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PAH is definitively diagnosed with a right heart catheterization showing a mean pulmonary arterial pressure greater than 20 mmHg at rest, with a pulmonary vascular resistance being 3 Woods Units or greater (indicative of pulmonary hypertension in the pre-capillary vasculature; the arteries and arterioles), and a pulmonary artery wedge pressure ...
Three major signaling pathways involved in the pathogenesis of pulmonary arterial hypertension. The molecular mechanism of pulmonary arterial hypertension (PAH) is not known yet, but it is believed that the endothelial dysfunction results in a decrease in the synthesis of endothelium-derived vasodilators such as nitric oxide and prostacyclin. [41]
Macitentan, sold under the brand name Opsumit, is an endothelin receptor antagonist developed by Actelion and approved for the treatment of pulmonary arterial hypertension (PAH). [5] Macitentan is a dual endothelin receptor antagonist, meaning that it acts as an antagonist of two endothelin (ET) receptor subtypes, ET A and ET B. [5]
The Chronic Thromboembolic Pulmonary Hypertension sGC-Stimulator Trial (CHEST) was a randomized, placebo-controlled trial aimed to analyse the efficacy and safety of riociguat in CTEPH patients. [20] After a 16-week riociguat treatment the patient's exercise capacity were evaluated by measuring the change in the six-minute walk test (6-MWT). [21]
Selexipag, sold under the brand name Uptravi, is a medication developed by Actelion for the treatment of pulmonary arterial hypertension (PAH). [3] [4] Selexipag and its active metabolite, ACT-333679 (or MRE-269, the free carboxylic acid), are agonists of the prostacyclin receptor, which leads to vasodilation in the pulmonary circulation. [5]
Since the main causes of right ventricular hypertrophy is tricuspid regurgitation or pulmonary hypertension (discussed above), management involves treatment of these conditions. [3] Tricuspid regurgitation is typically treated conservatively by aiming to treat the underlying cause and following up the patient regularly. [ 12 ]