Pediatric Heart Expert Forum
Life after Fontan
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Life after Fontan

Dear Doctor, my son with HLHS (almost 6 years of age) has underwent 3 steps of Fontan procedure (extracardial, with fenestration). My concern is that saturation is still 87-88, fenestration is open, bus we live without loosing protein. Is there a threat that as the child grows, the saturation will decrease if this fenestration is left open (our doctors do not speak of closing it)? What further tests must be performed (catetherisation, MRT), excl. ultrasound which we do every 4 months? And is 50% of right ventricle fraction sufficient? Should pressure in lungs be constantly observed, or it is no more relevant as the fenestration does its work?  What is life expectancy in your country of those having HLHS condition? (We are from Lithuania, Central Europe, that's why it is interesting for us). Thank you in advance.

Neringa
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Dear Neringa,

Patients with hypoplastic left heart syndrome (HLHS) who have undergone a fenestrated extracardiac Fontan do have a unique set of issues and concerns over their lifespan.  I cannot say what the lifespan is for HLHS patients, as there is great variation in their outcomes.  Overall, if there are absolutely no complications at all, they certainly are making it into middle age, but there are eventual problems with right ventricular failure as well as acquired liver disease.

With regard to the fenestration, that was put in place to allow for any elevation in right sided pressure to decompress across to the left side.  If your child is growing well, appropriately active, and does not have protein-losing enteropathy, it sounds like the fenestrated Fontan is doing relatively well.  The lower saturations are not ideal, but there is a small chance that they will increase over time.  If that happens, the fenestration may be closed.

I am not sure what you are asking about with the "50% right ventricle fraction."  If you mean that his RV ejection fraction is 50% (or even his shortening fraction), I would say that I'm not sure that I would trust that number.  The assumptions for calculating a left ventricular ejection fraction cannot be used for the right ventricle, because its shape is very different and it is hard to assess.  I would not get concerned about the actual number in this case, either--experienced echocardiographers can look at the function and qualitatively assess how good it is squeezing fairly accurately.  If your cardiologist says that the function appears normal, that is probably more accurate and helpful than any number.

We typically do not routinely perform catheterizations or other testing outside of echocardiography in patients with Fontans unless there is an indication, such as obstruction of the aortic arch or pulmonary arteries, or evidence of elevated right sided pressures, such as an enlarged liver, protein-losing enteropathy, or worsening saturations.  Typically, at this stage, a good history and examination, an ECG, echocardiography, and occasional Holter monitoring and exercise stress testing are sufficient
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My daughter who is 4 has had the fontan six months ago and has. Been having a lot of chest pains at least three times a day which also include pain in arms both sides of chest and back area. Her heart doctor has gave her an ekg. Blood work and an echo and x ray all have been normal do u have an idea what else could be causing her pains. Her oxygen has been great to us 85 to 96 on average.
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
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