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

Tacy E. Downing, Kiona Y. Allen, Andrew C. Glatz, Lindsay S. Rogers, Chitra Ravishankar, Jack Rychik, Jennifer A. Faerber, Stephanie Fuller, Lisa M. Montenegro, James M. Steven, Thomas L. Spray, Susan C. Nicolson, J. William Gaynor, David J. Goldberg

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147 Scopus citations


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.

Original languageEnglish
Pages (from-to)243-253.e2
JournalJournal of Thoracic and Cardiovascular Surgery
Issue number1
StatePublished - Jul 2017


  • cardiovascular surgery
  • congenital heart disease
  • mortality/survival
  • transplantation


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