My daughter is 31 months old and has tricuspid atresia with L-TGA. She underwent PA banding and atrial septostomy at 7 weeks of age and bi-directional Glenn at 6 months. She tolerated both surgeries remarkably well and is on track developmentally. Her oxygen saturation is generally in the low 80s.
She had a cardiac cath in Sept 2010 which showed mean pulmonary artery pressure of 13 mm Hg with a common atrial pressure of 9 mm Hg. Left ventricular end diastolic pressure was 10 mm Hg. There was no obstruction into the aorta. The aorta itself was widely patent. There was minimal right-sided (mitral) AV valve regurgitation. Cardiac function was good. The branch pulmonary arteries were of very good size and the hemi-fontan anastomosis was widely patent.
My question is regarding the timing of the stage 3 Fontan procedure. What are the arguments for an early Fontan? What are the potential risks of waiting a few years? With all of the research surrounding post-Fontan complications, we wonder if putting the surgery off as long as possible may be the best option for us considering how well our daughter is doing with her current circulation.
You are correct, it sounds like your daughter has quite satisfactory Stage 2 Glenn hemodynamics. As such she is likely to be functioning quite well. However, usually by grade school age, the oxygen levels begin to drop as the amount of blood being sent to the lungs through the Glenn shunt is relatively limited compared to the needs of the growing body. The completion of a Fontan is ultimately recommended to do 2 things; 1) to improve the circulating oxygen level by increasing the amount of blood being directed to the lungs, and 2) to decrease the work load of the single pumping chamber. The timing of Fontan completion is rarely urgent, and is worked out according to the preferences of the family, doctors and instituitonal patterns. We generally perform elective Fontan revisions between 2 and 4 years of age, generally waiting for a minimum weight of at least 30 lbs. You are correct that once a child has a Fontan circulation, the risk for developing complications related to the higher venous pressures in the liver and bowel, begins. The long term prognosis after a Fontan completion is still unknown, with many patients requiring pacemakers as they develop arrhythmias, and some have developed pump failure of the single pumping chamber (leading to heart transplantation). More information is still being acquired every year about this complex group of pateints who require intense, lifelong follow up.
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