Impact of guideline directed medical therapy on myocardial function in adults with congenital heart disease

Int J Cardiol. 2024 Nov 1:414:132413. doi: 10.1016/j.ijcard.2024.132413. Epub 2024 Aug 3.

Abstract

Background: Guideline-directed heart failure therapy with angiotensin receptor blocker/neprilysin inhibitor (ARNi) and sodium-glucose transporter inhibitors (SGLT2i) has been incrementally beneficial in improving outcomes in heart failure patients.

Objective: Evaluate the feasibility and efficacy of guideline-directed medical therapy (GDMT) in adults congenital heart disease (ACHD) patients.

Methods: In a retrospective cohort study, ACHD patients with either New York Heart Association (NYHA) Class II symptoms or systemic ejection fraction (EF) <45%, optimized on a combination of beta-blocker (BB), ARNi, mineralocorticoid receptor antagonist (MRA) and SGLT2i were evaluated.

Results: Forty-six patients with a mean age 42.6 ± 12.1 years prescribed GDMT were identified. Twenty-eight (61%) were male, 20 (43%) had a systemic right ventricle (RV) and 9 (20%) had single-ventricle physiology. Over the optimization period, 20 (43%) were sustained on ARNi and 42 (91%) on SGLT2i in addition to treatment with BB and MRA. Over a period of 45 weeks, echocardiography parameters for left ventricle (LV) ejection fraction (EF) (+7.5%, p = 0.006), systemic ventricle (SV) velocity time integral (VTI) (+1.9 cm, p = 0.012) and LV global longitudinal strain (GLS) (-2.5%, p = 0.005) improved when 3-4 medications were used versus 1-2 medications alone. The use of either ARNi or SGLT2i (+8.1%, p = 0.017) or in combination (+7.0%, p = 0.043) increased LVEF compared to the use of neither medication.

Conclusion: Combination GDMT is beneficial in improving myocardial characteristics in ACHD patients with systemic RV and LV.

Keywords: Adult congenital heart disease; Guideline-directed medical therapy; Heart failure; Single ventricle; Systemic right ventricle.

MeSH terms

  • Adult
  • Angiotensin Receptor Antagonists / therapeutic use
  • Cohort Studies
  • Female
  • Heart Defects, Congenital* / drug therapy
  • Heart Defects, Congenital* / physiopathology
  • Heart Failure / drug therapy
  • Heart Failure / physiopathology
  • Humans
  • Male
  • Middle Aged
  • Neprilysin / antagonists & inhibitors
  • Practice Guidelines as Topic
  • Retrospective Studies
  • Sodium-Glucose Transporter 2 Inhibitors / therapeutic use
  • Stroke Volume / drug effects
  • Stroke Volume / physiology
  • Treatment Outcome

Substances

  • Sodium-Glucose Transporter 2 Inhibitors
  • Angiotensin Receptor Antagonists
  • Neprilysin