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Persistent Pulmonary Hypertension of the Newborn

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Persistent Pulmonary Hypertension of the Newborn, PPHN, Persistent Fetal Circulation Syndrome

  • Epidemiology
  1. Incidence: 2 per 1000 live births
  • Pathophysiology
  1. Underlying lung or systemic conditions (see below) prevent transition from Fetal Circulation
  2. Increased pulmonary pressures lead to right-to-left shunt and Hypoxemia
  • Causes
  1. Meconium Aspiration Syndrome
  2. Neonatal Pneumonia
  3. Neonatal Sepsis
  4. Respiratory distress syndrome of the newborn
  5. Diaphragmatic Hernia
  6. Pulmonary hypoplasia
  • Risk Factors
  1. Gestational Diabetes
  2. Cesarean delivery
  3. Maternal Obesity
  4. Black race
  5. Maternal SSRI use (low risk)
  • Diagnostics
  1. Echocardiogram
  • Signs
  1. See Neonatal Respiratory Distress
  2. Systolic Murmur
  3. Loud Second Heart Sound
  4. Hypoxia
    1. Improves with Supplemental Oxygen
  • Management
  1. See Neonatal Respiratory Distress
  2. Consider N-CPAP or Endotracheal Intubation
  3. Consult neonatology
    1. Consider pulmonary vasodilators (Sildenafil, inhaled nitric oxide)
  4. ECMO has been used
  • References
  1. Hermansen (2015) Am Fam Physician 92(11): 994-1002 [PubMed]