Pathology and Pathobiology of Pulmonary Hypertension

Christophe Guignabert; Peter Dorfmuller

doi:10.1055/s-0033-1356496

Seminars in Respiratory and Critical Care Medicine, Inhaltsverzeichnis

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

¹INSERM UMR_S 999, Cardiovascular Research Department, “Pulmonary Hypertension: Physiopathology and Novel Therapies,” LabEx LERMIT, Le Plessis-Robinson, France

³School of Medicine, University of Paris-Sud, DHU Thorax Innovation (TORINO), Le Kremlin-Bicêtre, France

,

Peter Dorfmuller

¹INSERM UMR_S 999, Cardiovascular Research Department, “Pulmonary Hypertension: Physiopathology and Novel Therapies,” LabEx LERMIT, Le Plessis-Robinson, France

²Pathology Department & Research Department, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson, France

³School of Medicine, University of Paris-Sud, DHU Thorax Innovation (TORINO), Le Kremlin-Bicêtre, France

› Institutsangaben

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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Referenzen

References
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