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.