hi my name is fatema from india.my son is 17.He has had severe ebsteins anomaly of the tricuspid valve and he has had a bi directional glenn shunt operation five years before . he is doing fine except that he is looking a little more blue. Is fontan required now or we can wait.
Ebstein’s anomaly of the tricuspid valve is downward displacement of the septal leaflet of the tricuspid valve, with varying degrees of abnormality of the other two leaflets of the valve. In your son’s case, his valve was so abnormal and was significantly obstructing outflow to the lungs that he required a bidirectional Glenn shunt to allow blood to get to the lungs. For our other readers, this is where the superior vena cava, the vein that drains the head and neck veins, is attached directly into the pulmonary arteries, bypassing the right side of the heart and allowing that blood to get to the lungs.
Without evaluating your son further, I cannot say if a Fontan is warranted or, at this late stage, whether he would even survive it. He most likely would need a cardiac catheterization to evaluate his anatomy and hemodynamics to see if his heart function is adequate and the blood pressure across his lungs are low enough. This would give more information to say whether this would be required. There are some centers that are able to repair the tricuspid valve and to make it functional; I cannot say if your son’s tricuspid valve would be a candidate for this, though.
Thankyou so much for your reply. Your comment about he surviving the fontan at this age has got me worried. If his glenn shunt is performing well then is not advisable to wait? Is there an age limit for a fontan surgery to be performed? Im getting his 2decho done today to get a clear picture.
Will get back to you with the reports.
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