Abstract
Pulmonary arterial hypertension (PAH) is primarily a disease of pulmonary vascular resistance (PVR) caused by proliferation and contraction of vascular smooth muscle cells. PAH is a frequent complication of systemic sclerosis (SSc) observed with a prevalence of 8–12% (Arthritis Rheum 52:3792–800, 2005; Ann Rheum Dis 62:1088–93, 2003; Intern Med J 39:682–91, 2009) due to a proliferative arterial pulmonary microangiopathy. A recent meta-analysis of more than 3,500 SSc patients demonstrated a prevalence of PAH, based on right heart catheterization (RHC) of slightly less than 10% (J Rheumatol 37:2290–8, 2010). In order to diagnose PAH, it is necessary to establish the appropriate cardiopulmonary hemodynamics, mPAP ≥25 mmHg, PCWP or left ventricular end diastolic pressure ≤15 mmHg, and a PVR ≥240 dyn/cm−5 (3 Wood units); currently, the only available method for detecting and assessing these and other important cardiopulmonary hemodynamic parameters (i.e., cardiac output) is RHC; thus, RHC should be performed in all cases in which PAH is suspected.
Keywords
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Farber, H.W., York, M., Hachulla, E. (2012). Clinical Assessment of Pulmonary Hypertension. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_36
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DOI: https://doi.org/10.1007/978-1-4419-5774-0_36
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