Open Access

ARTICLE

Predicting long-term mortality after Fontan procedures: A risk score based on 6707 patients from 28 studies

Tarek Alsaied¹, Jouke P. Bokma², Mark E. Engel³, Joey M. Kuijpers², Samuel P. Hanke¹, Liesl Zuhlke⁴, Bin Zhang⁵, Gruschen R. Veldtman⁶

1 Children’s Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
2 Department of Cardiology, Academic Medical Center, Amsterdam, Interuniversity Cardiology Institute of the Netherlands, Netherlands Heart Institute, Utrecht, The Netherlands
3 Department of Medicine, Groote Schuur Hospital, The Cardiac Clinic, Department of Medicine, Groote Schuur Hospital and University of Cape Town, Cape Town, South Africa
4 Department of Pediatrics, Red Cross War Memorial Children’s Hospital and University of Cape Town, Cape Town, South Africa
5 Division of Biostatistics and Epidemiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
6 Adolescent and Adult Congenital Program, Children’s Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA

* Corresponding Author: Gruschen R. Veldtman, Professor of Pediatrics, Adolescent and Adult Congenital Program, Children’s Heart Institute, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA. Email:

Congenital Heart Disease 2017, 12(4), 393-398. https://doi.org/10.1111/chd.12468

Abstract

Background: 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.
Methods: 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.
Results: 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).
Conclusion: 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.

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