What is the role of idiopathic pulmonary hypertension in the etiology of persistent pulmonary hypertension of the newborn (PPHN)?

Updated: Sep 03, 2019
  • Author: Kate A Tauber, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Idiopathic pulmonary hypertension accounts for approximately 10% of the cases of PPHN. It is caused by impaired pulmonary relaxation after birth in the absence of parenchymal lung disease. One cause of idiopathic PPHN is constriction, or premature closure of the ductus arteriosus in utero, which can occur after exposure to aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs) (eg, ibuprofen, naproxen) during the third trimester. Evaluation of infants at autopsy shows significant remodeling of their pulmonary vasculature, with vascular wall thickening and smooth muscle hyperplasia. [23] Furthermore, the smooth muscle extends to the level of the intra-acinar arteries, which does not normally occur until late in the postnatal period. As a result, infants do not vasodilate their pulmonary vessels adequately in response to birth-related stimuli, and they present with hypoxemia and hyperlucent lung fields on radiography, which is termed black lung PPHN.

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