[New insights in the pathogenesis of pulmonary arterial hypertension]

Pulmonary arterial hypertension (PAH) is a severe and incurable cardiopulmonary disorder. Research from the past 10 years illustrates the complex and multifactorial aspects of PAH pathophysiology. Furthermore, latest advances in the field have led to a better understanding of the key components underlying this inadequate accumulation of pulmonary vascular cells within the pulmonary arterial walls, leading to pulmonary vascular remodelling. Among the underlying molecular and cellular mechanisms, pulmonary endothelial dysfunction, alterations of the inter-cell communications within the pulmonary arterial walls as well as defects of the inflammatory component and the loss of BMPRII activity play critical roles in the pathogenesis of the disease.