The Fontan

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.”

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2 Responses to “The Fontan”

  1. Pam Says:

    Steve,
    I can’t believe that we are now post-Fontan! Zach had his done July 12 by Dr. Bradley and is doing super! We are still reminding him not to wrestle his big sister or do flips off the couch! His sats are currently 90-92 and that could go up when he has his fenestration closed. He was a bundle of energy before the Fontan, so I don’t know what I’m going to do with him now! I think I need some sort of an operation to give me some more energy so I can keep up with him!
    We are blessed!
    Pam

  2. McCanless & Mary Clare Pennington » The Funky Heart Explains… Says:

    […] bit about the Fontan.  No wonder  I have trouble explaining what it is exactly.  Even the Funky Heart, who had the […]

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