Key result
Severe pulmonary arterial hypertension is proposed to be driven by a neoplastic, angioproliferative process of apoptosis-resistant endothelial cells rather than solely by vasoconstriction.
Population
Patients with severe pulmonary arterial hypertension (PAH) characterized by plexiform lesions
Design
Review
Authors
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Does not yet warrant shifting PAH management from vasodilators; leaves open antiproliferative strategies as a research priority.
Conceptualizing severe PAH as a quasi-malignant, angioproliferative disorder provides a new framework for developing antiproliferative and antiangiogenic therapies.
Cool et al. (2008) conducted a review in Severe Pulmonary Arterial Hypertension. Severe pulmonary arterial hypertension is proposed to be driven by a neoplastic, angioproliferative process of apoptosis-resistant endothelial cells rather than solely by vasoconstriction.
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