Congenital heart diseaseCohort study
Guideline-directed medical therapy and clinical outcomes in adults with Fontan palliation and systemic ventricular systolic dysfunction
- retrospective cohort study
Authors' conclusion
In adults with Fontan circulation and SV systolic dysfunction, higher GDMT intensity is associated with improved survival and reduced cardiovascular events. GDMT uptitration is associated with improved ventricular function and reduced congestion, suggesting a potential role for optimized medical therapy in this high-risk population.
Study context
- Population
- Adults (≥18 years) with Fontan palliation and systemic ventricular (SV) systolic dysfunction (SV ejection fraction <50%) (2003–2024)age 27 ± 9 years, 61% male
- Participants
- 182 adults
- Exposure
- Association between guideline-directed medical therapy (GDMT) intensity (standardized GDMT score) and clinical outcomes; GDMT uptitration vs no uptitration for SV systolic function and congestion
Main finding
As reported in the abstract
GDMT score
mean GDMT score
1.71 ± 1.53
Observational data: associations, not effects.
Further findings
All-cause mortality
| Comparison | HR (95% CI) | P value | |
|---|---|---|---|
| Higher GDMT score · independently associated with lower mortality | 0.68 (0.40–0.89) | P = 0.001 | |
| 0.512Hazard ratio (log scale) |
Cardiovascular events (composite hospitalization, transplantation, or death)
| Comparison | HR (95% CI) | P value | |
|---|---|---|---|
| Higher GDMT score · fewer cardiovascular events · These associations were consistent across subgroups. | 0.74 (0.53–0.95) | P = 0.02 | |
| 0.512Hazard ratio (log scale) |
| Outcome | change | P value |
|---|---|---|
| Change in SV ejection fraction (patients with follow-up data) · greater improvement with uptitration | +14% vs +3% | P = 0.01 |
| Change in longitudinal strain (patients with follow-up data) · greater improvement with uptitration | +19% vs +9% | P = 0.007 |
| Change in NT-proBNP (patients with follow-up data) · greater improvement with uptitration | −28% vs −15% | P = 0.009 |
All-cause mortality and cardiovascular events
- died
- 24%
- experienced cardiovascular events
- 47%
Original publication
Guideline-directed medical therapy and clinical outcomes in adults with Fontan palliation and systemic ventricular systolic dysfunction. Am Heart J. Published 25 August 2026. doi:10.1016/j.ahj.2026.107589
Summarised from the published abstract; every number is checked against it