Abstract 17017: Risk Prediction of Death and Failure in Patients With Atrio-pulmonary Connections: 40 Years Experience in Australia and New Zealand
Objective: To identify parameters predictive of death and failure in patients with an atrio-pulmonary connection Fontan.
Methods: Retrospective analysis of long-term follow-up data using the Australia and New Zealand Fontan Registry
Results: There were 245 patients surviving hospital discharge after an atriopulmonary Fontan from 1975 to 1994. At latest follow-up, 54 pts died, 29 underwent heart transplantation and 93 had Fontan failure (death, transplantation, Fontan conversion, protein losing enteropathy, severe left ventricular dysfunction on echocardiography or NYHA3). Twenty-eight years survival, freedom from death and transplantation and freedom from Fontan failure were respectively 62% (95%CI 55-71%), 58% (95% CI 50-67%) and 40% (95% CI 33-50%). Predictors of death and transplantation were dextrocardia (HR 1.8;p= 0.05), longer Fontan bypass time (HR= 1.01, p=0.05) and moderate or more late atrioventricular valve regurgitation (HR 3.97; p=0.02). One hundred and seven patients developed atrial arrhythmias, with first documented event at 15.7 years (IQR 10.5-19.3) post Fontan completion. Development of arrhythmia increased the likelihood of death or transplantation (HR 3.05, p<0.001) and Fontan failure (HR 4.7, p<0.001). Thromboembolic events occurred in 42 patients, 30 (71%) of whom were on warfarin but less than half (12/30) within therapeutic range. Thirty-nine patients underwent Fontan conversion. Threshold for conversion was lower in one center in which patients were operated earlier with better functional status, shorter history of arrhythmias and less antiarrhythmic medications. Conversion resulted in a reduced risk of Fontan failure (HR 0.48) in patients cared for by the center with lower conversion threshold but an increased risk in the remaining centres (HR 5.65) (p=0.023).
Conclusions: Arrhythmias is an early sign predicting failure of the Fontan circulation of those with an atrio-pulmonary Fontan. Fontan conversion is only associated with improved outcomes when timely performed.
Author Disclosures: C.L. Poh: None. D. Zannino: None. R.G. Weintraub: None. D.S. Winlaw: None. D.J. Radford: None. L.E. Grigg: None. G.R. Wheaton: None. A. Bullock: None. R.N. Justo: None. T.L. Gentles: None. D.S. Celermajer: None. Y. d'Udekem: None.
- © 2015 by American Heart Association, Inc.