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Sildenafil weaning after discharge in infants with congenital diaphragmatic hernia

Joanna Behrsin1, Michael Cheung, Neil Patel

  • 1Newborn Intensive Care Unit, The Royal Children's Hospital, Melbourne, VIC, Australia, jmbehrsin@hotmail.com.

Pediatric Cardiology
|July 24, 2013
PubMed

Insights

Sildenafil use in infants with congenital diaphragmatic hernia (CDH) and pulmonary hypertension (PAH) varies widely. Standardized dosing and weaning protocols are needed to optimize treatment and minimize risks.

Area of Science:

  • Pediatric Cardiology
  • Neonatology
  • Pharmacology

Background:

  • Sildenafil is prescribed for pulmonary hypertension (PAH) in infants with congenital diaphragmatic hernia (CDH).
  • Limited data exist for sildenafil dosing and weaning in this population.
  • Concerns arise from reports of increased risk with high-dose sildenafil in non-CDH PAH.

Purpose of the Study:

  • To analyze sildenafil usage, dosing, and weaning patterns in infants with CDH.
  • To identify variations in current clinical practice.
  • To inform the development of standardized treatment guidelines.

Main Methods:

  • Retrospective cohort study.
  • Analysis of sildenafil administration, dosage, and discontinuation in infants with CDH.
  • Evaluation of dosing and weaning rates.

Main Results:

  • 17% of infants were discharged on sildenafil (median dose 8 mg/kg/day).
  • Weaning occurred at a median rate of 0.1 mg/kg/week.
  • 29% of infants remained on sildenafil above 1.5 mg/kg/day at 1 year; one infant died from severe PAH.

Conclusions:

  • Sildenafil therapy at discharge is common for severe CDH.
  • Significant variation in dosing and weaning rates necessitates standardized protocols.
  • Standardization aims to optimize benefits and minimize adverse effects of sildenafil in CDH-PAH.

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