Clinical Assessment of Pulmonary Hypertension

Pulmonary hypertension (PH) is a frequent complication of systemic sclerosis (SSc), either as precapillary, postcapillary, or combined forms of PH. Pulmonary arterial hypertension (PAH) due to SSc is a severe and progressive disease due to combined vasculopathy and fibrogenesis. Early diagnosis and treatment are highly challenging in SSc-PAH and often require referral to a specialized PAH center. SSc-PAH has a poor prognosis if left untreated, accounting for around 30% of deaths among SSc patients. Diagnostic algorithms evolved in the last decade and have shown clinical value for detecting SSc-PAH early. Echocardiography still remains the most effective screening tool to suspect PAH in SSc patients, using the maximal tricuspid regurgitation jet (TRJ) velocity. Additional clinical information and biomarker levels are integrated in several diagnostic algorithms to refine PAH suspicion in patients with SSc. Right heart catheterization (RHC) is the gold standard for diagnosing PAH, as well as providing important prognostic parameters, such as cardiac index and right atrial pressure. PAH severity should be assessed at diagnosis and frequently reassessed after treatment initiation. Despite appropriate therapies, SSc-PAH is a devastating disease with a poorer prognosis than idiopathic PAH.