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28th May, 2026 12:00 AM
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Heart Failure Therapy Benefits Rare Structural Heart Defect

TOPLINE:

In adults with congenitally corrected transposition of the great arteries (cc-TGA) and systemic right ventricular (RV) dysfunction, a greater use of guideline-directed medical therapy (GDMT) was associated with improved systemic RV systolic function and a reduced risk for mortality, according to a recent brief report.

METHODOLOGY:

  • Researchers conducted a retrospective cohort study to evaluate the effect of GDMT on cardiac function, congestion, and cardiovascular outcomes in patients with cc-TGA.
  • The study included 213 adults with cc-TGA and systemic RV systolic dysfunction (RV ejection fraction < 50%) who underwent follow-up at the Mayo Clinic in the US from January 2013 to December 2024. Participants were aged 18 years or older, and 54% were men.
  • The intensity of heart failure therapy was assessed using a GDMT score calculated from baseline medications, including beta-blockers, angiotensin-converting enzyme inhibitors, angiotensin II receptor blockers, angiotensin receptor-neprilysin inhibitors, mineralocorticoid receptor antagonists, and SGLT2 inhibitors.
  • Participants were divided into two groups based on the median GDMT score (< 2.0 vs ≥ 2.0). Systemic RV systolic function was assessed using the RV free wall strain (RVFWS) measured using echocardiography at baseline and 1-year follow-up.
  • An improvement in RV systolic function was defined as a relative increase in the RVFWS more than 20% from baseline.

TAKEAWAY:

  • Overall, 25% of patients had significant improvements in RV systolic function.
  • Patients with a GDMT score ≥ 2.0 experienced a greater improvement in the RVFWS than those with a score < 2.0 (relative change, 17% vs 6%; P < .001). Each one-unit increase in the GDMT score was associated with 63% higher odds of improved RV systolic function (adjusted odds ratio, 1.63; 95% CI, 1.13-4.29).
  • The 5-year cumulative incidence of all-cause mortality was lower in patients with a GDMT score ≥ 2.0 than in those with a score < 2.0 (9% vs 22%; P = .007). Each one-unit increase in the GDMT score was linked to a 32% lower risk for mortality (P = .01).

IN PRACTICE:

“This finding, coupled with the association between GDMT use and survival, suggests that GDMT may be beneficial in patients with cc-TGA and RV systolic dysfunction and should be considered in these patients,” the researchers wrote. 

“The clinical benefits of GDMT use were observed only in patients with moderate/severe RV systolic dysfunction at baseline, and not in the patients with mild RV systolic function,” they added.

SOURCE:

The study was led by Sara Ahmed, MBBCh, Mayo Clinic in Rochester, Minnesota. It was published online on May 19 as a brief report in JACC: Heart Failure.

LIMITATIONS:

The retrospective and nonrandomized study design may have introduced inherent selection and ascertainment bias. Compliance with medical therapy could not be ascertained, and the effect of intervening medical or surgical treatments during follow-up could not be accounted for.

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DISCLOSURES:

No funding information was explicitly mentioned for this study. The authors disclosed having no competing relationships relevant to this study.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.


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