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High-Performing Fontan Patients
Journal article   Open access   Peer reviewed

High-Performing Fontan Patients

Tarek Alsaied, Runjia Li, Adam B. Christopher, Mark A. Fogel, Timothy C. Slesnick, Rajesh Krishnamurthy, Vivek Muthurangu, Adam L. Dorfman, Christopher Z. Lam, Justin D. Weigand, …
JACC. Advances (Online), Vol.3(10), p.101254
10/2024
DOI: 10.1016/j.jacadv.2024.101254
PMCID: PMC11414658
PMID: 39309667
url
https://doi.org/10.1016/j.jacadv.2024.101254View
Published (Version of record) Open Access

Abstract

Fontan patients exhibit decreased exercise capacity. However, there is a subset of high-performing Fontan (HPF) patients with excellent exercise capacity. This study aims to 1) create a Fontan-specific percent predicted peak VO2 tool using exercise data, 2) examine clinical factors associated with HPF patients, and 3) examine late outcomes in HPF patients. Patients in the multi-institutional Fontan Outcomes Registry Using CMR Examination above the age of 8 years who had a maximal exercise test were included. An HPF patient was defined as a patient in the upper Fontan-specific percent predicted peak VO2 quartile. Multivariable logistic regression was employed to investigate factors associated with the HPF and Cox regression was used to examine the association between HPF patients and late outcomes (composite of death or listing for cardiac transplant). The study included 813 patients (mean age: 20.2 ± 8.7 years). An HPF patient was associated with left ventricular morphology (odds ratio: 1.50, P = 0.04), mixed morphology (odds ratio: 2.23, P < 0.001), and a higher ejection fraction (odds ratio: 1.31 for 10% increase, P = 0.01). Patients with at least moderate atrioventricular valve regurgitation, protein-losing enteropathy, or who were using psychiatric medications, were less likely to be an HPF patient. After a mean follow-up of 3.7 years, 46 (5.7%) patients developed a composite endpoint. HPF had a lower risk of death or listing for cardiac transplant (hazard ratio: 0.06 [0.01, 0.25]). Patients with HPF have more favorable outcomes when compared to patients with lower exercise capacity. This large registry data highlights the role of exercise testing in providing personalized care and surveillance post-Fontan. [Display omitted]
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