Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure ≧25 mmHg with a pulmonary capillary wedge pressure of ≦15 mmHg at rest. The diagnosis and treatment of PAH is challenging for its similarities to other heart or lung disease. Right heart catheterization is an accurate diagnostic method of PAH. Clinical presentations, laboratory investigations and exercise test are helpful leading to a definite diagnosis. Medical imaging can also be an approach for the evaluation of patients’ outcomes following sufficient treatment. It is worthwhile mentioning that computed tomography angiography may reveal bronchial collaterals and magnetic resonance angiogram may delineate the presence of chronic thromboembolic PAH in such patients. Lung biopsy offers pathological evidence for the diagnosis of PAH, but it is not routinely performed. Biomarkers including antinuclear antibody, B-type natriuretic peptide, circulating endothelial cells and microRNA have been found to be important diagnostic indicators of PAH. Calcium channel blockers and vasodilators are effective medications for the treatment of PAH. Septotomy is a palliative procedure, and lung transplantation is a definitive solution for the advanced PAH. Apart from conventional and surgical managements, microRNA, mesenchymal stem cells and remote postconditioning provisionally show effects for the treatment of PAH.
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