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The Fontan circulation was one of the “big ideas” in congenital heart disease in the 1970s. This completely novel type of circulation was at first the source of some astonishment but, gradually, understanding of its properties progressed and there is now an expanding cohort of patients followed up with stable Fontan circulations.
In 1990, on the basis of an observational study of the survival and wellbeing of 334 patients, Fontan et al came to the disappointing conclusion that even after a perfect Fontan procedure there was a gradual decline in the functional status of patients that the authors could not attribute to anything other than the Fontan circulation itself.1 This and other long term follow up studies have provoked a wave of negativity about Fontan-type operations that has had repercussions for patients with complex anatomy facing radical operations.
Two European papers,2 ,3 describe cross sectional studies of medium and long term survivors of Fontan-type operations. They both focus on the non-cardiac complications of liver dysfunction, coagulation disorders, and protein losing enteropathy rather than the time related incidences of death, arrhythmias, and surgical complications, such as pathway obstruction, addressed in several other series. We have now to respond to the consensus that at least 40% of patients, most of whom are functioning tolerably well at late follow up, have a raised concentration of one or more liver enzymes, at least 25% have a depletion of some coagulation factors, and 7% have abnormally low serum albumin.
Venous hypertension, liver dysfunction, and low albumin concentrations
Are the abnormalities of liver function and coagulation explicable by the Fontan state as we understand it? Fundamental to the operation of all Fontan-type circulations is a chronic increase of systemic venous pressure to levels that would correspond to significant right heart disease in a biventricular circulation (fig 1 …