Fontan circulation and systemic disease – a retrospective cohort analysis over 35 years of follow-up
Authors' conclusion
This population-based study demonstrated that patients with a Fontan circulation had increased risk of SD, which in turn led to higher risk of mortality.
Study context
- Population
- Patients with Fontan circulation, Quebec Congenital Heart Disease (CHD) Database, up to 35 years of follow-upmatched cohort
- Participants
- 533 vs 10,280
- Exposure
- Fontan circulation vs isolated ventricular septal defect (VSD)
- Fontann = 533
- VSDn = 10,280reference
- Outcome
- Cumulative risk of systemic disease (≥1 hospitalization for extra-cardiac complications: liver, respiratory, gastrointestinal or renal disease)
- Follow-up
- 10- and 35-years follow-up
Methodological detailsmatched, population-based
- Matched
- Population-based
Main finding
As reported in the abstract
Cumulative probability of SD · 10 and 35 years
10 years
35 years
- Fontan vs VSD: P < 0.0001 — 4 to 7 times the probabilities in VSD
Observational data: associations, not effects.
Further findings
| Outcome | Fold increase in risk (95% CI) | |
|---|---|---|
| 10-year mortality: multisystem disease (>1 extra-cardiac organ) vs no SD (Fontan) | 3.38 (1.73–6.60) | |
| −50510Difference (linear scale) |
| Outcome | Fold higher risk (95% CI) |
|---|---|
| Risk factor for SD: cardiovascular complications (Fontan)Cardiovascular complications vs No cardiovascular complications | 4.1 (3.52–4.88) |
Original publication
Fontan circulation and systemic disease – a retrospective cohort analysis over 35 years of follow-up. Am Heart J. Published 17 October 2024. doi:10.
Summarised from the published abstract; every number is checked against it