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Persistent Pulmonary Hypertension of the Newborn, Pediatric

Jennifer J. Miller, MD and Temitope Akinmboni, MBBS Reviewed 10/2018
 


BASICS

DESCRIPTION

Clinical syndrome characterized by hypoxia and respiratory failure in a neonate due to a failure of the pulmonary vascular resistance (PVR) to decrease after birth, resulting in high...

DIAGNOSIS

HISTORY

  • Pregnancy history

    • History of oligohydramnios, which may be associated with pulmonary hypoplasia in the neonate

  • Problems during labor and delivery

    • Events that can cause fetal distress and...

TREATMENT

GENERAL MEASURES

  • All infants should be transferred to a level IV neonatal intensive care unit where high-frequency ventilation (HFV) and inhaled nitric oxide (iNO) are available. If the neona...

ONGOING CARE

PROGNOSIS

  • PPHN usually resolves either spontaneously or as the underlying parenchymal lung disease improves.

  • Survival rate is good even for neonates who receive ECMO. Survival rate and inci...

ADDITIONAL READING

  • Abman SH, Hansmann G, Archer SL, et al. Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation.  2015;132(2...

CODES

ICD9

747.83 Persistent fetal circulation 

ICD10

P29.3 Persistent fetal circulation 

SNOMED

233815004 persistent pulmonary hypertension of the newborn (disorder) 

FAQ

  • Q: Does iNO improve outcome in newborns with severe PPHN?

  • A: Yes. Although iNO has not been shown to reduce mortality, it has been shown to decrease the need for ECMO by 40% and reduce the length o...

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