P E R S I S T E N T P U L M O N A R Y H Y P E R T E N S I O N O F T H E N E W B O
R N ( P P H N ) / P E R S I S T E N T F E T A L C I R C U L A T I O N ( P F C )
INTRODUCTION:
Persistent pulmonary hypertension of the newborn (PPHN) occurs when the pulmonary
vascular resistance (PVR) fails to decrease at birth. It can occur as a complication of
lung diseases that cause respiratory failure in the newborn. These include perinatal
aspiration syndrome, pneumonia or respiratory distress syndrome. PPHN can also
occur by itself as idiopathic PPHN without associated parenchymal lung disease. PPHN
accounts for 10% of admissions to tertiary care NICUs and can range in spectrum from
mild and transient to severe or chronic pulmonary hypertension. PPHN is also a
common complication of lung hypoplasia which occurs with space occupying anomalies
in the chest, such as congenital diaphragmatic hernia or with oligohydramnios due to
prolonged leakage of amniotic fluid or renal dysplasia/agenesis. Finally, PPHN is a
complication of some lethal causes of respiratory failure such as alveolar-capillary
dysplasia (ACD) and genetic defects in surfactant synthesis. MAS accounts for majority
of cases (40%), followed by Pneumonia/sepsis (15%) and RDS (15%) and idiopathic
PPHN accounts for approximately a third of the cases (27%).
Infants who survive neonatal PPHN are at an increased risk for long term neuro-
developmental impairments and/or late hearing loss and need careful follow-up after
discharge.