Pediatrics

Pulmonary Hypertensive Crisis

Diagnosis

  • Acute ↓ BP, ↓ EtCO2, ↑ CVP
  • ↓ O2 saturation, may not be immediate in the absence of an intracardiac communication
  • ↑ airway pressures due to pulmonary artery distension & compression of small intrapulmonary airways
  • ↑ V/Q mismatch & Vd/Vt with subsequent hypoxemia due to ↓ pulmonary blood flow & small airway compression
  • RV diastolic hypertension → reduced LV filling and ↓ cardiac output
  • Bradycardia → Hypotension → Cardiac arrest

Diagnosis

  • Acute ↓ BP, ↓ EtCO2, ↑ CVP
  • ↓ O2 saturation, may not be immediate in the absence of an intracardiac communication
  • ↑ airway pressures due to pulmonary artery distension & compression of small intrapulmonary airways
  • ↑ V/Q mismatch & Vd/Vt with subsequent hypoxemia due to ↓ pulmonary blood flow & small airway compression
  • RV diastolic hypertension → reduced LV filling and ↓ cardiac output
  • Bradycardia → Hypotension → Cardiac arrest

  1. Initial Management

    Give 100% O2

    • Call for inhaled nitric oxide (iNO) 20-40 ppm
    • ↓ O2 saturation may not be immediate
    • 20-40 ppm

  2. Consider stat TEE and ECMO

  3. Deepen anesthetic/sedation, consider fentanyl 1 MICROgram/kg or ketamine 0.5-1 mg/kg

    • 1 MICROgram/kg
    • 0.5-1 mg/kg

  4. Administer muscle relaxant

  5. If poor perfusion, consider chest compressions early

  6. Hypotension Management

    If hypotensive, give vasopressin 0.03 units/kg bolus, then:

    • To maintain perfusion:
    • Vasopressin 0.17-0.67 milliunits/kg/minute = 0.01-0.03 units/kg/hour
    • OR
    • norepinephrine 0.05-0.3 MICROgrams/kg/min
    • 0.03 units/kg
    • Then infusion 0.17-0.67 milliunits/kg/minute = 0.01-0.03 units/kg/hour
    • 0.05-0.3 MICROgrams/kg/min

  7. Ventilation

    Ventilate with low airway pressures & long expiratory phase to maintain adequate tidal volume, avoid atelectasis and preserve FRC

  8. Maintain normocapnia or mild hypocapnia

  9. PEEP may worsen pulmonary hypertension

  10. Further Management

    Administer isotonic fluid judiciously to achieve normovolemia and to reduce acid load, correct acidosis with sodium bicarbonate

  11. Maintain NSR and AV synchrony

  12. Temperature: ensure normothermia

  13. Crisis Management

    If CARDIAC ARREST occurs or is imminent, give epinephrine 1-10 MICROgrams/kg

    • 1-10 MICROgrams/kg IV/IO

  14. If CARDIAC ARREST occurs, begin CPR and call for ECMO as CPR may be ineffective if no intracardiac communication

  15. Crisis Management (If Severe)

    Notify surgeon, call for help and code cart

  16. Call for Transesophageal Echocardiography

  17. Start chest compressions

  18. Give epinephrine 10 MICROgrams/kg IV/IO

    • 1-10 MICROgrams/kg IV/IO

  19. If cardiac arrest, go to CARDIAC ARREST

  20. Consider ECMO

Patient weight

Between 0.5 kg and 120 kg

Reset the checklist?

Checked steps, the timer and this crisis log are cleared. The patient’s weight is kept.

Finish the crisis?

The timer pauses, and you can review the case and the event log.