Heart failure & cardiomyopathiesResearch article
Adults with repaired systemic biventricular congenital heart disease with a systemic left ventricle and heart failure with reduce ejection fraction
- Cox regression analysis of heart failure (HF) therapy and outcomes
- standardized guideline-directed medical therapy (GDMT) score
Authors' conclusion
GDMT use at baseline, and GDMT uptitration were associated with improved outcomes and suggest a dose-dependent relationship between use of GDMT and risk of adverse outcomes. Furthermore, 67% of patients did not receive GDMT uptitration, suggesting suboptimal therapy and opportunities for improvement.
Study context
- Population
- Adults with repaired systemic biventricular congenital heart disease, systemic left ventricle, and HF with reduced ejection fraction (stage B/C HF and systemic left ventricular EF < 50%)258 (33%) had GDMT uptitration (∆GDMT score >0)
- Participants
- 778 adults
- Comparison
- Relationship between HF therapy and outcomes
Main finding
As reported in the abstract
GDMT scores · baseline and 1-year follow-up
baseline GDMT score
2 (1, 3)
∆GDMT score
0.56 (0.49, 0.62)
GDMT uptitration (∆GDMT score >0), of 778
258 (33%)
Further findings
| Outcome | adjusted HR (95% CI) | P value | |
|---|---|---|---|
| HF hospitalization — per 1 point increase in baseline GDMT score · lower risk | 0.83 (0.74–0.92) | P < 0.001 | |
| HF hospitalization — per 1 point increase in ∆GDMT score · lower risk | 0.71 (0.62–0.82) | P < 0.001 | |
| 0.512Adjusted hazard ratio (log scale) |
Mortality
| Comparison | adjusted HR (95% CI) | P value | |
|---|---|---|---|
| baseline GDMT score · lower mortality | 0.87 (0.76–0.98) | P = 0.01 | |
| ∆GDMT score · lower mortality | 0.84 (0.72–0.86) | P = 0.002 | |
| 0.512Adjusted hazard ratio (log scale) |
Original publication
Adults with repaired systemic biventricular congenital heart disease with a systemic left ventricle and heart failure with reduce ejection fraction. Am Heart J. Published 7 March 2026. doi:10.1016/j.ahj.2026.107422
Summarised from the published abstract; every number is checked against it