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Inhaled epoprostenol in PPHN


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Just wanted to share a recent and good experience with inhalation of epoprostenol in PPHN.

I currently work in a large level2-unit (≈8000 inborn/y) with no access to NO-inhalation. Infants born in our delivery ward with PPHN and needing level-3-care (i.e. NO/mechanical ventilation) needs to be transferred.

 

We recently had a baby with echo-verified PPHN, on CPAP and with saturations around 88-90% on 100% oxygen.

 

While preparing for premed/relaxation and intubation we connected our CPAP inhalation device and inhaled epoprostenol with surprisingly good response! We avoided intubation and transfer. We gave two inhalations about 30 min apart initially, and shortly our saturations were >95% and could start to decrease FiO2. Doses were repeated every 2nd hour until we reached  FiO2 <0.5, in total the baby recieved five inhalations. Before/during and after - the baby was cardiovascular stable (stable normal BP and pulse) and the 2nd echo at about 4h of age showed balanced shunt over the open duct (as opposed to right-left shunting at 1.5h after birth).

 

We used the dose suggested in this article:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3342750/

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Parents were informed of course. We also considered we could use epoprostenol off-label (like how we use most other drugs in the NICU) as others had used and reported about doses and responses in publications available on Pubmed.

 

In addition to case reports in this review from 2014 (http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3942674/), there are several case-reports and -series in the literature:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3342750/

http://pediatrics.aappublications.org/content/130/2/e442.long

http://www.ncbi.nlm.nih.gov/pubmed/12461501

 

While doing some research on this option last year I also found this news story from a hospital in California, not a peer-reviewed paper but adds a perspective also.

http://www.childrenscentralcal.org/PressRoom/IncredibleCare/Pages/ReducingCosts.aspx

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May i ask how much is the cost of this medication ?

Is it same like ponsenten orally

 

I don't know the cost. But we have the drug "in stock" as we sometimes (rarely) use it iv (http://www.ncbi.nlm.nih.gov/pubmed/8960484).

However, as our experience with iv-use is not good we have tried inhalation in a few cases using the prepared iv-solution (1000 nanogram/ml). As the inhaled dose (suggested in article above) is 50 ng/kg, we have added with NaCl up to a volume of 2 ml.

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