Insights Into the Pathobiology of Pulmonary Hypertension
Víctor I. Peinado 1, Isabel Blanco 2, Olga Tura-Ceide 1, Joan Albert Barberà 1
1 Department of Pulmonary Medicine, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain; Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Madrid, Spain; 2 Unitat d’Hipertensió Pulmonar, Servei de Pneumologia, Hospital Clinic-IDIBAPS, CIBERES, Universitat de Barcelona, Barcelona, Spain
*Correspondence: Isabel Blanco, Email not available
Abstract
Pulmonary hypertension is a complex disorder defined by an abnormal increase of pulmonary arterial pressure that may result in right ventricular failure and death. Pulmonary hypertension has a multifactorial aetiology and is currently classified into five groups based on histopathological appearance and treatment modalities. Our understanding of the pathobiology of pulmonary hypertension has evolved enormously in recent years. A condition that in the past was considered mainly determined by increased vascular tone is now seen as a vasculopathy in which structural changes are driven by excessive cell growth. In the present review we analyse mechanisms that may contribute to the pathobiology of pulmonary hypertension, including imbalance between vasoactive mediators, altered cell proliferation and apoptosis, dysfunctional endothelial repair and angiogenesis, and contributing factors such as inflammation.
