Association of Pre-Fontan Hemodynamics With Long-Term Outcomes After Fontan Palliation: A Study From the Pediatric Cardiac Care Consortium
Authors' conclusion
Elevated pre-Fontan mean pulmonary arterial pressure is a strong predictor of in-hospital and long-term post-Fontan risk of death or transplantation. Systemic right ventricle and delayed Fontan completion (>4 years of age) further increased risk.
Study context
- Population
- Patients undergoing first-time Fontan before 18 years of age with pre-Fontan catheterization, 1982–2011, US Pediatric Cardiac Care Consortium registry1,111 discharged with Fontan physiology
- Participants
- 1,175 patients
- Exposure
- Pre-Fontan mPAP tertiles
- Outcome
- In-hospital Fontan failure (death or takedown) and postdischarge death or transplantation
- Follow-up
- Median postdischarge 20.6 years
Methodological detailsmulticentre, registry, Outcomes identified through matching with the National Death Index and the Organ Procurement and Transplantation Network through 2022
- Multicentre
- Registry
- Outcomes identified through matching with the National Death Index and the Organ Procurement and Transplantation Network through 2022
Main finding
As reported in the abstract
Postdischarge death or transplantation, by pre-Fontan mean pulmonary arterial pressure
Each 1-SD increase in mean pulmonary arterial pressureEstimate (95% CI)P value
Death or transplantation, estimated at discharge
adjusted HR 2.20(1.62–3.00)
P < 0.01In-hospital failure
adjusted OR 1.33(1.00–1.77)
P = 0.05Each 1-SD increase in mean pulmonary arterial, as printed
Observational data: associations, not effects.
Further findings
| Outcome | Estimate | P value | |
|---|---|---|---|
| Decline of mean pulmonary arterial pressure hazard after discharge · per year after discharge | adjusted HR per year 0.97 (95% CI 0.95–0.99) | P < 0.01 | |
| Death or transplantation, other independent risk factorsSystemic right ventriclevs systemic left ventricle | adjusted HR 2.39 (95% CI 1.65–3.46) | P < 0.01 | |
| Death or transplantation, other independent risk factors>4 years of agevs 2 to 4 years of ageDelayed Fontan completion | adjusted HR 1.80 (95% CI 1.25–2.60) | P = 0.02 | |
| 0.20.5125Ratio (log scale) |
- Decline of mean pulmonary arterial pressure hazard after discharge: hazard declined 3% per year; resolved by 17 years (systemic RV) and 23 years (systemic LV)
Original publication
Association of Pre-Fontan Hemodynamics With Long-Term Outcomes After Fontan Palliation: A Study From the Pediatric Cardiac Care Consortium. Circulation. Published 22 January 2026. doi:10.
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