Posts Tagged ‘Fontan Procedure’

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.”


We can fix that!

February 3, 2010

I keep writing about advances in cell therapy, cell transplantation, and cell replacement options that are coming in the future. You might think that this is all dream technology – the kind of ideas you have that always start with “Wouldn’t it be nice if…”

All this is exciting stuff, but it won’t be ready tomorrow. Medical research takes time – as much as you want something to work, you can’t rush it. I am sure that Heart Defect researchers know the percentages, they know that roughly 105 people per day are born with a Congenital Heart Defect (CHD) – but they also know that if they don’t get this right, they could inadvertently make a bad situation much worse. And sometimes “getting it right” means trial on human subjects. Early versions of the Fontan Procedure didn’t live up to expectations, but further research and study has made it into a better operation. Sometimes you just have to hold your breath and step into the unknown.

Since we don’t hear that much about medical research until something hits (or goes wrong), you may wonder who’s out there? How many companies are looking for answers? I can’t give you a total number, but a recent report, Tissue Engineering, Cell Therapy and Transplantation: Products, Technologies & Market Opportunities, Worldwide, 2009-2018, gives us some good information about tissue and cell research. The numbers are staggering: In the report, 148 companies are profiled (Click here for a list) and they account for a total of over $1.5 Billion US Dollars spent annually.


The report isn’t for “normal” people – it’s intended for corporations and foundations that might want to drop a spare million dollars or so into a research project but aren’t sure where to start.  (Of course, the only thing you need to obtain a copy of the report is $2,950!) Looking over the detailed table of contents is a real eye opener, it contains not only a list of companies working in the field but also worldwide research goals. There is not a research project titled “Limiting/Curing Congenital Heart Defects” listed anywhere, but CHD related projects are widespread. One of the major goals is combating Congestive Heart Failure (CHF) – Heart Failure often accompanies a CHD, and reducing or eliminating CHF will really cut down on some of the healthcare costs we all endure. More than half of my medication is designed to keep my CHF at bay. Another research goal is replacing heart valves, and a third is heart transplantation – issues that CHDers could very well have to deal with.

And that’s not all. Looking down the list of possible therapies, you notice that someone has a plan to replace or repair almost every body part! I’m hitting the “ol’ man” stage and my bones are beginning to creak, I’d like to have a talk with some of these people.

So take heart – we’re not out here alone.

Computer Assisted Fontan Surgery

December 4, 2009

I just don’t “get” the Fontan. At all.

I was recently asked by a CHD organization to write an article about the Fontan Procedure for their newsletter. I had to turn them down; I had to say that no matter how many diagrams I studied, and how many textbooks I have read, I just don’t see how that operation works. Thankfully there are people who are a lot smarter than I am who know how to explain it, and a few of them understand how to surgically alter a defective heart to make the Fontan work. Thank goodness for that, because if they didn’t, there would be a lot fewer of us around.

I was scheduled for a Fontan in 1988; that surgery went completely wrong and they barely got me off the table. I never got the Fontan, but many others from that time period have needed a Fontan Revision or Fontan Conversion. (After clicking the link, pay close attention to Section V.)

One of the difficulties in understanding lies in the fact that the Fontan has several variations, and is almost “customized” for the patient. Now there is a new computer simulation available to test various versions of the Fontan on a defective heart. The simulation will help determine blood flow patterns to help surgeons choose which variation of the Fontan works best for that patient.

The two researchers who developed the computer simulation have also designed a Y-shaped graft to be used as part of the Fontan. The Y connected will be used to join the Inferior Vena Cava (The major blood vessel leading to the heart from the lower part of the body) to both branches of the Pulmonary Artery, not just the single connection that is used now. According to the Abstract, the Y graft “is expected to be put into clinical use within a few months.” I am not sure if that means it is ready for clinical trials or has passed the trials and is ready for real world use.

Either way, these developments look promising!

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.

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.