Heart failure & cardiomyopathies

Adults with repaired systemic biventricular congenital heart disease with a systemic left ventricle and heart failure with reduce ejection fraction

Am Heart JPublished Cox regression analysis of heart failure (HF) therapy and outcomes

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 guideline-directed medical therapy (GDMT) uptitration (∆GDMT score >0)
Participants
778 adults
Comparison
Relationship between HF therapy and outcomes
Methodological detailsstandardized GDMT score
  • Standardized GDMT score

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

OutcomeAdjusted HR (95% CI)P value
HF hospitalization — per 1 point increase in baseline GDMT score · lower risk0.83 (0.74–0.92)P < 0.001
HF hospitalization — per 1 point increase in ∆GDMT score · lower risk0.71 (0.62–0.82)P < 0.001

Mortality

ComparisonAdjusted HR (95% CI)P value
Baseline GDMT score · lower mortality0.87 (0.76–0.98)P = 0.01
∆GDMT score · lower mortality0.84 (0.72–0.86)P = 0.002

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

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