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. 2024 Sep 9;3(10):101254.
doi: 10.1016/j.jacadv.2024.101254. eCollection 2024 Oct.

High-Performing Fontan Patients: A Fontan Outcome Registry by Cardiac Magnetic Resonance Imaging Study

Tarek Alsaied  1 , Runjia Li  2 , Adam B Christopher  1 , Mark A Fogel  3 , Timothy C Slesnick  4 , Rajesh Krishnamurthy  5 , Vivek Muthurangu  6 , Adam L Dorfman  7 , Christopher Z Lam  8 , Justin D Weigand  9 , Joshua D Robinson  10 , Rachael Cordina  11 , Laura J Olivieri  1 , Rahul H Rathod  12 ; FORCE Investigators
Collaborators, Affiliations

High-Performing Fontan Patients: A Fontan Outcome Registry by Cardiac Magnetic Resonance Imaging Study

Tarek Alsaied et al. JACC Adv. .

Abstract

Background: Fontan patients exhibit decreased exercise capacity. However, there is a subset of high-performing Fontan (HPF) patients with excellent exercise capacity.

Objectives: 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.

Methods: 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).

Results: The study included 813 patients (mean age: 20.2 ± 8.7 years). An HPF patient was associated with left ventricular morphology (OR: 1.50, P = 0.04), mixed morphology (OR: 2.23, P < 0.001), and a higher ejection fraction (OR: 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 (HR: 0.06 [95% CI: 0.01-0.25]).

Conclusions: 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.

Keywords: Fontan; exercise testing; single ventricle; univentricular heart.

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Conflict of interest statement

The FORCE registry is funded through a grant from 10.13039/100020415Additional Ventures and Evan’s Heart. The project described was supported by the 10.13039/100000002National Institutes of Health through Grant Number UL1 TR001857, KL2 TR001856, and/or TL1 TR001858. Dr Rathod received research grant support from Mezzion Pharmaceuticals as the Global PI for the FUEL-2 trial which is a drug RCT in Fontan patients. Dr Alsaied is a center PI for the same trial and also receives similar grant support. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Figures

None
Graphical abstract
Figure 1
Figure 1
Association of Peak VO2in Males and Females With Age and Weight In the upper panel, the peak VO2 increased with age and peaked around the age of 22 years in males and age of 18 years in females before starting to decline as the patients got older.
Figure 2
Figure 2
Forest Plot: ORs of Factors Associated With High-Performing Fontan Patients (Highest Quartile of Fontan-Specific Percent of Predicted VO2)
Figure 3
Figure 3
Time to Event Analysis: Composite Outcome of Death or Heart Transplant Listing Stratified by Fontan-Specific Percent of Predicted VO2Quartiles The high-performing fontan (HPF) group had lower hazard for the composite outcome. HPF = high-performing Fontan.
Central Illustration
Central Illustration
Predictors of High-Performing Fontan Patients and Their Outcomes: The FORCE Study Summary of the study in patients with fontan who had a cardiac MRI and an exercise stress test. High-performing fontan (HPF) is associated with significantly lower risk for death or transplant. The predictors of HPF by multivariable analysis are shown.
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Comment in

References

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