Defining Diastolic Dysfunction Post-Fontan: Threshold, Risk Factors, and Associations with Outcomes

Authors

• Tarek Alsaied, Heart Institute, UPMC Children's Hospital of Pittsburgh, Department of Pediatrics, Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA. Electronic address: alsaiedt@upmc.edu.
• Runjia Li, Department of Biostatistics, University of Pittsburgh, Pittsburgh, PA, USA.
• Haley Grant, Department of Biostatistics, University of Pittsburgh, Pittsburgh, PA, USA.
• Mary D. Schiff, Heart Institute, UPMC Children's Hospital of Pittsburgh, Department of Pediatrics, Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
• Yu Li, Department of Pediatrics, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
• Adam B. Christopher, Heart Institute, UPMC Children's Hospital of Pittsburgh, Department of Pediatrics, Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
• Jacqueline Kreutzer, Heart Institute, UPMC Children's Hospital of Pittsburgh, Department of Pediatrics, Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
• Bryan H. Goldstein, Heart Institute, UPMC Children's Hospital of Pittsburgh, Department of Pediatrics, Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
• Jonathan H. Soslow, Division of Pediatric Cardiology, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, TN, USA.
• Yue-Hin Loke, Division of Pediatric Cardiology, George Washington University School of Medicine & Health Sciences, Washington, DC, USA.
• Mark Fogel, Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
• Timothy C. Slesnick, Department of Pediatrics, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, GA, USA.
• Rajesh Krishnamurthy, Department of Radiology, Nationwide Children's Hospital, Columbus, OH, USA.
• Vivek Muthurangu, UCL Centre for Cardiovascular Imaging, Institute of Cardiovascular Science, University College London, London, UK.
• Adam L. Dorfman, Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI, USA.
• Christopher Lam, Department of Diagnostic and Interventional Radiology, The Hospital for Sick Children, and Department of Medical Imaging, University of Toronto, Toronto, ON, Canada.
• Justin D. Weigand, Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA.
• Joshua D. Robinson, Department of Pediatrics, Ann & Robert H. Lurie's Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
• Laura J. Olivieri, Heart Institute, UPMC Children's Hospital of Pittsburgh, Department of Pediatrics, Division of Cardiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
• Rahul H. Rathod, Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Document Type

Journal Article

Publication Date

7-10-2025

Journal

American heart journal

DOI

10.1016/j.ahj.2025.07.007

Keywords

Fontan; Fontan circulatory failure; cardiac magnetic resonance; diastolic dysfunction; heart failure with preserved ejection fraction

Abstract

BACKGROUND: Following the Fontan procedure, patients with single ventricle physiology are at high risk of diastolic dysfunction (DD) and elevated end-diastolic pressure (EDP). OBJECTIVE: This study aims to determine (1) the optimal EDP threshold correlated with adverse outcomes post-Fontan and (2) the clinical and imaging predictors of DD. METHODS: The study included patients from the Fontan Outcome Registry using CMR Examinations (FORCE) who underwent cardiac catheterization and cardiac magnetic resonance (CMR) within a two-year window. The composite outcome was defined as all-cause mortality, sustained atrial or ventricular arrhythmia, plastic bronchitis, protein-losing enteropathy, or listing for transplantation. The EDP cutoff was determined using the lowest Brier score from Cox proportional hazard models. RESULTS: The study included 861 patients (mean age 16.4 ± 9.3 years). Mean EDP was 9.0 ± 3.5 mm Hg, with DD defined at an optimal EDP threshold >13 mm Hg. Patients were followed for a median of 3.6 years after catheterization. By univariable analysis patients with DD were more likely to have Fontan associated liver disease (40% vs 29%, p=0.03) and kidney disease (19% vs 6%, p<0.001). In multivariable analyses, DD was associated with the composite outcome (HR 3.37, 95% CI: 2.03-5.59, p<0.001). Ninety-seven patients (11.3%) had DD. Multivariable analysis demonstrated that older age at catheterization, greater body mass index (BMI), non-left ventricular morphology, and higher ventricular end-diastolic volume (EDV) were associated with DD. CONCLUSION: DD, defined as an EDP >13 mm Hg, is linked to over threefold higher risk of adverse outcomes. Risk factors for DD include older age, higher BMI, non-left ventricular morphology, and larger EDV. The presence of risk factors may warrant screening catheterization to identify DD and modify care accordingly.

APA Citation

Alsaied, Tarek; Li, Runjia; Grant, Haley; Schiff, Mary D.; Li, Yu; Christopher, Adam B.; Kreutzer, Jacqueline; Goldstein, Bryan H.; Soslow, Jonathan H.; Loke, Yue-Hin; Fogel, Mark; Slesnick, Timothy C.; Krishnamurthy, Rajesh; Muthurangu, Vivek; Dorfman, Adam L.; Lam, Christopher; Weigand, Justin D.; Robinson, Joshua D.; Olivieri, Laura J.; and Rathod, Rahul H., "Defining Diastolic Dysfunction Post-Fontan: Threshold, Risk Factors, and Associations with Outcomes" (2025). GW Authored Works. Paper 7610.
https://hsrc.himmelfarb.gwu.edu/gwhpubs/7610

Department

Pediatrics