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Clinical Assessment of Pulmonary Hypertension

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Scleroderma

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.

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Author information

Authors and Affiliations

  1. Pulmonary Hypertension Center, Boston Medical Center, Boston University, Boston, MA, USA

    Harrison W. Farber MD

  2. Arthritis Center, Boston Medical Center/Boston University, Boston, MA, USA

    Michael York MD

  3. Department of Internal Medicine, Claude Huriez, Lille, France

    Eric Hachulla MD, PhD

Authors
  1. Harrison W. Farber MD
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  2. Michael York MD
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  3. Eric Hachulla MD, PhD
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Corresponding author

Correspondence to Harrison W. Farber MD .

Editor information

Editors and Affiliations

  1. Feinberg School of Medicine, Northwestern University, E. Huron Street 240, Chicago, 60611-2909, Illinois, USA

    John Varga

  2. Royal Free and University College, Medical School, University College London, Rowland Street, London, NW3 2PF, United Kingdom

    Christopher P. Denton

  3. School of Medicine, Div. Rheumatology, Johns Hopkins University, Eastern Avenue 5200, Baltimore, 21224, Maryland, USA

    Fredrick M. Wigley

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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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