Fontan circulation: success or failure?

In this paper by Mondesert et al. (2013, Canadian Journal of Cardiology) critically review the putative success of the Fontan procedure that is commonly used to treat children (and adults) with complex congenital heart defects such as hypoplastic right or left heart syndrome and so on. This is a fantastic review and opinion by these Canadian researchers that highlights the difficulties associated with the Fontan circulation and discusses avenues for future research. What I found most revealing is that they highlight the “bad news” or potential complications, which is a big contrast to our experience with pediatric cardiologists or cardiothoracic surgeons that sometimes highlight only the “good news” of the Fontan procedure. Success can favor those who are prepared and it is important to be aware of these potential complications resulting from the Fontan circulation and also emphasize the need for more research in treating congenital heart defects.

I also think that this paper has a great overview (with diagrams) of the Fontan circulation showing the different variations of the Fontan procedure: 1) modified classic version, 2) intracardiac lateral tunnel, and 3) extracardiac tunnel.

Here are some interesting points from this paper:

1) The Fontan does not cure patients with congenital heart defects. The Fontan procedure was first introduced in 1971 and it is obviously a life saving procedure that many children (and parents) have benefited from. However, as you would suspect given that the Fontan procedure is a type of “palliative care”, it doesn’t “cure” or “fix” the congenital heart defect. You might almost view it as a bridge to another surgery or even another health complication. However, it is quite amazing that the current rate of death around the time of the Fontan (soon after) is <2%.

2) Those with the Fontan circulation do not have ‘normal’ heart physiology or functioning. Two major complications that might have many “downstream” effects are the following effects on increasing (“hypertension”) and decreasing (“hypotension”) blood pressure depending upon its location (veins or arteries). First, with Fontan circulation, there is “systemic venous hypertension”, which means that the blood pressure in the veins (blood going back to the heart) in the body is higher than in individuals with normal heart function (not Fontan circulation). There are many negative consequences that may be caused by systemic venous hypertension (congestive heart failure, edema or swelling, dysfunction of the liver, potentially protein-losing enteropathy) that are basically related to the distribution of fluids in the body. A second complication is “pulmonary arterial hypotension” where the blood pressure in arteries going towards or in the lungs (hence pulmonary) is lower than in individuals with normal heart function. There are also a number of negative consequences associated with pulmonary arterial hypotension such as cyanosis (blue lips!) or lack of exercise capacity. As this paper summarizes, many of the subsequent medical conditions and deaths that follow the Fontan procedure (either in the short- or long-term) are thought to originate from this change in systemic and pulmonary blood pressure.

3) Patients that undergo the Fontan procedure have limited lifespans (enjoy them now). In those that survive the Fontan procedure (and probably released from the hospital), 90% survive or do not need a heart transplant 10 years post-Fontan, whereas these numbers drop to 83% 20 years post-Fontan, and 70% 25 years post-Fontan. This study declares that one type of Fontan (i.e., modified classic, intracardiac lateral tunnel, or extracardiac tunnel) has not been associated with higher survival or success of patients. But surely because some of these techniques have been used for varying amounts of time we need more study to confirm this. All of this information about the causes of death following the Fontan procedure is reported in Khairy et al. (2008, Circulation 2008;117:85-92)

4) Individuals that had the Fontan procedure most often die from heart failure, stroke (thrombosis), or some unexplained sudden death. Of note is the fact that the risk of death from heart failure is quite low within 10 years of the Fontan procedure but increases with time after 10 years post-Fontan.

5) A few risk factors associated with increased risk of death or need for heart transplant are highlighted. A) One risk factor that seems to be important in predicting death or need for a heart transplant is the development of atrial tachycarrhythmias (meaning some heart rhythm problem where the heart is beating too fast), patients with these rhythm problems may have a higher risk of death. B) Second, the risk of death from a stroke or thrombosis increases dramatically 15 years post-Fontan yet the use of antiplatlet or anticoagulation therapy (asprin or warfarin, respectively) was associated with a decrease in the risk of death from stroke. C) Patients that developed protein-losing enteropathy, only had the right ventricle (e.g., hypoplastic left heart syndrome), or had increase blood pressures had a higher risk of death.

6) Not surprisingly, as time passes from the date of the Fontan procedure, the risk of death or need from a heart transplant increases. The authors note that this could be from some sudden death or heart failure, but it could also be from a gradual decline in heart function that is hard to document. Obtaining detailed health profiles from patients with Fontan circulation over their lifetime might help reveal early indicators associated with a heightened risk of death later in life.

How does the Fontan fail? The authors describe and summarize three different ways or categories in which the Fontan can “fail” (that is the patient dies or needs a heart transplant). Here is a summary:

1) The first category that they use is “ventricular dysfunction” where the ejection of the blood out of the heart (remember, ventricles eject blood!) or the blood pressure inside the heart becomes abnormal. This maybe is not surprising given that patients with the Fontan circulation have only one ventricle that is doing the work of two. This is a good reason why heart catheter procedures are performed so that these blood pressures inside the heart can be documented. One way to treat such ventricular dysfunctions is through drugs such as enalapril that can affect ventricular function. However, these authors indicate that benefits for administering enalapril are not entirely clear and they may actually depend upon whether the patient has hypoplastic right or left heart. A more recent drug that has been proposed to treat this ventricular dysfunction is sildenafil (viagra). In trials with adults that had heart failure and children that had the Fontan procedure, administering sildenafil increased some measures of ventricular performance. However, more studies are needed here.

2) The second category in which the Fontan can fail is through “systemic complications”, which are those where the altered Fontan circulation affects other parts of the body negatively. i) First, patients that need the Fontan often have low oxygen saturations in their blood (cyanosis) and this can persist even after the Fontan. In particular, in fenestrated Fontan procedure where a small hole is made in the patch of material put inside the heart to alter circulation (which can increase survival soon after the Fontan), oxygen saturations may remain lower for some time after the Fontan. This chronic cyanosis could have other pervasive effects on the body. The authors suggest that surgical intervention to close the fenestration might be necessary in some cases. It does seem like much more work about the long-term complications of the fenestrated Fontan are needed.

ii) Because of the altered Fontan circulation, many patients develop liver (hepatic) dysfunction. As was indicated above, patients with Fontan circulation have systemic venous hypertension and what a few studies have found summarized in this paper is that patients with Fontan circulation have venous pressures in or near their liver that may be 3-4 times higher than normal. Over the long-term, this hepatic dysfunction may have some major negative consequences but this is far from understood. However, in the autopsy of patients with Fontan circulation, many of them show signs of liver cirrhosis or other characteristics associated with liver dysfunction. In many cases, the authors indicate, the negative effects of hepatic dysfunction (which start right after the Fontan) are not apparent until 10 years after the Fontan. One future area of research is to identify early signs of hepatic dysfunction potentially using non-invasive measures such as levels of particular metabolites in the blood ore presumably also in the urine or feces. Unfortunately, there is as of yet no real promising avenues to treat hepatic dysfunction in patients with Fontan circulation. However, the authors do indicate that drugs that lower systemic venous pressure could be beneficial and also that combined heart and liver transplant is an option.

iii) Patients with Fontan circulation often die from stroke or thrombosis. This is likely caused by many factors such as changes in blood flow or even the placement of a synthetic material in the heart. Identifying all of the risk factors associated with death from thrombosis is another area of research. Interestingly, the authors state that another study shows that risk of death from thrombosis is similar for patients with the intra-cardiac lateral tunnel or extra-cardiac tunnel (Robbers-Visser et al., 2010 Eur J Cardiothorac Surg 2010;37:934-41) yet there is still no consensus on whether the use of anticoagulation (warfarin) or antiplatelet (asprin) substances are necessary (Canter 2011, J Am Coll Cardiol 2011;58:652-3).

iv) Protein-losing enteropathy has to be the worst diagnosis a patient with the Fontan circulation can receive. PLE essentially means that important proteins are lost and there are many swellings of particular areas of the body. The worst part about it is that it can come on very quick but it can also develop slowly.  The authors indicate that the most common early symptom of PLE is that a child complains their clothes or shoes don’t fit and sometimes they have diarrhea. Doctors than confirm PLE by finding low levels of protein or albumin in the blood and finally showing that a particular protein produced by the liver is excreted in the feces at a high concentration. Why it occurs in some patients with Fontan circulation but not others is not really clear but nonetheless 3-10% of patients with Fontan circulation develop PLE. The causes of PLE are not really known but the low oxygen saturations in the gut may underlie PLE. Treating PLE is complicated and include increasing cardiac output, administration of drugs that are anti-inflammatory (heparin, steroids), or a combination of budesonide (steroid) and sildenafil (again, viagra).

v) Similar to PLE, plastic bronchitis is also a scary but rare side-effect of the Fontan circulation (<1-2% of patients develop it). Airways become obstructed by debris and other substances and the linings of the lungs become inflamed and swell. The cause of plastic bronchitis is also unknown but might be similar to the cause of PLE. Treating plastic bronchitis is still largely experimental and involves drugs used for asthma (inhaled steroids) or mechanical clearing of the obstructions. Cardiac transplantation can also improve the outcome.

vi) Many patients have heart arrhythmias afther the Fontan procedure (the authors say up to 40% of patients). Normal sinus rhythm is disrupted by the Fontan procedure and a fast (tachyarrhythmias) or slow (bradyarrhythmias) can result. The obvious treatment for these heart rhythm problems is to implant a pacemaker or treat through other means such as ablation or drugs (for tachyarrhythmias). The authors indicate that the current consensus indicate that patients with an intra-cardiac lateral tunnel Fontan have a similar risk of developing an arrhythmia as patients with the extra-cardiac Fontan (cite a variety of studies especially Khairy and Poirier, 2012, Circulation 126,2516-2525). Patients that are older at their Fontan procedure tend to have a higher risk of developing heart rhythm problems. Somewhat scarily, atrial tachyarrhythmias may not be very obvious and may cause sudden death, so surveillance for these rhythm problems is important.

3) Now we come to the final category of how patients with the Fontan circulation can die. The final way that may be the most pernicious is through chronic deterioration. As the years tick by after the Fontan procedure, heart function gets worse, which is reflected in the decline in the ability to do aerobic exercise. For example, for patients that had the Fontan early in life, they may have exercise capacity that is highly reduced (44%) compared to normal patients and this capacity to do exercise tends to decline in a linear fashion each year (declines 2.6% each year). At thirty years of age, patients with Fontan circulation have much reduced exercise capacity (55% less than normal) and the number of health problems and hospitalization rates increase dramatically. This is probably not surprising since, again, one ventricle is doing the work of two. How could we increase or preserve cardiac function with age? One way is through drugs such as a recent study showing that children given sildenafil (viagra) for 6 weeks have improved exercise capacity compared to those not given the drug (Goldberg et al., 2011, Circulation 2011;123:1185-93). The obvious solutions are heart transplantation, but again, because the Fontan circulation has already wreaked havoc in the body such as negatively affecting hepatic or kidney function. So patients with Fontan circulation may still be in poor shape even after a heart transplant. The final, seemingly far-fetched, option is to use a mechanical device that assists the heart. As mentioned above, ventricular dysfunction increases with time since Fontan, which could cause chronic failure of the Fontan. One way to improve survival is to use mechanical devices (“Fontan assist device”) that assist blood flow out or into the heart. For example, mechanical devices that increase arterial blood pressure or decrease venous pressure. This is an interesting topic but the authors say that these devices hold promise but only in the distant future.

Link to this paper:

http://dx.doi.org/10.1016/j.cjca.2012.12.009

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35 thoughts on “Fontan circulation: success or failure?

  1. Pingback: Are ACE inhibitors like enalapril and captopril beneficial? | Congenital Heart Defect Research Blog

  2. Quite meaningless article since there are no other options today, all it does is to scare CHD Parents and does not bring anything…i.e. rubbish!

    • Dear Benny, Thanks for reading this and taking the time to comment. I can’t speak for the authors of this paper but the point of this blog is certainly not to scare CHD parents or assert that there are other viable options available for children with CHD. It is simply to make myself more informed/educated about the complications of CHD’s and the Fontan procedure in particular. The Fontan procedure is clearly not perfect and being more aware of the complications it creates might help us to recognize symptoms or complications earlier on. Treatment of CHD’s is certainly more nuanced than simply reaching the Fontan and being done with it. This is a great publication by these authors because it demonstrates that there is still a lot of work to be done in the treatment of CHD’s and they aren’t worried about challenging the status quo. Is it scary to know that the Fontan is not perfect as a CHD parent? Certainly but I personally would rather be informed about the risks.

  3. Hi,
    Am a survivor of a single-ventricle congenital defect and am leading a very normal life from the past many years.

    I understand that the paper just intends to put light on Fontan Complications. But as a grown up surviving the problem, I have done a lot of R & D regarding the issue.

    From the experience till now and the Lifestyle am leading and the R & D and my Doctor’s Opinion, I just feel that this paper is just focussing on the pitfalls and down falls o Fontan Procedure. I personally know that its a highly complex procedure but still there are people who survive and lead a very normal life.

    I just request that instead of focussing on the down falls of the Fontan procedure, Please even highlight the positivity of Fontan.

    All people live life just hoping for the best. It implies to both normal and special people like me. Please don’t think of Scaring people of CHDS. I am a grown-up, I understand the trueness of this hopeless Blog, So Its ok.
    But there may be parents who are in the phase of deciding whether there child should undergo Fontan or not. If they Encounter this Blog, they will step down.

    So dear parents, Please ignore this blog as Fontan is not a procedure which causes so many harnesses,as discussed here. And Now a days they are performing an advanced Fontan operation namely Fenestrated Fontan operations which in majority Eliminates the PLE occurance.

    According to the research which I have been doing on Fontan, Its stated that Most of the patients live normally after Fontan. All fail to assert the outcomes after 3 decades post Fontan due to the fact that after the Advancement of the Fontan, the people are still in there post 3 decades after Fontan.

    So,Kindly, please don’t consider this Article.

    • Dear Anamika, Thanks for reading this and for your great comment. You are an inspiration for all parents of young children with CHDs. I couldn’t agree with you more that the Fontan procedure is certainly NOT useless. I don’t think this was the point of this research article nor is it the point of the blog. The point is to highlight there is still a long way to go and we should be putting research money and time into figuring out how to fix these problems.

      The procedure is not perfect but knowing all of these negative aspects that this research paper highlights, I would never ever not have wanted the Fontan procedure to be performed on my daughter. I certainly don’t think that knowing the truth should discourage anyone from having their child undergo the Fontan. I certainly wouldn’t be discouraged as I see every day that children with the Fontan procedure can have happy and normal lives. Hearing from people like you also encourages me knowing that kids with CHDs can live happy and normal lives as adults.

      One note about your comment about the fenestrated Fontan. From what I understand, the fenestrated Fontan is almost always performed (routine in the US and UK) now and in the past 15-20 years. However, the fenestration is often allowed to naturally close or it is closed via a heart catheterization procedure within 1-2 years after the Fontan (at least in US and UK). Some research papers have now suggested that a recent treatment for PLE is to re-open the fenestration. This seems a bit confusing but if we expect medicine to follow the advice of scientific research, doctors and scientists are going to tinker with their methods until they figure out the ‘best’ one based upon current knowledge.

      • Thanks for your Reply. But I would certainly tell you to do more Research on more Articles based upon Fontan procedures. Coz PLE is not surely to be caused ONLY after Fontane. Its a diseasewhich pertains as a matter and NOT caused only by Fontan.

        And there is a statistics which tells that only 18 to 20% of the people who undergo Fontan will get into the complication of PLE. Its a fact and thats what even Doctors tell.

        And as per your statement that ” I would never ever not have wanted the Fontan procedure to be performed on my daughter”. As a matter of fact, some kids will be born with multiple heart ailments. Like me as well and Fontan will be the one shot solution for all the issues. I request you to be optimistic and even throw little hope in people who are having Ailments. Articles like this just make the heart beat fast and believe that there is no way beyond.

        And your Point of Fenestrated Fontan, I understand that its being done as a general practice and when the Fenestration closes, then the problems pertain. No, Its not so. Once the Fenestrated Fontan is done, then the occurance of PLE is rare.

        Don’t mistake my comments as discouraging, but the way you have written “After 10 years”, “After 20 years, time keeps ticking”. Am sorry to tell this, but even a common normal man, won’t be knowing what lies after 25 years.

        You put the Advantages v/s Disadvantages,no problem. But please don’t discourage people.

        Thats my only request. Thanks

  4. Dear Anamika, I certainly don’t think that I am discouraging people. I am just summarizing my notes on their article.

    I just want to reiterate that I certainly WOULD approve of having the Fontan procedure on my daughter again even after knowing of its disadvantages. I would always make that decision again and I am so happy that she did have the Fontan procedure.

    Thanks again for your comments here. If you have a reference for the statement that “Once the Fenestrated Fontan is done, then the occurance of PLE is rare.”, I would love to hear about it. That is what I had hoped that this blog could become. A place where information could be shared! So thanks again.

    • Its not about discouraging people. But the way you have summarised it just puts light on the Pitfalls of Fontan Procedure. In the morning when I encountered the blog, I just put myself in my parent’s position and assumed that what my mom would have felt. So I kept saying, Discouraging.

      Well, Even I have researched a lot about the Fontan procedure as I myself have to live life with it. There are some research papers which tells that once if BDG, Fontan is done, that means you can lead a normal life and your heart will be like a new heart.

      I have consulted renowned doctors available in the country and they have all said that a person with Fontan and Glen can lead a Normal life. All the pitfalls you have mentioned are not always occuring. Only if the Lungs pressure is very high, during Fontan surgery only then these situations occur. But all of these pitfalls mentioned above have treatment. You can do research and find out.

      When the lung pressure is within 15 during the fontan procedure, then those people are termed as Good fontanes and can lead a normal life. If its more than that they perform a band operation due to which the pressure decreases and then the Fontan is performed.

      More than all of the above things, I feel that the Strong hope and Positivity in life will keep things going well. I hope the blogs which you post from now will enforce Hope and positivity in CHDS.

      If you need the reference of all of the above of my statements, pl. give me your ID. I will mail you the materials and further share info in a NEW HOPEFUL Blog.

      Thanks for your time 🙂

      • Kindly send me the references – we have a daughter who had a fontan Extracardiac TCPC without a window & now has PLE resulting in massive ascites due to high pressure in lungs causing low albumin , She has not increased in height & has moderate mitral valve regurgitation. They now want to do a heart transplant.
        Is it too late for a window to be put into the extracardiac conduit???
        & will a band operation help to get her lung pressures below 15???
        Kindly em advice.clinic@yahoo.com

      • Dear Adam,

        I have tried to send you these papers but the email address isn’t working.

        Thanks for reading the blog and your comment. I am so sorry to hear about your daughter. That is one of my worst fears and the reason why I started to get interested into this research about congenital heart defects.

        Unfortunately I can’t offer any medical advice because I am definitely not qualified to do so. Have you asked the doctors about these issues? I wonder if you brought in these papers and asked them their opinion what they would think. What hospital are you at (if in US)? I think if I was in your situation I would try to get other opinions. In particular with the children’s hospital at Boston University.

        Again, I’m so sorry to hear about your daughter and I wish you and your family the best.

  5. As a Fontan survivor of 27 years thank you for the article! I am blessed to live a “healthy” life, as for having one working ventricle will let you! I have an enlarged liver right now, and am being tested for PLE, also I have a pacemaker and have since 9 years old! I’m 31 years old, engaged, live with my fiance and our dog, and totally have a normal life, except I have had to stop working but I started back at college full time online instead of just sitting around. The reason I’m not angry about this blog is it does pertain to me, to what is going on right now in my life, would I change things and not be alive, of course not! I would have taken better care of myself if I was informed of all this though, see the above attitudes you see are those who think like I used to. That nothing would happen, that I was normal, and while I am in a great sense, I would have put more focus on taking care of myself. I would have eaten better, slept more, understood the importance of never missing medication, realized that stress was something I needed to cope with, cried less, laughed more … and while I am not in the position of needing hospitalization, or surgery, or anything right now … it can come … if I make it there …

    What I want to thank you for is your honesty … and what I want to express to anyone who reads this and gets angry is please don’t … education is key to surviving a long time, so while you don’t need to focus on it, keep it there in your mind so you know your limitations, trust me I pushed mine! I shouldn’t have and wouldn’t have the past I have if I would have realized this is not just “the surgery that gives you a normal life” because this is the surgery you have to care for, consider, and understand … so when you see symptoms of these things you can alert your cardiologist, how many of us have had symptoms of PLE most of our lives and blamed the medications, or knew we were swelling when eating high sodium and ignored it, or smoked a cigg (even on SOA the mother with CHD smoked!), the older generation did not get told all this, so please know we need this information. Don’t be angry, you are now informed and we are all praying it doesn’t happen … I wish I could have known more than the positives … it would not have shocked me so much when the negatives showed up!

    • Thanks for reading and for your inspiring comments Erin. You are right that our parents did not know things children with CHD’s are “not supposed to do” (the same is true for children without CHD’s!). Hopefully we can change some of those things and help kids with CHD live a longer and healthier life.

    • My son is a 33 year old who had fontan and fontan revision now has developed ple he was placed on ecmo but it clotted off causing some small strokes I need help any more information out there related to this ple please let me know u can email me tammysoardhelton@yahoo.com. any help would be appreciatted. Specialist. Or treatments

  6. Just want to mention I’m a parent of a 9 year old who had his Fontan more than 5 years ago and so far he’s doing fine, but as a parent not one day goes by without me checking his conditions, i.e. heart beat, edemas, skin, etc, and constantly worrying about tomorrow – the reason for this is articles like this. The stress on the parents is enormous!

    Yes Fontan has its issues but as long as there is no other options people must be careful about certain statements. And what troublesome is that very little is being done regarding alternatives to Fontan and very little is being done regarding treatment & solutions for PLE & plastic bronchitis which is truly disturbing.

  7. Hi
    Thank you for your blog. As a Mom to a 16 year old boy with HLHS/Fontan and also a Nurse Practitioner I appreciate your commitment to looking at how we can enhance long term results for people with Fontans. I too, find some overly “optimistic” providors. I am not pessimistic but want to be as vigilant as I can be with my son’s health to prevent future complications in an unknown state.
    Thank you again,
    Christine Ouellette

    • Hi Christine – Thank you for your comment and for reading. That is very encouraging to know about your 16 year old with HLHS. I completely agree with you about being vigilant and we are not pessimistic. Becoming pessimistic about the future of our heart kids is a recipe for failure. We are optimistic about the future of our daughter but we think new approaches to old problems may sometimes be needed. So we need more research about any alternative surgical procedures compared to the Fontan or how to mitigate the negative consequences of the Fontan before it is too late.

      Ben

  8. My son is 24 and lives on disability with the added problem of not being able to attend college. It is hard to watch him struggle physically with low 02 sats. He was 92% until about 18 and then dropped and is now 83% at rest. We were referred for heart transplant, but found that his antibodies are too high and rejection would be over 90%. Sildenifil has been a help and he has been on that for almost 2 years. I truly wish we had been more proactive during teen years. He has holes in the baffle due to high pressures (I always wondered what would happen as he grew since the Fontan is done at 2 years of age and he is so much bigger now).
    It is very important to really ask the tough questions from the very beginning. There may be advances that work for the new younger patients, but I had always hoped that would be the case for my son. Yet, he turns 25 next month and we have no solutions at hand. To say this is difficult for all of us is like saying it is chilly in the middle of a blizzard. We are taking it all one day at a time. I just wished for a much better life for him.

  9. Hello, I am a CHD survivor at 28. I had the Fontan at 2 years old and had been doing great. I had my first child this past year, via c-section, and a week later. Developed a 7-inch pulmonary embolism that once again required open-chest surgery. This was hard for me, not only physically, but emotionally and mentally as I was away from my newborn for 3 weeks and took care of her while recovering- not easy when your sternum has been sawed open.
    I’m doing much better now, and still beating odds. Statistics have always been against me, but I have overcome numbers with God’s help. Having a child was a risk, but I did it. A 7-inch PE should have taken me down, but I took it down.
    Just some encouragement for those who fear the numbers. God ultimately has control of my life, and that is all that matters to me.

  10. My daughter had lots of the above complications including PLE which she died from last June at the age of 21. The Fontan is not a fix .She enjoyed 6 good years but PLE showed up at the age of 7 and dogged her till she died. She experienced 2 strokes at the age of 10. Liver fibrosis, renal impairment, tense ascites. She didn’t grow, or go into puberty. She suffered a lot. We tried for transplant but she wasn’t well enough. The Fontan was our only choice when she was born and still would be her only choice. I feel like we weren’t given much information when she was born about the Fontan surgery and it’s complications. But I would choose it again because it did give her 2 decades with us. Her heartbroken mother

    • I am very sorry to hear about your daughter. I would absolutely agree that Fontan is the only choice and that we would choose it again. However, I think that doesn’t mean we shouldn’t know the adverse consequences (so that we could be more vigilant for them) and that we should be seeking ways to improve treatment of the problems.

      • I totally agree that we should know the problems with the Fontan. In 1993 when she was born we were told nothing but I guess we were still pioneers .

      • After 19 years at another hospital in Pennsylvania we finally took my daughter to see Dr Rychik at CHOP. She was seen in the single ventricle survivorship clinic which is the most comprehensive care she could have gotten. Of course with her advanced PLE it was many years too late. But Dr Rychik is an expert in post Fontan problems especially PLE. This is the kind of care she needed 20 years before but it wasn’t available then. I would point people with single ventricles to at least consult down there if they are having problems and even better before they have problems.

  11. I really appreciate your blog! Very much. 9 year old son is 5 yr . post fontan. Each time he has problems we go through rigamarole with pediatrician . his oxygensats going down, chest pain, headaches. Cardiologist restricted exercise a year ago and this seemed to let our boy grow. He’s @ 91% resting and well. Today we got treated like fat smokers at the pediatrician. Major motor is beyond him. He does start to run, can’t go far and very blue, but he’s very active.nver stops until outright illness forces him. But peds. Doc says he needs
    To exercise more. Cardiologist says “buck up” @ chest pain, peds . doc says we must have stress, need therapy, and chides my son to tell the “truth” about headaches and blurry vision. The rest of America can stream into the emergency room at every twinge, heart attack warnings in every magazine, but our brave boy has to suck it up, and we are asked if there is stress in our house as if it is an indictment…..oh no! No stress here. Sorry for venting…I appreciate the figging you’ve done. Thanks again!

  12. Hi Katie
    This is from a Mom of a 17 year old boy with HLHS Fontan (fontan at age 2) and I am a Nurse Practitioner (coincidentally I work in cardiology). Doesn’t sound like you’re having an easy time of it. If he hasn’t had recent stress testing and /or cardiac cath and/or cardiac MRI maybe he needs some testing to see what is going on.
    As far as activity – when my son was little I never limited him. I just said – take a break if your tired.
    Good luck – not always easy.

  13. My grandson is 22 and had a Fontan procedure. He has been doing very well since then wil O2 levels 96. He wants to move to Denver. Does anyone have experience with high altitude and this condition. Currently we reside in the Virgin Islands, at sea level and I worry that this would stress his heart. Also, he had a fenestration procedure at twelve years of age and his most recent echo showed that that closure is no longer there. What does that mean? I understand the fenestration procedure is controversial. Md in Puerto Rico says do nothing since he is doing well. On no meds other than aspirin. Exercise tolerance is good.

  14. Thank you for the article. I am a 47 year old woman born with double inlet left ventricle. Went through my entire childhood with O2 sats in the high 50’s, low 60’s. I received a lateral tunnel fontan procedure AFTER my first child was born when I was 22 years old. Already an adult having a “paediatric surgery”. I had my surgery at Toronto General Hospital in Ontario, Canada. They were going to do a hemi-fontan with fenestration, but while in surgery they decided to complete the full fontan and did not fenestrate. They put the leads for a pacemaker in just in case I got into trouble post surgery. (I did not).

    Just to give parents and other fontan patients out there a little bit of hope, just because all of these are potential complications, does not necessarily mean they will develop. I am 23 years post-fontan with none of these symptoms. My oxygen sats run at about 94%, I do have a decreased exercise tolerance, however it it 100% better than it was pre-fontan. I went on to have two more children post-fontan.

    My only hiccup so far was developing pulmonary emboli after the c-section birth of my son and abdominal surgery to repair a hernia 13 years ago. This was treated with warfarin. I continue warfarin therapy daily simply due to the “sluggish” fontan circulation.

    I have no arrhythmia’s, no PLE, no need for the pacemaker yet. They are beginning to check my liver – but the liver specialist is not convinced there is any cirrhosis, because I am simply not having any symptoms.

    I lead a fairly normal life. I quit the high stress job in the corporate world after my son was born, and just work from home now. My cardiologist thought it best for me as the responsibility of maintaining a household with 3 kids was more than enough without the high stress job. So that was me taking care of me. I try to stay as active as I can and I try to eat well.

    The fontan procedure gave me life. Gave me the chance to live and see my daughter grow up. She was just 1 year old when I had the surgery. She is now 24. I will never regret my decision to have the surgery. Am I an anomaly? I hope not. I would love to hear from other patients out there that have had good results as well.

    We have to deal with what life gives us. All we can do is trust and have faith. Blessings to all of you out there with fontan’s or parents of fontan patients.

  15. When I read this blog first it was like ..heart beats faster , worrying about the future of my daughter who done with Fontan last December . I am happy with the results seeing the O2 saturation 94%. And reading Kim’s story is such an inspiration. True we have to accept as the life comes with , not always thinking about the downside of it. Always try to find the positive side . And who know how much science and medicine will develop in next decade. Surely we will find better solution to this problems.
    When I see my daughter with full positive attitude towards everything in life after she gone through so much at such tender age .. that is the essence to life to me.

    • I agree – worrying will always be with us for our kids with CHDs. We do have to accept it but the way I cope is trying to be prepared and ready with new knowledge or techniques. Best of luck.

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