Kids with Alagille Syndrome range quite widely in the extent of the misformation of their heart and liver. Sasha had no pulmonary artery connecting heart and lungs: a big hole between her heart chambers let the blood mix and a number of very small arteries brought blood to her lungs. Some AGS kids have very mild forms of stenosis.
It was explained to us early on that the decisions surrounding a child who just has a heart condition is very much complicated when the liver is also compromised. Particularly, the option of a liver transplant is not available to those with serious cardiac conditions.
The hope is that the liver can improve over time as the heart improves. However as Sasha's heart was repaired by surgery, improving blood flow, her liver deteriorated. It is assumed that she developed portal hypertension (the pressure differential demonstrating the condition was going to be measured in IGT had we decided to go ahead with the TIPS procedure) and this produced bleeding varices which ended Sasha's life.
We think there is a lesson there for Alagille Syndrome children with serious heart conditions. The extent of the liver damage (paucity of bile ducts) is unknown. Some livers can hold their own for some years with medical supports and even improve, others deterioriate with some failing quickly.
The difference between the second two categories (slow vs fast liver deterioration) is not well understood.
In Sasha's life we think there were two impacts. The bleeding episode just after her first birthday is suggestive of early portal hypertension. This may suggest that as her blood flow improved, with cathetarizations and a first surgery at three months, her liver condition deteriorated. Does the liver deteriorate on its own or does the extra flow of blood to the liver potentially impact negatively on the liver.
Then in CCU she bled after surgery for several days and her lower organs were deprived of blood and the numbers showed the liver in a toxic condition, approaching failure, and then the numbers improved progressively. This impact to her liver might have further worsened the naturally occuring portal hypertension.
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