child with chd

[Bm69]

My daughter was born at 35 weeks and was dx with tricuspid atresia and hypoplastic right ventricle.  6 days after she was born she underwent her first open heart surgery an aorto pulmonary window.   In the summer of 1999 she underwent her 2 sx the fontan. After the first surgery there  were some complications with supra ventricular tachycardias and respiratory complications both of her lungs collapsed for which she had to stay connected to a ventilator for a longer period of time.  Overall cardiac wise she is stable, respiratory wise there ups and downs.  On Feb/2011 she needed sx to remove her gallbladder during the sx the surgeon noticed some hardening of the liver.  She was then referred to a  liver specialist, during the consult the specialist told me that the liver was been affected because most likely the pressure inside the fontan circulation were high.  My daughter had a liver biopsy which showed liver fibrosis and also had a cardiac cath which confirmed the suspicion of the liver specialist it also showed some stenosis on the right pulmonary branch and a stent was placed.  On 7/12 my daughter had a liver US as a follow up.  The US showed  liver disease progression as well as narrowing in the IVC  adjacent to the liver.  How common is that children with the Fontan tents to have liver disease?  Is there any treatment?  In regards to the IVC is there any way to reverse the stenosis? Thank you for you opinion.;

[Marie M Gleason, M.D.]

Fenestrating a Fontan usually brings down the central filling pressures a bit, but they are still higher than in a normal person.  So that may still be enough to lead to liver changes.  Right now there is no known treatment to reverse cirrhosis.  This important area is being looked at and we are trying to come up with ideas of how to impact on this problem in the future. But we still need to get more information as to the prevalence of this problem, and the degree of liver abnormalities that are seen.

[Bm69]

How likely is that cardiac cirrhosis after the Fontan can be reverse? Is there any treatment  to prevent further damage to the liver?

[Bm69]

Thank you for your reply.    

There is also another area that I'm concerned about, my daughters liver enzymes are within normal range to me that is a good news, but knowing that she already has some sort of liver disease, how come it doesn't show yet on the labs.  Is it possible that the liver is not as damaged and that is why is not showing in the labs?  In your opinion do you think that by reopening the fenestration will help bring the down the pressures in the Fontana circulation?  I understand that her spo2 will be lower.

[Marie M Gleason, M.D.]

Your post brings up an important, relatively new concern for the older patients with Fontan operations.  In looking at the survivors in their early adult years, there have been findings of liver cirrhosis, which is likely due to higher than normal pressures in the liver over the course of a couple of decades.  All Fontan patients will have higher than normal pressures in the inferiro vena cava and liver compared to those of us with 2 pumping chambers, but even within the Fontan population, the pressures in the Fontan baffle can be quite variable.  If there is a narrowing somewhere along the line, that contributes to the higher pressure and should be treated.  Stenting can be performed in the inferior vena cava just like it can be performed in the lung arteries.  It sounds like your daughter might need to have a stent placed in her IVC if there is a narrowing.  But you should check with the cardiologist who did her recent cardiac catheterization, as they likely have pressure measurements and images from that area.  In asymptomatic patients with Fontan operations who have liver biopsies done in their teens just for surveillance purposes, some liver fibrosis is usually present.  This is not the same as cirrhosis, where there is more significant damage to the functional liver cells.  Regardless, the long term health of the liver will now be a focus of concern in the Fontan population, now that enough patients have survived into adulthood and it is being discovered as an issue.