This handout is for persistent pulmonary hypertension of the newborn (pphn). Your care team identified this based on: pre-/post-ductal spo₂ gradient > 10% (or pao₂ gradient > 20 mmhg) in term / near-term neonate — classic pphn sign.
Other reasons your team may use this plan: severe hypoxemia in term / near-term neonate with clear or minimally abnormal cxr — pure pphn phenotype; echo showing elevated rv pressure (tr jet), flat septum, r→l shunting across pda / pfo — pphn diagnostic anchor (steinhorn body of work); pphn drivers: meconium aspiration, neonatal pneumonia, asphyxia, congenital diaphragmatic hernia, late-preterm rds, idiopathic.
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| inhaled_nitric_oxide_iNO_20_ppm | iNO 20 ppm via ventilator circuit; titrate down 5 ppm q4h based on OI response; minimum 5 ppm to avoid rebound; wean off over 12-24 h | inhaled_via_ventilator | continuous inhaled | First-line per Cochrane Barrington 2017 (PMID 28056166): iNO 20 ppm effective in term / near-term hypoxic respiratory failure without diaphragmatic hernia; reduces death + ECMO with NNT ~ 5. CDH cohort has reduced response (consider earlier ECMO). Composite procedural (medical gas, not a single RxCUI for inhalation form) |
| sildenafil | sildenafil 0.5-1 mg/kg PO q6h (start at 0.5 mg/kg; titrate to response); IV form 0.4 mg/kg over 3 h then 1.6 mg/kg/day continuous (Steinhorn J Pediatr 2009 PMID 19836028) | PO (preferred) or IV | q6h (PO) | PDE5 inhibition potentiates cGMP → pulmonary vasodilation; adjunct to iNO and weaning aid; Steinhorn et al J Pediatr 2009 (PMID 19836028) IV form well-tolerated. Lactation: limited data (Neofax 2024 caution); IV form not lactation-relevant (infant-administered) |
| milrinone | milrinone 0.25-0.75 mcg/kg/min IV continuous (start at 0.25; titrate); optional 50 mcg/kg loading dose over 30-60 min (consider omitting in hypotensive infants) | IV continuous | continuous infusion | Inotropic + vasodilatory; especially useful in PPHN with LV dysfunction or post-bypass; monitor BP closely (hypotension common); Neofax 2024 |
| dobutamine | dobutamine 5-20 mcg/kg/min IV continuous (start at 5; titrate to cardiac output and BP response) | IV continuous | continuous infusion | Beta1 inotropic + mild pulmonary vasodilator; supports cardiac output without aggressive afterload increase; Neofax 2024 |
| poractant alfa | poractant alfa 200 mg/kg intratracheal (same dosing as RDS engine) | intratracheal_LISA_or_INSURE_or_ETT | single dose; may repeat 100 mg/kg if persistent need | Parenchymal driver (MAS, pneumonia, RDS) requires surfactant; cross-reference neonatal.respiratory-distress-syndrome.v1; Sweet 2022 + Neofax 2024 |
| hfov_high_frequency_oscillatory_ventilation | HFOV with optimal MAP titrated to lung volume on CXR; frequency 8-12 Hz typical | mechanical_ventilation | continuous | HFOV improves lung-recruitment in MAS / pneumonia / severe RDS-PPHN overlap; lung-protective for PPHN with parenchymal disease |
| ecmo_extracorporeal_membrane_oxygenation | VV or VA ECMO per institutional protocol; weight ≥ 2 kg + GA ≥ 34 wk + reversible cause + no major CNS injury | extracorporeal_VV_or_VA | continuous until reversibility | ELSO neonatal guidelines (2023) — ECMO improves survival in OI ≥ 40 with reversible cause; CDH cohort has earlier ECMO threshold |
Plan: PPHN — iNO + sildenafil + milrinone + dobutamine + surfactant for parenchymal driver; HFOV + ECMO non_pharm (Cochrane Barrington 2017 PMID 28056166; Steinhorn IV sildenafil PMID 19836028; BPD-PH Berkelhamer Steinhorn 2018 PMID 30384985)
High-risk follow-up clinic at 3-6-12-24 mo (Bayley III, neurodev). Hearing screen (AABR) before discharge + audiology surveillance (PPHN + iNO + ECMO survivors at risk for sensorineural hearing loss). Echo at 1-3-6 mo if persistent PH suspected (route to BPD-PH evaluation per Berkelhamer Steinhorn 2018 PMID 30384985). Pulmonology follow-up if BPD or chronic lung disease. RSV prophylaxis (nirsevimab) per AAP / ACIP.
Guideline: Berkelhamer SK, Mestan KK, Steinhorn RH — BPD-Associated PH Update (Semin Perinatol 2018 PMID 30384985, Steinhorn co-author body of work on PPHN); Cochrane Barrington 2017 (PMID 28056166) iNO for term / near-term hypoxic respiratory failure; Steinhorn IV sildenafil (J Pediatr 2009 PMID 19836028) for adjunct + weaning.