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Persistent Pulmonary Hypertension. What is PPHN?. “Failure of the postnatal fall in pulmonary vascular resistance resulting in left-to-right shunting of deoxygenated blood at the atrial, ductal, and pulmonary levels with consequent severe hypoxemia” Boden and Bennett, 2004.

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Presentation Transcript
what is pphn
What is PPHN?

“Failure of the postnatal fall in pulmonary vascular resistance resulting in left-to-right shunting of deoxygenated blood at the atrial, ductal, and pulmonary levels with consequent severe hypoxemia”

Boden and Bennett, 2004

let s start at the beginning
Let’s start at the beginning
  • Three shunts
    • Ductus venosus
    • Foreman Ovale
    • Ductus Arteriosus
delivery changes everything
Delivery Changes Everything
  • Exposure of lungs to oxygen
  • Pulmonary vasculature dilates
  • Closure of ductus arteriosus
  • Closure of foramen ovale
etiologies
Etiologies
  • Acute Pulmonary Vasoconstriction
  • Pulmonary Bed Hypoplasia
  • Idiopathic Pulmonary Hypertension
etiologies1
Etiologies
  • Acute Pulmonary Vasoconstriction due to acute perinatal events
    • Parenchymal lung disease (MAS leading cause of PPHN)
    • Hypoventilation due to asphyxia
    • Hypothermia
    • hypoglycemia
etiologies2
Etiologies
  • Pulmonary Bed Hypoplasia
    • Diaphragmatic hernia
    • Oligohydramios secondary to PPROM
    • Congenital malformation of the lungs
etiologies3
Etiologies
  • Idiopathic Pulmonary Hypertension
    • Fetal constriction of the ductus arteriosus in utero
    • Remodeling of the fetal pulmonary vasculature in response to fetal stress, hypoxia, or pulmonary hypertension
physical exam
Physical Exam
  • Cyanosis
  • Respiratory distress
  • Loud, single S2 or harsh systolic murmur
  • Poor perfusion
  • Pre- and Post- ductal gradient
differential diagnosis
Differential Diagnosis
  • Diaphragmatic Hernia
  • Congenital Heart Lesion
  • Meconium Aspiration
  • Pneumonia
  • Pulmonary hypoplasia
  • Pneumothorax
work up
Work Up
  • ABG
  • Oxygen challenge test
  • CBC
  • Glucose
  • CXR
  • Echocardiography