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Pulmonary Arterial Hypertension

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Molecular Pathology of Lung Diseases

Part of the book series: Molecular Pathology Library ((MPLB,volume 1))

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Abstract

Pulmonary arterial hypertension is and has often been classified into two separate categories: primary pulmonary (arterial) hypertension, presently more often called idiopathic pulmonary arterial hypertension (iPAH), and secondary pulmonary arterial hypertension.1 As in many other diseases, the secondary form was defined by presence of proven causes or risk factors, whereas the diagnosis of the primary form could be made only after excluding other causes of pulmonary arterial hypertension. In 1998, a clinical classification of pulmonary arterial hypertension was put forth, the so-called Evian classification.2 In a recent conference in Venice a revised clinical classification of pulmonary arterial hypertension was proposed (Table 58.1).3 Both the original and the revised classification was based on a clinical subdivision aimed at separating different categories within each category based on pathophysiology, clinical presentation, and therapeutic options. The clinical classifications were more or less based on assumptions that subsets of pulmonary arterial hypertension have a comparable spectrum of pathologic changes.

World Health Organization’s clinical classification of pulmonary hypertension (Venice 2003).

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Author information

Authors and Affiliations

  1. Department of Pathology, University Medical Center Groningen, Groningen, The Netherlands

    Wim Timens MD, PhD (Professor)

Authors
  1. Wim Timens MD, PhD
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Editor information

Editors and Affiliations

  1. Department of Pathology, Penn State Milton S. Hershey Medical Center, Hershey, PA, USA

    Dani S. Zander MD

  2. Institute of Pathology, Laboratories for Molecular Cytogenetics, Medical University of Graz, Graz, Austria

    Helmut H. Popper MD

  3. Department of Pathology, University of Texas Health Science Center, San Antonio, TX, USA

    Jaishree Jagirdar MD

  4. Weill Medical College of Cornell University, New York, NY

    Abida K. Haque MD

  5. Department of Pathology, San Jacinto Methodist Hospital, Baytown, TX, USA

    Abida K. Haque MD

  6. Pathology and Laboratory Medicine, Weill Medical College of Cornell University, New York, NY

    Philip T. Cagle MD  & Roberto Barrios MD  & 

  7. The Methodist Hospital, Houston, TX, USA

    Philip T. Cagle MD  & Roberto Barrios MD  & 

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Timens, W. (2008). Pulmonary Arterial Hypertension. In: Zander, D.S., Popper, H.H., Jagirdar, J., Haque, A.K., Cagle, P.T., Barrios, R. (eds) Molecular Pathology of Lung Diseases. Molecular Pathology Library, vol 1. Springer, New York, NY. https://doi.org/10.1007/978-0-387-72430-0_58

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  • DOI: https://doi.org/10.1007/978-0-387-72430-0_58

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-72429-4

  • Online ISBN: 978-0-387-72430-0

  • eBook Packages: MedicineMedicine (R0)

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