Objectives: This study reviewed late clinical features after Kawashima operation to confirm the impact of scheduled subsequent early Fontan completion.
Methods: 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.
Results: 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.
Conclusions: 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.
Keywords: Antegrade pulmonary blood flow; Fontan completion; Kawashima operation; Pulmonary arteriovenous malformations.