Clinical outcomes of early scheduled Fontan completion following Kawashima operation

This study reviewed late clinical features after Kawashima operation to confirm the impact of scheduled subsequent early Fontan completion. Of the 17 consecutive patients who underwent the Kawashima operation between 1987 and 2010, 11 underwent the procedure as inter-stage palliation (scheduled Fontan group). Ten of these patients underwent subsequent early Fontan completion after a median interval of 0.6 years. The remaining 6 patients underwent the Kawashima operation initially as definitive surgery (non-scheduled group). Late Fontan completion was performed in 4 of these patients as salvage surgery to prevent progression of hypoxia after a median interval of 6.6 years. All patients completed follow-up; the mean follow-up period was 12 ± 7 years (range 0.6–28.2). Cumulative survival at 10 years was 66% in the non-scheduled group and 79% in the scheduled group (p = 0.66). Pulmonary arteriovenous malformations developed after Kawashima operation in all 4 patients without antegrade pulmonary blood flow in the non-scheduled group but in only 2 of 11 patients in the scheduled group, both of which completely resolved after Fontan completion. In the scheduled Fontan group, 3 patients developed venovenous malformations between the Fontan pathway and the pulmonary veins or atrium after Fontan completion. Issues related to pulmonary arteriovenous malformations after Kawashima operation resolved with early scheduled Fontan completion. However, data on long-term outcomes are limited and the risk of death continues throughout early life. For unsuitable Fontan candidates, the Kawashima operation with antegrade pulmonary blood flow may provide definitive palliation.