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The Defining Characteristics of Pulmonary Arterial Hypertension

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Pulmonary Hypertension

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Abstract

Pulmonary Arterial Hypertension (PAH) is a multifactorial progressive cardiopulmonary disease characterized by smooth muscle cell proliferation, hypertrophy of the vascular medial layer, arteriolar muscularization and endothelial cell proliferation. The persisting challenge in understanding the pathobiology of PAH is to define how the structural, cellular and molecular characteristics of the normal pulmonary circulation are disrupted in the disease setting. Although the exact pathophysiology remains unknown, there is increasing evidence to suggest an important role for inflammation and metabolic reprograming in the development of PAH. In this review, we highlight the known characteristic pathological features of human and experimental PAH. Additionally, we discuss how inflammatory signaling, including various chemokines, cytokines, immune cells, macrophages and TGF-β contribute to the PAH phenotype. Finally, we review emerging evidence that disordered cellular energetics and hypoxia signaling is characteristic in PAH. This chapter highlights the complexity and multiple pathobiologic disturbances that are defining characteristics of PAH.

Author contributed equally with all other contributors.

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    Authors and Affiliations

    1. Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado, Denver, Research Complex 2, Room 9001, Mail stop C-272, 12700 East 18th Avenue, Aurora, CO, 80045, USA

      Rahul Kumar PhD & Jeffrey C. Robinson MD

    2. Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado Hospital, Aurora, CO, USA

      Rubin M. Tuder MD

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    1. Rahul Kumar PhD
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    2. Jeffrey C. Robinson MD
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    3. Rubin M. Tuder MD
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    Correspondence to Rahul Kumar PhD .

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    Editors and Affiliations

    1. Cardiovascular Medicine Dept, Harvard Medical School Brigham and Women’s Hospital, Boston, Massachusetts, USA

      Bradley A. Maron

    2. Pulmonary & Critical Care Medicine, Stanford University Medical Center, Stanford, California, USA

      Roham T. Zamanian

    3. Pulmonary Critical Care Medicine Dept Cardiovascular Medicine Dept, Brigham and Women's Hospital Harvard Medical School, Boston, Massachusetts, USA

      Aaron B. Waxman

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    Kumar, R., Robinson, J.C., Tuder, R.M. (2016). The Defining Characteristics of Pulmonary Arterial Hypertension. In: Maron, B., Zamanian, R., Waxman, A. (eds) Pulmonary Hypertension. Springer, Cham. https://doi.org/10.1007/978-3-319-23594-3_2

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    • DOI: https://doi.org/10.1007/978-3-319-23594-3_2

    • Publisher Name: Springer, Cham

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