About this Research Topic
Pulmonary hypertension (PH) is a disorder of the pulmonary vasculature defined by increased mean pulmonary arterial pressure (mPAP) leading to right ventricle (RV) hypertrophy and dysfunction, right-sided heart failure and ultimately death. PH is a common complication of chronic lung diseases (CLD) including idiopathic pulmonary fibrosis (IPF) or chronic obstructive pulmonary disease (COPD) where it is classified as Group III PH by the WHO. It can also be associated with cardiovascular conditions such as left-heart disease (classified as Group II PH) or appear on its own as pulmonary arterial hypertension (PAH) and classified as Group I PH. In all of these cases the diagnosis of pulmonary hypertension is strongly associated with increased morbidity and mortality. The most common causes of PH are associated with Group II or III PH, yet research efforts to uncover the mechanisms leading to PH in these diseases remains under-investigated. The pathologic process in PH is characterized by extensive vascular remodeling; including enhanced proliferation of pulmonary artery smooth muscle cells (PASMC) leading to narrowing and obliteration of the vessel lumen resulting in increased vascular tone. Similarly, due to the increased pressure load in the pulmonary vasculature, the RV tries to compensate resulting in remodeling, hypertrophy, dysfunction and eventually right-sided heart failure and death. However, despite the devastating effects of PH, the mechanisms that lead to remodeling in the pulmonary vasculature and to right ventricle failure are not fully understood.
The focus of this research topic is to enhance our understanding of the mechanisms that contribute to the pathophysiology of pulmonary hypertension and right ventricle hypertrophy. Contributions on Group II or III PH are particularly encouraged.
Keywords: Vascular remodeling, pulmonary artery smooth muscle cells, pulmonary artery endothelial cells, apoptosis, macrophages, fibroblasts, extracellular matrix, pulmonary arterial hypertension
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