Conversion from total cavopulmonary shunt to Fontan circulation: improved cyanosis with an 11-year interval.

We experienced a conversion case from total cavopulmonary shunt (TCPS) to the Fontan circulation with an 11-year interval in the left isomerism and an interruption of the inferior caval vein. Marked cyanosis (pulse oximetry measuring 74%) progressed 8 years after TCPS because of the prominent development of bilateral pulmonary arteriovenous fistula (PAVF), which was diminished after the Fontan conversion, and recent pulse oximetry measured 95% 22 months after the conversion. We believe that this report is a case with the longest interval from TCPS to Fontan in which PAVF was diminished and marked cyanosis was improved.