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Semin Perinatol. 2014 Mar;38(2):78-91. doi: 10.1053/j.semperi.2013.11.004.

Update on PPHN: mechanisms and treatment.

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  • 1Center for Developmental Biology of the Lung, State University of New York, Buffalo, NY.
  • 2Center for Developmental Biology of the Lung, State University of New York, Buffalo, NY; Division of Neonatology, Department of Pediatrics, Women and Children's Hospital of Buffalo, 219 Bryant St, Buffalo, NY 14222. Electronic address:


Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of failed circulatory adaptation at birth, seen in about 2/1000 live born infants. While it is mostly seen in term and near-term infants, it can be recognized in some premature infants with respiratory distress or bronchopulmonary dysplasia. Most commonly, PPHN is secondary to delayed or impaired relaxation of the pulmonary vasculature associated with diverse neonatal pulmonary pathologies, such as meconium aspiration syndrome, congenital diaphragmatic hernia, and respiratory distress syndrome. Gentle ventilation strategies, lung recruitment, inhaled nitric oxide, and surfactant therapy have improved outcome and reduced the need for extracorporeal membrane oxygenation (ECMO) in PPHN. Newer modalities of treatment discussed in this article include systemic and inhaled vasodilators like sildenafil, prostaglandin E1, prostacyclin, and endothelin antagonists. With prompt recognition/treatment and early referral to ECMO centers, the mortality rate for PPHN has significantly decreased. However, the risk of potential neurodevelopmental impairment warrants close follow-up after discharge for infants with PPHN.

© 2013 Published by Elsevier Inc.


Hypoxic respiratory failure; Nitric oxide; Persistent fetal circulation; Pulmonary vascular resistance; Systemic vasodilators

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