The review explains the pathogenesis of plexiform lesions in severe pulmonary arterial hypertension, emphasizing endothelial cell dysfunction, proliferation, and transdifferentiation.
Endothelial lesion features in severe PAH may inform future targets; leaves open therapeutic translation pending interventional trials.
Severe pulmonary arterial hypertension, whether idiopathic or secondary, is characterized by structural alterations of microscopically small pulmonary arterioles. The vascular lesions in this group of pulmonary hypertensive diseases show actively proliferating endothelial cells without evidence of apoptosis. In this article, we review pathogenetic concepts of severe pulmonary arterial hypertension and explain the term "complex vascular lesion ", commonly named "plexiform lesion", with endothelial cell dysfunction, i.e., apoptosis, proliferation, interaction with smooth muscle cells and transdifferentiation.
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Sakao et al. (2009) studied this question.
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