Posts Tagged ‘single ventricle’

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.

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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.”