Clinical assessment of pulmonary hypertension

Pulmonary arterial hypertension (PAH) is defined by a mean pulmonary artery pressure (mPAP) ≥25 mmHg, pulmonary capillary wedge pressure (PCWP) or left ventricular end-diastolic pressure ≤15 mmHg, and a pulmonary vascular resistance (PVR) ≥3 Wood units. PAH is due to pulmonary vascular remodeling predominating on pulmonary arteries of less than 500 µm of diameter. Currently, the only available method for detecting and assessing cardiopulmonary hemodynamic parameters is right heart catheterization (RHC). Thus, RHC should be performed in all cases in which PAH is suspected. PAH is a frequent complication of systemic sclerosis (SSc) observed with a prevalence of 8-12 %, and it is one of the leading causes of death in systemic sclerosis. Despite advances in treatment options for PAH, long-term prognosis remains poor for SSc-associated PAH (SSc-PAH). Although prompt diagnosis and treatment of PAH may have significant impact on survival rates, early detection of SSc-PAH continues to be challenging due to several factors ranging from limitations of the current screening tools and the complexities of the disease.