@Article{chd.12468,
AUTHOR = {Tarek Alsaied, Jouke P. Bokma, Mark E. Engel, Joey M. Kuijpers, Samuel P. Hanke, Liesl Zuhlke, Bin Zhang, Gruschen R. Veldtman},
TITLE = {Predicting long-term mortality after Fontan procedures: A risk score based on 6707 patients from 28 studies},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {12},
YEAR = {2017},
NUMBER = {4},
PAGES = {393--398},
URL = {http://www.techscience.com/schd/v12n4/39175},
ISSN = {3071-1738},
ABSTRACT = {<b>Background:</b> Reported long-term outcome measures vary greatly between studies in Fontan
patients making comprehensive appraisal of mortality hazard challenging. We sought to create a
clinical risk score to assist monitoring of Fontan patients in the outpatient setting.<br/>
<b>Methods:</b> A systematic review was conducted to evaluate risk factors for long-term (beyond the
first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90
patients were included or ≥20 long-term mortalities we reported. Risk factors for long-term mortality were determined. The pooled hazard ratios were used to create components of a clinical
score for long-term mortality using meta-analysis techniques.<br/>
<b>Results:</b> Twenty-eight studies were included. The total number of patients was 6707 with an average follow-up of 8.23 ± 5.42 years. There were 1000 deaths. Thirty-five risk factors for late
mortality were identified and classified into 9 categories and their relative hazards were used to
derive the initial components of a weighted, practical and clinically based Fontan risk score (ranging from 0 to 100). The final score included 8 risk factors: anatomic risk factors, elevated
preoperative pulmonary artery pressure, atriopulmonary Fontan, heart failure symptoms, arrhythmia, moderate/severe ventricular dysfunction or atrioventricular valve regurgitation, protein losing
enteropathy, and end organ disease (cirrhosis or renal insufficiency).<br/>
<b>Conclusion:</b> In patients with Fontan circulation, the influence of readily available risk factors can
be quantified in an integer score to predict long-term mortality. Prospective validation and refinement of this risk score will be undertaken.},
DOI = {10.1111/chd.12468}
}