The Fontan Procedure has been used since the 1970s to treat people with a single ventricle physiology -- also known as a univentricular heart. It has also been used in the last few decades to treat hypoplastic left heart syndrome (HLHS) or hypoplastic right heart syndrome (HRHS). Now there are enough long-term survivors of the Fontan Procedure for the medical establishment to know what kinds of consequences having such a radical physiology poses. When the heart's sole function is to pump blood to the body, and it travels passively to the lungs for oxygen, how does that anatomy affect other organs, specifically the liver?
Dr. Fred Wu of Boston Children's Hospital is an expert in working with adults with congenital heart defects with liver involvement. Today's episode is a continuation of the conversation he had with Anna last week. In today's program he talks about whether or not Stage IV of the Fontan is actually transplantation, what advice he feels Fontan Survivors and parents of children born with single ventricle hearts need to hear and finally, he gives us his prediction for what the future holds for this unique population.