Posted December 20, 20231 yr 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!
December 22, 20231 yr 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.
February 29, 2024Feb 29 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
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