Congenital heart diseaseCohort study
Low Ventricular Chamber Stiffness Identifies a High-Risk Eccentric Remodeling Phenotype in the Fontan Circulation: A FORCE Registry Analysis
- Registry cohort study
- paired catheterization and CMR
Authors' conclusion
Contrary to prevailing assumptions, lower rather than higher chamber stiffness was associated with adverse Fontan outcomes, identifying a high-compliance phenotype characterized by ventricular dilation and eccentric remodeling.
Study context
- Population
- Patients with a Fontan circulation (FORCE registry)median age 14.7 years
- Participants
- 814 patients
- Exposure
- Lower vs higher ventricular chamber stiffness
- Outcome
- Composite adverse outcome:
- Death
- Transplantation listing
- Sustained atrial or ventricular arrhythmias
- Protein-losing enteropathy
- Plastic bronchitis
- Follow-up
- Median 2.7 years (0.9-6.1 years)
Main finding
Composite adverse outcome · median follow-up 2.7 years
Adjusted hazard ratio
1.30 per 1-SD decrease
95% CI 1.04–1.61
Lower β Z score
adjusted HR 1.30 per 1-SD decrease (95% CI 1.04–1.61)
Per 1-SD decrease, as printed
As reported in the abstract
Observational data: associations, not effects.
Further findings
Chamber stiffness β (whole cohort)
- Median β
- 0.034 mL-1 (0.025-0.046)
Correlates of lower β Z score
- Lower β Z score associated with dominant RV, eccentric remodeling with reduced EF, increased collateral flow and intracardiac recirculation, greater symptom burden and higher arrhythmia prevalence, despite similar filling pressures
Discrimination for adverse outcomes
- β Z score <−0.71, β<0.025 mL-1, or BSA-indexed EDV >103 mL/m²: similarly modest discrimination (AUC)
- 0.59
- Greater discrimination in dominant RVs
Original publication
Low Ventricular Chamber Stiffness Identifies a High-Risk Eccentric Remodeling Phenotype in the Fontan Circulation: A FORCE Registry Analysis. Circulation. Published 28 August 2026. doi:10.1161/circulationaha.126.081043
Summarised from the published abstract; every number is checked against it