Pulmonary hypertension (PH) is a disorder of the pulmonary circulation in which elevated pressure in the pulmonary vascular circuit can when severe, lead to right heart failure and eventuallycause death. Pulmonary arterial hypertension (PAH) is a subtype of PH. Current recommendations suggest that screening should be performed only on persons at increased risk for developing PAH. The diagnosis of PAH is usually made during
the process of evaluating symptoms such as dyspnoea, exercise intolerance, chest pain, or syncope.
Electrocardiographic changes, elevated brain natriureteric peptide (BNP) levels, and echocardiographic Changes may suggest the diagnosi however, current recommendations state that a right-heart catheterization (RHC) is “strongly advised” to formally make the diagnosis of PH. A ventilation—perfusion (V/Q) scan should be per-formed to evaluate for chronic thromboembolic disease. Other tests include thyroid function tests, liver function tests (LFT), antiphospholipid anti-bodies, testing for collagen vascular diseases, and testing to detect the human immunodeficiency vi-rus (HIV).“] A baseline BNP. All patients with PH should undergo baseline pulmonary function
testing including spirometry, determination of lung volumes, and evaluation of the diffusion limitation for carbon monoxide (DLCO). Quantification of exercise tolerance is often performed by measuring the distance that a patient can walk in 6 minutes (6MWD).General Management—Related ISSues include supplemental oxygen, anticoagulation with warfarin, therapy with prostacyclin analogs (prostanoids) including epoprostenol, treprostinil, iloprost etc., endothelin receptor antagonists (bosentan), Phosphodiesterase type 5 (PDES) in-
hibitors such as sildenafil or tadalafil; and Atrial septostomy (AS), pulmonary thromboendarterectomy and finally lung transplantation. The prognosis of untreated PAH is poor how-ever, with therapy the expected survival and quality of life have improved dramatically
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