Long-term survival after the Fontan operation: Twenty years of experience at a single center

Objective: Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term post-Fontan survival in a modern patient cohort. Methods: All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed. The primary outcome was the composite endpoint of Fontan takedown, heart transplantation, or death before 2013. Results: Follow-up rate was 99.2%. Survival with intact Fontan circulation was 94% at 1 year (95% confidence interval [ 95% CI], 92%-95%), 90% at 10 years (95% CI, 88%-92%), 85% at 15 years (95% CI, 82%-88%), and 74% at 20 years (95% CI, 67%-80%). Distinct risk factors were identified for early (<= 1 year) and late composite outcomes. Independent risk factors for early outcome included prolonged pleural drainage (hazard ratio [HR], 4.4; P<.001), intensive care unit stay>1 week (HR, 2.4; P <. 001), Fontan before 1997 (HR, 3.3; P <. 001), preoperative atrioventricular valve regurgitation (HR, 2.0; P <. 001), and longer crossclamp time (HR, 1.3 per 10 minutes; P <. 001). Late outcome was predicted by atrioventricular valve regurgitation prior to Fontan (HR, 2.0; P <.001), and post-Fontan ICU stay>1 week (HR, 2.4; P <. 001). Conclusions: Long-term mortality after Fontan operation remains substantial. Risk factors for death or loss of Fontan circulation differ between the early and late postoperative periods. Long-term survival has not improved appreciably over the last decade, suggesting that alternatives to the Fontan are warranted.