Abstract
Severe pulmonary arterial hypertension, whether idiopathic or secondary, is characterized by structural alterations of microscopically small pulmonary arterioles. The vascular lesions in this group of pulmonary hypertensive diseases show actively proliferating endothelial cells without evidence of apoptosis. In this article, we review pathogenetic concepts of severe pulmonary arterial hypertension and explain the term "complex vascular lesion ", commonly named "plexiform lesion", with endothelial cell dysfunction, i.e., apoptosis, proliferation, interaction with smooth muscle cells and transdifferentiation.
Publication types
-
Research Support, N.I.H., Extramural
-
Research Support, Non-U.S. Gov't
-
Review
MeSH terms
-
Animals
-
Apoptosis* / genetics
-
Cell Communication* / genetics
-
Cell Proliferation*
-
Cell Transdifferentiation* / genetics
-
Disease Progression
-
Endothelial Cells / metabolism
-
Endothelial Cells / pathology*
-
Humans
-
Hypertension, Pulmonary / genetics
-
Hypertension, Pulmonary / metabolism
-
Hypertension, Pulmonary / pathology*
-
Hypertension, Pulmonary / physiopathology
-
Muscle, Smooth, Vascular / metabolism
-
Muscle, Smooth, Vascular / pathology
-
Pulmonary Artery / metabolism
-
Pulmonary Artery / pathology*
-
Pulmonary Artery / physiopathology
-
Severity of Illness Index
-
Signal Transduction