A disturbing report

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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7 Responses to “A disturbing report”

  1. Amy B. Says:

    Great Article Steve!! It is thrilling to see that we are moving beyond “merely surviving” to thriving and living longer lives.

    I am hearing more an more stories about ped’s cardiologists exploring alternatives to the Fontan with their patients.

    Also very exciting that research is looking very promising for the pump that you mention to help reduce the risk of PLE.

    Support research…. support research!!!!

  2. Terri Grabb Says:

    Six years ago we walked into a cardiologist office and heard stories of hope and bright futures for children with HLHS. They glossed over the hard parts and made it seem like they had conquered the difficulties of this horrid disease. We had no idea. Our son after 9 open heart surgeries a failed Fontan surgery died like many others. Our hospital was not a small un-known hospital with just a few attempted surgeries under its belt. It was University of Michigan, where it sees hundreds of patients monthly with Dr. Bove.

    I don’t regret our decision for one minute to choose life and hope. I would go through those years all over again but its time for research to begin and thrive again for our littlest heart warriors to our oldest survivors. We need to keep moving forward so no family has to say goodbye due to a failed surgical attempt.

    Terri Grabb
    Mom to angel Corbin Marc Grabb

  3. Lisa Says:

    And THIS is why we need to fund research!!! We’ve got to keep improving!

  4. Amanda Says:

    Um, Steve . . . you met at least twelve people who’ve had the Fontan when you were in Texas! 🙂 Or did you mean big people? Then I guess it was just Ana and Michael there. But most of the kids were post-Fontan who attended and only a couple aren’t on the path.

    We have literally hundreds of Fontanians on either side of the line in Hypoplastic Right Hearts. Dr. Fraser was very transparent about many of the issues brought up in his presentation, and I’m so sorry (for many reasons) that you weren’t able to hear that.

    I think that’s why our group is SO important. We had a member join just yesterday have very similar complications to those Liam had. Not all hypoplasts (as you well know) are ideal Fontan candidates, but it seems as though only the most healthy who might have some sort of valve repair or vent growth have an option other than Fontan or transplant.

    I am not afraid of the report for two reasons – 1. We cross that bridge when we come to it and 2. they keep rebuilding the bridge. 2 is a curse and a blessing because you really can’t unilaterally apply what you learn about one cohort to another. They’ve changed the Fontan so many times, and are still changing it based on what they’ve learned, that the complications of one generation may or may not be the same complications we’ll see in the next.

    We need to know, and parents who don’t want to know frustrate me because if we’re not diligent and teach our kids to be the same, who will? I’m glad this report exists, it leads us to ask informed questions. Besides, after what we’ve already been through it takes a lot more than one pdf to scare me – I’m a heart mom.

    Walk on that Water!

    • Steve Says:

      I was thinking about the “big people” I know, Amanda. 🙂 And I am sorry I missed Dr. Fraser, too! (For those of you who may not know, I fell and broke my wrist shortly after arriving in Houston for the CHD Conference sponsored by Hypoplastic Right Hearts. After returning to the hotel from the ER I stayed in my room all day Friday to rest and recover. Dr. Frasier was one of the speakers I missed.)

  5. Phizz Says:

    I’m a parent of a ten year old boy with PLE following the Fontan and live in England . Just back from a 5 day coast to coast USA trip looking at research into PLE and talked to Dr Rychik for a couple of hours.

    He’s looking to start a “Single Ventricle Survivorship” program at Childrens Hospital of Philadelphia where Fontan patients would have gut and bone exams to find problems early. As he said, “It’s not enough to save lives we have to keep them healthy.”

    The negative language is to attract the attention of other Medical Professionals to fix this problem. I agree with Amanda, survival figures are always historic so we don’t know what it means for this generation. It makes sense to hope!

  6. Leasha Says:

    Hi, I’m 30 (in two weeks) and I’m a fontan survivor from the UK. I’ve just read this report and didn’t realise how serious the op was (mine was done in 1983).
    My survival comes from NOT reading these kind of things, they are negative and should be ignored unless you’re in the medical profession!

    I live my life as any other person would, I drink, I’m a light smoker and I live my life believe me!!!

    I only know of 2 out of 8 defects that I have, those being DILV and PS (no idea what the others are). I take warfarin and have yearly check ups. My naïveté has brought me this far and will take me further (maybe I’ll be the longest fontan survivor one day with this attitude).

    All I’m trying to say is don’t read to much into it!! I was supposed to die…and I didn’t! I read recently that a child doesn’t know statistics and that is probably why they fight and combat diseases and cancers better than adults. If I knew or my parents let on about stats I may not have made it all them years ago…positivity is the way forward!

    I can’t fault the work the medical professionals do into improving this procedure and I would recommend it if needed, just don’t read to much into it on the Internet because this alone is enough to make your heart pump faster.

    My advice is to focus on what survivors have to say, not what the stats report 😉

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