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Persistent Pulmonary Hypertension of Newborn

PPHN is severe hypoxemic respiratory failure due to failure of pulmonary vascular resistance to fall after birth.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Persistent Pulmonary Hypertension of Newborn?

Persistent pulmonary hypertension of the newborn (PPHN) is the failure of fetal high pulmonary vascular resistance (PVR) to fall after birth. Incidence 1.9/1000 live births; mortality 4-33% (decreased with iNO+ECMO). Most common in late preterm and term newborns.

Etiology: idiopathic (vascular remodeling), perinatal asphyxia, meconium aspiration syndrome (MAS), congenital diaphragmatic hernia (CDH), pneumonia/sepsis, RDS, congenital alveolar dysplasia. Pathophysiology: increased PVR → right-to-left shunt at PDA + PFO → severe hypoxemia.

Clinical: respiratory distress, cyanosis (saturation difference between right hand and lower extremity ≥5%), hypoxemia (PaO₂ <50 despite FiO₂ 1.0), oxygenation index (OI) >25. Diagnosis: echocardiogram (high RV pressure, right-to-left shunt). Treatment: O₂, mechanical ventilation (HFOV), surfactant, iNO 20 ppm (gold standard), sildenafil PO 0.5-2 mg/kg/dose q6h, milrinone, ECMO if OI >40.

Symptoms

Severe respiratory distress (after birth)
Central cyanosis, oxygen saturation <90%
Tachypnea, retractions, grunting
Pulse oximetry difference (right hand vs lower extremity ≥5%)
Cardiac murmur (TR, PDA)
Lethargy, poor feeding

Risk Factors

Meconium aspiration syndrome (MAS)
Perinatal asphyxia (low Apgar)
Congenital diaphragmatic hernia (CDH)
Congenital pneumonia, sepsis
Maternal SSRI use
Polyhydramnios, oligohydramnios

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Cyanosis after birth (urgent)
  • Severe respiratory distress (urgent)
  • Oxygen saturation <90% (urgent)
  • MAS history
  • CDH antenatal diagnosis
  • Hydrops fetalis

Treatment Methods

01
Oxygen + mechanical ventilation (HFOV)
02
Surfactant (RDS, MAS)
03
Inhaled nitric oxide (iNO) 20 ppm (gold standard)
04
Sildenafil PO 0.5-2 mg/kg q6h
05
Milrinone (LV dysfunction)
06
ECMO (OI >40, refractory)

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Çocuk Sağlığı ve Hastalıkları Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.