Persistent Pulmonary Hypertension of the Newborn

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

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II. Epidemiology

  1. Incidence: 2 per 1000 live births

III. 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

IV. Causes

  1. Meconium Aspiration Syndrome
  2. Neonatal Pneumonia
  3. Neonatal Sepsis
  4. Respiratory distress syndrome of the newborn
  5. Diaphragmatic Hernia
  6. Pulmonary hypoplasia

V. Risk Factors

  1. Gestational Diabetes
  2. Cesarean delivery
  3. Maternal Obesity
  4. Black race
  5. Maternal SSRI use (low risk)

VI. Diagnostics

  1. Echocardiogram

VII. Signs

  1. See Neonatal Respiratory Distress
  2. Systolic Murmur
  3. Loud Second Heart Sound
  4. Hypoxia
    1. Improves with Supplemental Oxygen

VIII. 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

IX. References

  1. Hermansen (2015) Am Fam Physician 92(11): 994-1002 [PubMed]

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