Key result
Severe thromboembolic PH selectively upregulates endothelin B receptors and boosts endothelin-1 production.
Severe pulmonary hypertension caused by thromboembolic disease is associated with selective upregulation of Endothelin B receptor gene expression.
No change to CTEPH management; leaves open selective ETB targeting for future human studies.
These data suggest that changes in the ET signaling system in PHT caused by thromboembolic disease are not limited to an increased production of ET-1: they also affect ET receptor gene expression.
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Bauer et al. (2002) studied this question. Severe pulmonary hypertension caused by thromboembolic disease selectively upregulates endothelin B receptor gene expression in addition to increasing endothelin-1 production.
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