Congenital heart diseaseCohort study
Association of Pre-Fontan Hemodynamics With Long-Term Outcomes After Fontan Palliation: A Study From the Pediatric Cardiac Care Consortium
- Retrospective cohort study
- multicentre
- registry
Outcomes identified through matching with the National Death Index and the Organ Procurement and Transplantation Network through 2022
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
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 factors — systemic right ventricle vs 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 age vs 2 to 4 years of age · delayed 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:10.1161/circulationaha.125.076880
Summarised from the published abstract; every number is checked against it