Infant with Congenital Diaphragmatic Hernia, Pre and Post-operative Care — Universal Care Before Surgical Repair, Hemodynamic Stabilization — 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: Hemodynamic Stabilization Before CDH Surgical Repair

Goals

  • Stable hemodynamics
  • Meet physiologic targets for surgical repair

Considerations for Initiating Vasoactive Infusion Therapy

  • Assess:
    • Heart rate and blood pressure
    • Perfusion on physical exam, base deficit, lactate
    • Review/obtain Echo to assess function
  • Correct:
    • Acidosis, electrolyte abnormalities

Guidance on Pre-ECMO, Pre-Repair Hemodynamics

Primary RV Dysfunction Primary LV Dysfunction and Biventricular Dysfunction
  1. Initiate epinephrine 0.03 mcg/kg/min and titrate as needed up to 0.1 mcg/kg/min
  2. Initiate hydrocortisone 1 mg/kg/dose every 6 hrs
  3. Start iNO at 20 ppm (pre-ductal saturations < 85%, +/- shunting by 10%, significant increase in FiO2)
  4. Consider alprostadil (PGE-1) if ductus restrictive (< 24 hrs) or if right ventricular failure (> 24 hrs)
  5. Consider low dose dopamine 3-5 mcg/kg/min; avoid
    > 7 mcg/kg/min in the setting of biventricular dysfunction
  6. Discuss additional PH meds with PH Cardiology
  1. Initiate epinephrine 0.03 mcg/kg/min and titrate as needed up to 0.1 mcg/kg/min
  2. Initiate hydrocortisone 1 mg/kg/dose every 6 hrs
  3. If escalating epinephrine > 0.05 mcg/kg/min, consider adding vasopressin at 6 milliunits/kg/hr
    Increase by 6 milliunits/kg/hr as needed to a max dose of 24 units/kg/hr
    • Consider milrinone 0.25 mcg/kg/min in discussion with PH Cardiology if adequate BP on non-escalating doses after
      24 hrs of life
General Management
  • Optimize lung recruitment to avoid atelectasis or overdistension
    • May include decreasing MAP or PEEP
  • If metabolic acidosis, especially if base deficit > -5
    • Consider sodium bicarbonate bolus or sodium acetate rider
  • Optimize sedation and consider neuromuscular blocking agent
  • Epinephrine for hypotensive crisis (1 mcg/kg/dose), can be used as an adjunct if needed for maintenance of perfusion just prior to or during ECMO cannulation
  • Order from Neonatology Emergency Medication Order Set
Miscellaneous
  • VA ECMO considered once > 40 mL/kg of fluid administered
  • Epinephrine for hypotensive crisis can be used as an adjunct if needed for maintenance of perfusion just prior
    to and during ECMO cannulation
    • Dose: 1 mcg/kg/dose
  • Order from Neonatology Emergency Medication Order Set

Medication Considerations

Vasopressin
  • Will increase LV afterload by its effects on systemic vascular resistance so there is potential to worsen LV function and cardiac output, especially with higher doses. May be useful in infants with tachycardia (HR > 160) as it is catecholamine sparing.
  • Should be used in conjunction with inotropic support and frequent POCUS/Echo to evaluate LV function.
  • Hyponatremia is an expected side effect and requires sodium supplementation. Follow serum and urine sodium closely. May limit use if hyponatremia is significant despite supplementation.
iNO Avoid until LV function has recovered, especially if there is left to right shunting at the PFO and right to left shunting at the PDA.
Alprostadil (PGE-1)
  • Limit use of alprostadil (PGE-1) in first 24 hrs – if initiating can consider lower dose 0.005 mcg/kg/hr given risk of hypotension.
  • Consider in the face of a concern for coarctation or a restrictive ductus with right ventricular dilation and/or failure. Ideally wait until > 24 hrs before initiating.
  • Team discussion with neonatologist and PH cardiologist prior to initiating.
  • Given the systemic vasodilation and hypotension associated with alprostadil
    (PGE-1), ensure intravascular volume status is replete and mean BP is adequate with or without vasoactive infusions.
  • Moderate PDA: repeat ECHO in 12-24 hrs to re-assess size to determine need for alprostadil (PGE-1).
Milrinone
  • Initiate without a loading dose.
  • Renally cleared: In the setting of impaired renal function and oliguria, increased risk of systemic adverse effects such as hypotension.