Effect of Fontan-Associated Morbidities on Survival With Intact Fontan Circulation

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

Research output: Contribution to journalArticlepeer-review

70 Scopus citations


Although survival after the Fontan operation has improved, little is known about the burden of major medical morbidities associated with the modern total cavopulmonary connection (TCPC). A total of 773 consecutive patients who underwent a first Fontan operation at our institution between 1992 and 2009 were retrospectively reviewed. All subjects underwent TCPC (53% lateral tunnel, 47% extracardiac conduit). Median length of follow-up was 5.3 years (interquartile range 1.4 to 11.2), and 30% had follow-up >10 years. Freedom from a composite medical morbidity outcome (protein-losing enteropathy, plastic bronchitis, serious thromboembolic event, or tachyarrhythmia) was 47% at 20 years (95% confidence interval [CI] 38 to 55). Independent risk factors for morbidity included pre-Fontan atrioventricular valve regurgitation (hazard ratio [HR] 1.7, 95% CI 1.2 to 2.4, p = 0.001), pleural drainage >14 days (HR 1.5, 95% CI 1.01 to 2.2, p = 0.04), and longer cross-clamp time (HR 1.2 per 10 minutes, 95% CI 1.06 to 1.3, p = 0.004) at the time of TCPC. Surgical era, Fontan type, and ventricular morphology were not associated with the composite outcome. Presence of Fontan-associated morbidity was associated with a 36-fold increase in the risk of subsequent Fontan takedown, heart transplantation, or death (95% CI 17 to 76, p <0.001). For patients without any component of the composite outcome, freedom from Fontan failure was 98% at 20 years (95% CI 96 to 99). Medical morbidities after TCPC are common and significantly reduce the longevity of the Fontan circulation. However, for those patients who remain free from the composite morbidity outcome, 20-year survival with intact Fontan circulation is encouraging.

Original languageEnglish
Pages (from-to)1866-1871
Number of pages6
JournalAmerican Journal of Cardiology
Issue number11
StatePublished - Jun 1 2017


Dive into the research topics of 'Effect of Fontan-Associated Morbidities on Survival With Intact Fontan Circulation'. Together they form a unique fingerprint.

Cite this