Jonathan M. Klein, MD
Peer Review Status: Internally Peer Reviewed

Exclusion Criteria:

  1. Neonates < 34 weeks gestation (NO inhibits platelet aggregation . Thus, it should be used with great caution in neonates <34 weeks and only at the discretion of the attending neonatologist.)
  2. Congenital heart disease (except incidental PDA, ASD, or VSD)

Enrollment Criteria (should fulfill all of the following):

  1. Diagnosis of persistent pulmonary hypertension of the newborn (PPHN)
  2. Sufficient cardiac evaluation to r/o congenital heart disease, may need echocardiogram to r/o structural disease
  3. Mean airway pressure of at least 12 - 15 cm H2O on HFOV (SensorMedics) with adequate inflation (9-rib expansion) to ensure delivery of NO.
  4. Arterial pH > 7.40 or if still acidemic despite vigorous attempts at pharmacologic alkalinization with adequate ventilation (PaCO2 ² 60 mm Hg).
  5. Pressor/Inotropic drug
  6. aDO2 ³ 600 mm Hg by 2 ABG's 30 minutes apart or PaO2 ² 70 mm Hg on FIO2 = 1.0. AaDO2 = PAO2 - PaO2, PaO2 = arterial PO2, PAO2 = alveolar PO2 = FiO2 (713) - PaCO2/0.8. 

Nitric Oxide (NO) Therapy (see NO-HFOV flow sheet):

  1. Initiate NO therapy, after meeting eligibility criteria and obtaining parental consent.
  2. Continue maximal conventional treatment.
  3. Start at 40 ppm nitric oxide for 1 hour. If PaO2 does not improve, increase to 80 ppm for 1 hour. After PaO2 improves (³ 70 mm Hg) decrease NO to 40 ppm, if PaO2 remains ³ 100-150 mm Hg, decrease NO to 20 ppm and maintain. If PaO2 continues to remain ³ 100-150 mm Hg for more than 24 h consider weaning NO to 10 ppm and maintain until shunting has resolved. If PaO2 drops below 60 mm Hg, restart NO at previous dose and maintain until shunting has resolved. Test for resolution of shunting every 2 to 3 days by stopping the NO for 10-15 minutes and checking the PaO2. Duration of NO therapy will vary with etiology of pulmonary hypertension.
  4. Follow methemoglobin (met-hgb) levels at 1, 2, and 4 hours then Q6h - 8hwhile on 40 ppm until met-hgb level is stable. If NO < 40 ppm follow met-hgb Q12h.
  5. Notify Dr. Jim Bates (#3830) of patient enrollment.
  6. The in-line concentration of NO will be continually analyzed by Respiratory Therapy unless if on Star on HFV then measured Q2h. If the concentration of NO measured is greater than ordered by 10% or less than ordered by more than 20%, the flow of NO will be readjusted.