Pulmonary hypertension (PH) is diagnosed by observing an elevation in mean pulmonary arterial (PA) pressure above 25 mmHg at rest or 30 mmHg with exercise. Patients usually present with much higher levels of PA pressure, but only vague and insidious symptoms of increasing fatigue and dyspnea. Some patients are diagnosed only after syncopal episodes.
Category I PH, also known as PA hypertension (PAH), includes idiopathic PAH (IPAH), familial PAH (FPAH), and acquired PAH (APAH).
Recent research has suggested that it might be possible to reverse the pathology of pulmonary arterial hypertension (PAH), a disorder that can be rapidly progressive and fatal despite current treatments including prostacyclin.
The role of chronic inflammation and autoimmunity will be important in the development of novel models of PAH. Emerging treatments for the disease are currently aimed at inducing apoptosis of abnormal vascular cells that obstruct blood flow and also at promoting regeneration of “lost” distal vasculature.
Sources:
J. Clin. Investigation
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