• Gregory King, Julian Ayer, David Celermajer, Dominica Zentner, Robert Justo, Patrick Disney, Diana Zannino, and Yves d'Udekem.
• Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia; Department of Paediatrics, The University of Melbourne, Melbourne, Victoria, Australia; Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia.
• J. Am. Coll. Cardiol. 2019 Feb 26; 73 (7): 810-822.

BackgroundAtrioventricular valve failure (moderate or greater regurgitation, or valve operation) is a risk factor for adverse outcomes in patients undergoing Fontan palliation.ObjectivesThis study determined the incidence of atrioventricular valve failure and its clinical impact on patients undergoing Fontan palliation.MethodsA retrospective cohort longitudinal study was conducted using patient data extracted from an existing bi-national, population-based registry.ResultsA total of 1,468 patients who underwent Fontan palliation were identified; complete follow-up data were available for 1,199 patients. Six hundred eighty-six patients had 2 atrioventricular valves, 286 had a single mitral valve, 130 had a common atrioventricular valve, and 97 had a single tricuspid valve. A total of 132 repairs were performed in 110 patients, and 15 replacements were performed in 13 patients. The cumulative incidence of atrioventricular valve failure at 25 years of age for patients with a common atrioventricular, single tricuspid, single mitral, and 2 atrioventricular valves was 56% (95% confidence interval [CI]: 46% to 67%), 46% (95% CI: 31% to 61%), 8% (95% CI: 4% to 12%), and 26% (95% CI: 21% to 30%), respectively. In patients without valve failure, freedom from Fontan failure at 10 and 20 years post-Fontan palliation was 91% (95% CI: 89% to 93%) and 77% (95% CI: 73% to 81%), respectively, compared with 77% (95% CI: 69% to 85%) and 54% (95% CI: 42% to 68%), respectively, in patients with valve failure (hazard ratio: 2.43; 95% CI: 1.74 to 3.39; p < 0.001).ConclusionsAtrioventricular valve failure occurs frequently in patients undergoing Fontan palliation. Patients with valve failure are twice as likely to have their Fontan circulation fail than those without valve failure.Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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