Pulmonary arterial hypertension is a syndrome resulting from restricted flow through the pulmonary arterial circulation resulting in increased pulmonary vascular resistance and ultimately leading to right heart failure.
It is a part of the spectrum of pulmonary hypertension, which is hemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure ≥25 mmHg at rest.
Typical symptoms include progressive dyspnea on exertion, palpitations, fatigue, weakness, angina, syncope and abdominal distention.
Use of the oral phosphodiesterase-5 inhibitor udenafil in the treatment of patients with pulmonary arterial hypertension is safe and leads to a significant increase in exercise capacity, according to the results of a phase IIB trial.
The reduced mortality/morbidity benefit previously demonstrated with selexipag in patients with pulmonary arterial hypertension (PAH) may be more pronounced when treatment is initiated soon after diagnosis, according to results of a post hoc analysis of the GRIPHON* study presented as a poster at ATS 2019.
Patients with chronic thromboembolic pulmonary hypertension (CTEPH) may experience improvements in exercise capacity and other outcomes when treated with a continuous, subcutaneous, high dose of treprostinil, according to the phase III CTREPH* trial.
Pulmonary arterial hypertension (PAH) may be the first manifestation of primary Sjögren's syndrome (pSS) in patients with Raynaud’s phenomenon, high-titre rheumatoid factor, hepatic injury or pericardial effusion, according to a recent study.
Patients with combined pulmonary fibrosis and emphysema (CPFE) are likely to develop pulmonary hypertension, and this likelihood may be explained by the summed extents of interstitial lung disease (ILD) and emphysema, according to a study.
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