What is the role of prostacyclin in the treatment 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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Prostacyclin is a vascular endothelium–derived product of arachidonic acid metabolism with potent vasodilatory activity. It also has inhibitory effects on platelet aggregation, inflammation, and vascular smooth muscle proliferation. Inhaled prostacyclin (epoprostenol) may act synergistically with iNO to cause effective pulmonary vasodilation. It has been used successfully in older patients with pulmonary hypertension but not commonly for PPHN. Treatment with epoprostenol in a randomized control trial has shown improvements over placebo in exercise capacity as assessed by the distance walked in 6 minutes, quality of life, pulmonary hemodynamics, and survival in idiopathic pulmonary hypertension. [47]  In a 2018 retrospective study (2004-2016) that evaluated the efficacy of inhaled epoprostenol in neonates with PPHN, investigators noted a significant improvement in the oxygen index after 12 hours of treatment, followed by a rebound effect with discontinuation but no potential side effects. [48]

Its use requires permanent vascular access as it has very short half life (~5 min), and any abrupt interruption in its delivery due to catheter dislodgment, blockage, or leak may result in potentially fatal rebound pulmonary hypertension. Its effect can wean overtime owing to the phenomenon of tachyphylaxis. [49] Commonly observed adverse effects are due to systemic vasodilation and include headache, dizziness, facial flushing, jaw pain, leg cramps, and gastrointestinal upset.

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