacifico was fast and he had a system; he often completed six heart operations or more in a single day.

“I will speak to you last,” he said to my parents. My mother gasped and began to cry.

“That’s not the way we handle that,” the surgeon’s assistant said, but there was no reassurance. Something was up, they could tell.

Finally it was their turn. There was scar tissue in my chest, a lot of scarring. More than had been anticipated. They had unintentionally torn it making the incision and suddenly blood was everywhere. Pacifico had used every trick he knew – and even made up a few new ones on the spot – and I had needed twenty units of blood, but things were stable at the moment. If we chose to go ahead, he would continue the operation.

It was what he didn’t say that hung in the room. If we go ahead, we could easily tear something else and it could happen again. And we may not be able to stop the bleeding this time.

Just because Daddy told him to call it off didn’t mean it would be any easier. Everything in my chest was being held together with baling wire and bubble gum; he had to make the repairs more permanent and leave the operative field in some type of order for the next surgeon, if anyone ever tried again. Although it would be a good 24 hours before I turned the corner and started getting better, I made it through.

The selection of a surgeon is the most important thing when going into heart surgery. Every surgeon is good, he or she wouldn’t be holding the knife if they weren’t. But when you are talking about operating on an infant with a heart the size of a plum and blood vessels measured in millimeters, “good” just doesn’t exist. This is working at a level that is well beyond the capacities of the average person.

So how do you pick the right surgeon? There’s a good rule of thumb that I often quote: The doctor you need does not practice in a town of 5,000 people.

That’s right – if you live in a smaller town (my hometown has less than 500 people!) you aren’t going to find the doctor that you need. You can make a quick determination just by observing the doctor on the first examination. If they are thrilled to see your child because “They are just so unusual!”… grab your kid’s arm and RUN. You’re in the wrong place. What you need is a doctor who has seen enough heart defects that they are almost boring. And those doctors work in prominent medical centers that see a lot of patients. Ask how many operations they do every year on children like yours, and the higher the number, the better. Post-operative care is just as important as the surgery itself, maybe more so.

(There’s always an exception: If you are at a hospital connected with a medical school, your physician may bring several of his students around to examine your child and learn about their bad heart. Medical students are a good thing; they may remember your child and use what they learned to save a life later in their career!)

Not only do you want a large hospital with lots of surgical experience and a surgeon who is experienced, you want the right type of surgeon. The doctor who did granny’s triple bypass is not who you are looking for. Rarely does a bypass specialist go digging around in the heart, you want a Congenital Heart Surgeon. Adults who need surgery for a Congenital Heart Defect may be surprised (and worried!) to learn that the best person to do their operation is a pediatric surgeon, but most Congenital Cardiac Surgery is performed on children.

You can’t control everything, and you may have to make some snap decisions, but following these simple rules (Major Medical Center, lots of operative experience, surgeon with lots of Congenital Heart Surgery experience) can tilt the odds in your favor.