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Paeds Topicsfetal-neonatal-and-perinatal

Paeds · fetal-neonatal-and-perinatal

Persistent pulmonary hypertension of the newborn

Also known as Persistent pulmonary hypertension of the newborn

Fellowship guide to persistent pulmonary hypertension of the newborn.

high8 referencesUpdated 11 July 202611 min readVerification in progress

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RACP DWEMRCPCH Theory

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  • Missed or delayed diagnosis is the primary risk

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fetalneonate

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Target exams

RACP DWEMRCPCH Theory

Red flags

  • Missed or delayed diagnosis is the primary risk

Life stages

fetalneonate

Care settings

delivery-roomnicu

Clinical exam formats

written-only

Board mappings

  • Persistent pulmonary hypertension of the newborn
  • Long case: persistent pulmonary hypertension of the newborn
  • Communication: persistent pulmonary hypertension of the newborn
  • Patient Care: persistent pulmonary hypertension of the newborn
  • Medical Expert: persistent pulmonary hypertension of the newborn

Overview & Definition

A term infant born after meconium-stained liquor grunts, tachypnoeas, and turns blue within the first hour of life despite supplemental oxygen. This is the classic presentation of persistent pulmonary hypertension of the newborn (PPHN), one of the most time-critical diagnoses in neonatology. [1]

PPHN arises when the pulmonary vascular resistance fails to fall normally after birth. Blood continues to bypass the lungs through fetal channels — the foramen ovale, ductus arteriosus, and intrapulmonary shunts — producing profound, often refractory hypoxaemia. The condition carries a mortality of 4–33% and survivors face substantial neurodevelopmental risks. [1]

What defines PPHN at the bedside?

The hallmark is a right-to-left shunt that persists after birth, evidenced by a pre-ductal SpO₂ (right hand) that exceeds the post-ductal SpO₂ (left foot or lower limb) by more than 10%. Pulmonary vascular resistance remains above systemic vascular resistance, so deoxygenated blood is shunted away from the lungs through the ductus arteriosus or foramen ovale. [1]

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References8ShowHide
  1. [1]Singh Y Pathophysiology and Management of Persistent Pulmonary Hypertension of the Newborn Clin Perinatol, 2021.PMID 34353582
  2. [2]Chambers CD Selective serotonin-reuptake inhibitors and risk of persistent pulmonary hypertension of the newborn N Engl J Med, 2006.PMID 16467545
  3. [3]Christou H Inhaled nitric oxide reduces the need for extracorporeal membrane oxygenation in infants with persistent pulmonary hypertension of the newborn Crit Care Med, 2000.PMID 11098980
  4. [4]Abman SH Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society Circulation, 2015.PMID 26534956
  5. [5]Finer NN Nitric oxide for respiratory failure in infants born at or near term Cochrane Database Syst Rev, 2006.PMID 17054129
  6. [6]Martinho S Persistent Pulmonary Hypertension of the Newborn: Pathophysiological Mechanisms and Novel Therapeutic Approaches Front Pediatr, 2020.PMID 32850518
  7. [7]Siefkes HM Management of systemic hypotension in term infants with persistent pulmonary hypertension of the newborn: an illustrated review Arch Dis Child Fetal Neonatal Ed, 2021.PMID 33478959
  8. [8]Fei Q Comparison of Different Treatments of Persistent Pulmonary Hypertension of the Newborn: A Systematic Review and Network Meta-Analysis Crit Care Med, 2024.PMID 38363176

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