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Current evidence for pharmacologic therapy following stage 1 palliation for single ventricle congenital heart disease

Meredith C G Broberg1, Ira M Cheifetz1,2, Sarah T Plummer2

  • 1Department of Pediatrics, Division of Pediatric Cardiac Critical Care, University Hospitals Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA.

Expert Review of Cardiovascular Therapy
|July 18, 2022
PubMed

Insights

Infants with single ventricle heart disease need careful medication management between surgical stages. Digoxin and aspirin show promise for improving outcomes in this vulnerable interstage period.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Pharmacology

Background:

  • Infants with single ventricle congenital heart disease face critical risks between surgical palliation stages.
  • Current pharmaceutical strategies for this interstage period lack robust evidence and vary by institution.

Purpose of the Study:

  • To review the medical management of infants in the interstage period after Stage I palliation.
  • To evaluate the evidence for angiotensin-converting enzyme inhibitors and digoxin in this population.

Main Methods:

  • A scoping review of existing literature on interstage pharmaceutical treatment.
  • Focused review on angiotensin-converting enzyme inhibitors and digoxin.
  • Discussion of other commonly used medication classes.

Main Results:

  • Limited evidence supports many interstage medications due to disease heterogeneity and confounding factors.
  • Digoxin is associated with improved interstage mortality but lacks large randomized controlled trials.
  • Aspirin for thrombotic complication prevention is linked to better outcomes.

Conclusions:

  • Digoxin and aspirin should be considered for interstage management when appropriate.
  • Careful risk-benefit assessment is crucial before adding other medications, mindful of polypharmacy risks.
  • Further research is needed to establish evidence-based pharmaceutical guidelines for single ventricle heart disease palliation.
Abstract

Keywords:
Congenital heart diseaseNorwood procedurehypoplastic left heart syndromeinfantinterstageneonatepediatric cardiologysingle ventriclestage 1 palliation

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