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Proposed model illustrating the role of endothelial dysfunction in microvasculopathy associated with CTEPH. Dysfunction of the pulmonary endothelium leads to an altered balance of endothelium-derived vasoactive mediators including NO, endothelin-1 and prostacyclin. Other mediators such as cytokines, chemokines and growth factors may also contribute to pulmonary microvasculopathy. CTEPH, chronic thromboembolic pulmonary hypertension.
