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Persistent Pulmonary Hypertension

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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Persistent Pulmonary Hypertension

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  1. Persistent Pulmonary Hypertension

  2. 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

  3. Let’s start at the beginning • Three shunts • Ductus venosus • Foreman Ovale • Ductus Arteriosus

  4. Delivery Changes Everything • Exposure of lungs to oxygen • Pulmonary vasculature dilates • Closure of ductus arteriosus • Closure of foramen ovale

  5. Etiologies • Acute Pulmonary Vasoconstriction • Pulmonary Bed Hypoplasia • Idiopathic Pulmonary Hypertension

  6. Etiologies • Acute Pulmonary Vasoconstriction due to acute perinatal events • Parenchymal lung disease (MAS leading cause of PPHN) • Hypoventilation due to asphyxia • Hypothermia • hypoglycemia

  7. Etiologies • Pulmonary Bed Hypoplasia • Diaphragmatic hernia • Oligohydramios secondary to PPROM • Congenital malformation of the lungs

  8. 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

  9. Physical Exam • Cyanosis • Respiratory distress • Loud, single S2 or harsh systolic murmur • Poor perfusion • Pre- and Post- ductal gradient

  10. Differential Diagnosis • Diaphragmatic Hernia • Congenital Heart Lesion • Meconium Aspiration • Pneumonia • Pulmonary hypoplasia • Pneumothorax

  11. Work Up • ABG • Oxygen challenge test • CBC • Glucose • CXR • Echocardiography

  12. X-ray Can Reflect Underlying Pathology MAS GBS

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