Defining diastolic dysfunction post-Fontan: Threshold, risk factors, and associations with outcomes

Alsaied, T; Li, R; Grant, H; Schiff, MD; Li, Y; Christopher, AB; Kreutzer, J; Goldstein, BH; Soslow, JH; Loke, YH; Fogel, MA; Slesnick, TC; Krishnamurthy, R; Muthurangu, V; Dorfman, AL; Lam, C; Weigand, JD; Robinson, JD; Olivieri, LJ; Rathod, RH; FORCE Investigators

doi:10.1016/j.ahj.2025.07.007

Congenital heart disease

Defining diastolic dysfunction post-Fontan: Threshold, risk factors, and associations with outcomes

Am Heart JPublished 10 Jul 2025Retrospective cohort study

Main finding · Composite outcomeResultHazard ratio 3.37 95% CI 2.03–5.59

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Authors' 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.

Study context

Population: Post-Fontan patients with single ventricle physiology with cardiac catheterization and CMR within 2 years (FORCE registry)mean age 16.4 ± 9.3 years
Participants: 861 patients

Exposure: Diastolic dysfunction vs no diastolic dysfunction
Diastolic dysfunction (DD)n = 97
Outcome: Composite of:
All-cause mortality
Sustained atrial or ventricular arrhythmia
Plastic bronchitis
Protein-losing enteropathy
Listing for transplantation
Follow-up: Median 3.6 years after catheterization

Methodological detailsregistry-based (FORCE)

Registry-based (FORCE)

Main finding

Composite outcome

Hazard ratio

3.37

95% CI 2.03–5.59

P < 0.001

DDvs No DD

HR 3.37 (95% CI 2.03–5.59); P < 0.001

As reported in the abstract

Optimal end-diastolic pressure (EDP) threshold for DD (lowest Brier score): >13 mm Hg

Observational data: associations, not effects.

Further findings

Mean EDP (whole cohort)

Mean EDP: 9.0 ± 3.5 mm Hg
DD threshold (optimal EDP): >13 mm Hg

Prevalence of DD

Patients with DD: 97 (11.3%)

Fontan-associated liver and kidney disease, DD vs no DD

Fontan-associated liver disease, DD vs no DD: 40% vs 29%, P = .03
Kidney disease, DD vs no DD: 19% vs 6%, P < .001

Univariable analysis

Predictors of DD

Older age at catheterization, greater body mass index (BMI), nonleft ventricular morphology and higher ventricular end-diastolic volume (EDV) associated with DD

Multivariable analysis

Original publication

Defining diastolic dysfunction post-Fontan: Threshold, risk factors, and associations with outcomes. Am Heart J. Published 10 July 2025. doi:10.1016/j.ahj.2025.07.007

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