Infant with Congenital Diaphragmatic Hernia, Pre and Post-operative Care — Universal Care Before Surgical Repair: Pulmonary Hypertension — Clinical Pathway: Inpatient and ICU

Infant with Congenital Diaphragmatic Hernia Clinical Pathway, Pre and Post-operative Care — Inpatient and ICU

Universal Care for Infants with CDH Before Surgical Repair: Pulmonary Hypertension

Goals

  • Resolved or non-reactive pulmonary hypertension
  • Normal right heart function
Assess Severity of PH
  • Consult PH Cardiology
  • Evaluate clinical signs and symptoms for baseline severity
    • e.g., FiO2, Echo findings, tolerance of care without desaturations, shunting, hypercarbia, hemodynamic instability
  • PH spells are severe cyanotic episodes (pre and post-ductal saturation < 85%) or ductal shunting events (saturation difference more than 10% pre and post-ductal)
Environment
  • Create environment of minimal stimulation
    • Lights off, earmuffs, eye shield, cluster care, no bath, quiet voices
  • Provide adequate and appropriate sedation to prevent severe cyanotic or ductal shunting events
  • Goal Hgb 12-14 g/dL
Echo
  • Obtain early sedated (procedural fentanyl +/- vecuronium) Echo with hospital provider or attending present for infants with:
    • Left CDH with liver up
    • Right CDH
    • Respiratory/hemodynamic instability
  • Consider repeat Echos to monitor response to interventions as indicated
  • Unstable or Severe CDH
    • Imaging focus
      • Left heart or arch hypoplasia
      • Ductal restriction
      • Presence of left SVC
      • Direction of shunting at PDA and PFO
      • Ventricular function
    • Note: Continue w/full Echo as infant able to tolerate
  • Stable Infants
    • Complete Echo as infant tolerates