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Single-Center Experience with Sacubitril/Valsartan in Patients with Congenital Heart Disease

Anusha Konduri1, Ashley Duimstra2, Ray Lowery2

  • 1University of Michigan Congenital Heart Center, Ann Arbor, MI, USA. konduria@med.umich.edu.

Pediatric Cardiology
|July 4, 2025
PubMed

Insights

Angiotensin II receptor blocker and neprilysin inhibitor (ARNI) therapy in pediatric congenital heart disease (CHD) patients showed potential benefits but also risks like hypotension and kidney injury. Careful monitoring and individualized treatment are crucial for this population.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Heart failure (HF) is a major cause of morbidity and mortality in patients with congenital heart disease (CHD).
  • Evidence-based therapies for HF in CHD are limited, unlike in acquired cardiovascular diseases.
  • Angiotensin II receptor blocker and neprilysin inhibitor (ARNI) therapy shows promise in adult HF but is underexplored in pediatric CHD.

Purpose of the Study:

  • To evaluate the safety and efficacy of Sacubitril/Valsartan (ARNI) in pediatric and young adult patients with CHD.
  • To assess the impact of ARNI therapy on clinical and laboratory parameters in this specific patient group.

Main Methods:

  • Retrospective chart review of 29 pediatric and young adult CHD patients treated with ARNI (Sacubitril/Valsartan).
  • Data collected on patient demographics, CHD type (including single ventricle), ARNI treatment duration, and adverse events.
  • Comparative analysis of clinical and laboratory data before and after ARNI initiation.

Main Results:

  • 52% of patients had single ventricle CHD; median follow-up was 8 months.
  • 41% experienced hypotension requiring dose adjustment or discontinuation; 2 patients developed acute kidney injury (AKI).
  • Significant reduction in systolic blood pressure (p=0.01) and increases in serum creatinine and potassium (p=0.02, p=0.03) were observed.
  • A trend towards improved ventricular systolic function was noted, but confounded by concomitant HF medications.

Conclusions:

  • ARNI therapy in pediatric and young adult CHD patients presents both potential benefits and risks, including hypotension and AKI.
  • Close monitoring and individualized treatment strategies are essential for managing ARNI therapy in this population.
  • Larger, prospective trials are needed to establish clear treatment guidelines for ARNI use in pediatric CHD.

Keywords:
ARNICongenital heart diseaseHeart failure

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