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

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.

Department

Pediatrics

Link to Full Text

Share

COinS