Congenital heart disease

Heart failure therapy in congenitally corrected transposition of the great arteries: effect on right ventricular function and survival

Eur Heart JPublished study

Authors' conclusion

GDMT use was associated with improved outcomes in patients with moderate/severe RV systolic dysfunction.

Study context

Population
Adults with congenitally corrected transposition of the great arteries (cc-TGA) presenting with systemic right ventricular (RV) systolic dysfunction
Participants
213 patients
Comparison
Higher baseline guideline-directed medical therapy (GDMT) score and ΔGDMT score
Outcome
Improved RV systolic function (relative increase in RVFWS of >20%)
Follow-up
1-year follow-up
Methodological detailsassessed at baseline and 1-year follow-up
  • Assessed at baseline and 1-year follow-up

Main finding

As reported in the abstract

Patients with improved RV systolic function

25%

Further findings

Improved neurohormonal activation (relative decrease in NT-proBNP levels of >20%) · 1-year follow-up

Patients with improved neurohormonal activation
56%
Higher baseline GDMT score and ΔGDMT score were associated with improvement in neurohormonal activation

Mortality

Patients who died
34 (16%)
Higher baseline GDMT score and ΔGDMT score were associated with a lower risk of mortality

Composite adverse event endpoint (heart failure hospitalization, death, and heart transplantation)

Higher baseline GDMT score and ΔGDMT score were associated with a lower risk of the composite adverse event endpoint

Baseline and change in GDMT score

Baseline GDMT score
1.97 ± 1.27
ΔGDMT score (GDMT at 1-year minus GDMT at baseline)
0.74 ± 0.39

Baseline RVFWS

Baseline RVFWS
−18 ± 5%

Baseline NT-proBNP

Baseline NT-proBNP
369 (142, 1004) pg/mL

Clinical benefits of GDMT use, by severity of RV systolic dysfunction (subgroup)

Clinical benefits of GDMT use were observed only in patients with moderate/severe RV systolic dysfunction, and not in patients with mild RV systolic dysfunction

Original publication

Heart failure therapy in congenitally corrected transposition of the great arteries: effect on right ventricular function and survival. Eur Heart J. Published 4 November 2026. doi:10.1093/eurheartj/ehag854

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