Hi, A case of ex micropremmie with IUGR who has severe PPHN and BPD. Following a severe PPHN crisis experts suggested trial of Bosentan in additional to the sildenafil already started. further course of oral steroids also started. Worked well and weaned ventilation over two weeks.

I have minimal experience with Bosentan and wondered how much it is being used as an adjunct? Recognising used as a second line agent when things are in extremis. (no response to iNO). Questions such as typical length of courses being used? Successes? issues? no hepatic impairment at present.

Thanks!

I have no personal experience but bosentan briefly mentioned in our new national guideline about "hemodynamic problems in the newborn" and I also found some guideance in a regional guideline ( @Gustaf Lernfelt VG-region delivers!)

It is mentioned as an addition to "for example" sildenafil, and both sources warn for liver function impairment. One interesting aspect - seems bosentan couple to two receptors, both endothelin-a and endothelin-b , and those mediate opposite effects (ET-a vasoconstrictive and ET-b vasodilatory). So, a paradoxal effect early in the treatment is that the PPHN worsens, they write.

Hi @Stefan Johansson & @Gustaf Lernfelt

 

If possible I would very much like a copy of the mentioned VG-region guideline  (I´ve sent you an email Stefan).

Best,

Jesper