Key result
Diastolic dysfunction linked to ~3-fold higher risk of adverse outcomes post-Fontan.
Why the study?
Patients with single ventricle physiology post-Fontan are at high risk of diastolic dysfunction and elevated end-diastolic pressure, but the optimal EDP threshold and predictors of DD were not well defined.
Does diastolic dysfunction (EDP >13 mm Hg) increase the risk of adverse outcomes in patients post-Fontan procedure?
Cohort (n=861)
Does diastolic dysfunction (EDP >13 mm Hg) increase the risk of adverse outcomes in patients post-Fontan procedure?
In patients post-Fontan procedure, an end-diastolic pressure >13 mm Hg defines diastolic dysfunction and is associated with a more than 3-fold higher risk of adverse clinical outcomes.
Answers are grounded in this paper and cite their sources.
EDP >13 mm Hg may aid post-Fontan risk stratification; leaves open whether targeting diastolic dysfunction alters outcomes.
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 2-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 = .03) and kidney disease (19% vs 6%, P < .001). In multivariable analyses, DD was associated with the composite outcome (HR 3.37, 95% CI: 2.03-5.59, P < .001). Ninety-seven patients (11.3%) had DD. Multivariable analysis demonstrated that older age at catheterization, greater body mass index (BMI), nonleft 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 3-fold higher risk of adverse outcomes. Risk factors for DD include older age, higher BMI, nonleft ventricular morphology, and larger EDV. The presence of risk factors may warrant screening catheterization to identify DD and modify care accordingly.
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Alsaied et al. (2025) conducted a cohort in Post-Fontan single ventricle physiology (n=861). Diastolic dysfunction (EDP >13 mm Hg) vs. No diastolic dysfunction (EDP ≤13 mm Hg) was evaluated on Composite outcome of all-cause mortality, sustained atrial or ventricular arrhythmia, plastic bronchitis, protein-losing enteropathy, or listing for transplantation (HR 3.37, 95% CI 2.03-5.59, p=<.001). Diastolic dysfunction (EDP >13 mm Hg) was associated with a significantly higher risk of the composite adverse outcome post-Fontan (HR 3.37; 95% CI 2.03-5.59; P<0.001).
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