Angiotensin converting enzyme inhibitors and interstage failure in infants with hypoplastic left heart syndrome

Doris P Yimgang1, John D Sorkin2, Charles F Evans3

  • 1Department of Epidemiology and Public Health, University of Maryland School of Medicine, Baltimore, Maryland, USA.

Insights

Angiotensin converting enzyme inhibitors did not improve interstage outcomes in infants with hypoplastic left heart syndrome. This study found no significant benefit in reducing interstage failure for these single ventricle defect patients.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease Research
  • Pharmacological Interventions in Neonates

Background:

  • Angiotensin converting enzyme inhibitors (ACEIs) are frequently prescribed post-Norwood procedure for single ventricle defects.
  • Limited data exist on the efficacy of ACEIs in improving interstage outcomes for these vulnerable infants.
  • Hypoplastic left heart syndrome (HLHS) is a critical congenital heart defect requiring complex surgical palliation.

Purpose of the Study:

  • To investigate the association between ACEI use and interstage failure in infants with HLHS.
  • To evaluate the impact of ACEIs on adverse outcomes between the first and second stages of surgical palliation.
  • To provide evidence-based guidance on ACEI therapy in HLHS management.

Main Methods:

  • Retrospective cohort study utilizing the National Pediatric Cardiology Quality Improvement Collaborative database (2008-2015).
  • Logistic regression models and propensity score matching were employed to analyze exposure-outcome associations and control for confounding variables.
  • Inclusion criteria comprised neonates diagnosed with hypoplastic left heart syndrome.

Main Results:

  • The study included 1,487 neonates; 39% received ACEIs post-Norwood procedure.
  • Interstage failure (death, transplant, or ineligibility for Stage 2 surgery) occurred in 11% of patients.
  • While unadjusted analysis showed higher failure rates with ACEIs (OR=1.44), propensity score matching revealed no significant difference (aOR=1.29, P=0.18).

Conclusions:

  • ACEI therapy did not demonstrate a significant beneficial effect on reducing interstage failure in infants with HLHS.
  • Even after accounting for patient characteristics, ACEIs did not improve outcomes in this high-risk population.
  • Further research may be needed to explore alternative or adjunctive therapies for optimizing interstage care in single ventricle physiology.
Abstract

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