Pulmonary arterial hypertension (PAH) is a rare, progressive disease characterised by elevated pulmonary arterial pressure and vascular resistance that eventually leads to right ventricular failure and death
Early identification and intervention are key to changing the course of the disease
PAH is a severe and often fatal complication of CTD:
PAH is a leading cause of death in patients with SSc, accounting for >50% of deaths in SSc-PAH patients
Adapted from Badesch et al. 2010
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ALT, alanine aminotransferase; AST, aspartate aminotransferase;CHD, congenital heart disease; CI, confidence interval; CTD, connective tissue disease; ERA, endothelin receptor antagonist; ERS, European Respiratory Society; ESC, European Society of Cardiology; HIV, human immunodeficiency virus; HPAH, heritable pulmonary arterial hypertension; HR, hazard ratio; IPAH, idiopathic pulmonary arterial hypertension; LFT, liver function test; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension; PoPH, portopulmonary hypertension; RHC, right heart catheterisation; SSc, systemic sclerosis; TRV, tricuspid regurgitation velocity; ULN, upper limit of normal; 6MWD, six-minute walk distance