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Semin Respir Crit Care Med 2013; 34(05): 551-559
DOI: 10.1055/s-0033-1356496
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pathology and Pathobiology of Pulmonary Hypertension

Christophe Guignabert
1   INSERM UMR_S 999, Cardiovascular Research Department, “Pulmonary Hypertension: Physiopathology and Novel Therapies,” LabEx LERMIT, Le Plessis-Robinson, France
3   School of Medicine, University of Paris-Sud, DHU Thorax Innovation (TORINO), Le Kremlin-Bicêtre, France
,
Peter Dorfmuller
1   INSERM UMR_S 999, Cardiovascular Research Department, “Pulmonary Hypertension: Physiopathology and Novel Therapies,” LabEx LERMIT, Le Plessis-Robinson, France
2   Pathology Department & Research Department, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson, France
3   School of Medicine, University of Paris-Sud, DHU Thorax Innovation (TORINO), Le Kremlin-Bicêtre, France
› Author Affiliations
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Publication History

Publication Date:
13 September 2013 (online)

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Abstract

Pulmonary hypertension is a devastating, life-threatening disorder with no curative options, characterized by elevated pulmonary vascular resistance and secondary right ventricular failure. Although the etiologies of pulmonary arterial hypertension are multiple and its pathogenesis is complex, there is growing evidence that inflammation, endothelial dysfunction, aberrant vascular wall cell proliferation, as well as mutations in the bone morphogenetic protein receptor type 2 gene play a crucial role in triggering pathological vascular remodeling. The present article outlines the current understanding of this disease from the point of view of pathology and pathobiology.

Keywords

pulmonary arterial hypertension - pulmonary vascular remodeling - endothelial dysfunction - cellular cross talk - inflammation - BMPR2
 

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