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Associations between Bodyweight and Clinical Outcome in Patients Post-Fontan Procedure: A Systematic Review

Emma Payne1,2, Thomas Wilson2,3, Marjan Haghighi1,4, Zoe McCallum2,3, Yves d’Udekem5, Julian Ayer1,4,*

1 The University of Sydney, Sydney, Australia
2 The University of Melbourne, Melbourne, Australia
3 The Royal Children’s Hospital, Melbourne, Australia
4 The Royal Prince Alfred Hospital, Sydney, Australia
5 The Children’s National Hospital, Washington, USA

* Corresponding Author: Julian Ayer. Email:

Congenital Heart Disease 2022, 17(6), 617-639.


Background: Patients born with a single ventricle circulation commonly experience growth failure in early life, which is associated with adverse outcomes in infancy. However, associations between bodyweight or weight trajectory and clinical outcome post-Fontan procedure are yet to be determined. Methods: On the 1st of July 2021, a systematic review was performed in MEDLINE, EMBASE, the Cochrane Library, and Scopus of studies of patients with clinical outcome data post-Fontan procedure and association with bodyweight. Quality of studies was evaluated by Newcastle–Ottawa scale for cohort studies and Joanna Briggs Institute tool for cross-sectional studies. Results: Of 527 studies that underwent title and abstract screening, 15 were selected for final review. An increased risk of adverse post-Fontan outcomes was found for low weight patients, consistent with findings in infants. Whilst there is some evidence to suggest increased mortality in overweight adult patients, studies are conflicting as to whether overweight status is associated with increased heart failure. Increased BMI is associated with diminished exercise capacity and deceased physiological functioning. Negative weight trajectory is associated with adverse outcomes in the peri-Fontan period, whereas a positive weight trajectory is associated with increased Fontan failure in adulthood. Abnormal BMI (high or low) is associated with increased heart failure and poorer performance in quality-of-life scores. Conclusions: Bodyweight is a modifiable risk factor for poor clinical outcome in patients with a single ventricle circulation. Recognizing associations between bodyweight and Fontan pathophysiology may help to define patient-centered exercise and diet interventions that minimize patient morbidity and mortality.


Cite This Article

Payne, E., Wilson, T., Haghighi, M., McCallum, Z., D’Udekem, Y. et al. (2022). Associations between Bodyweight and Clinical Outcome in Patients Post-Fontan Procedure: A Systematic Review. Congenital Heart Disease, 17(6), 617–639.

This work is licensed under a Creative Commons Attribution 4.0 International License , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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