State-of-the-Art Paper
Development and Pathology of Pulmonary Hypertension

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The Development and Pathology working group was charged with reviewing the present knowledge, gaps in understanding, and areas for further studies in a broad range of themes. These themes in pulmonary vascular biology and pathobiology involved: 1) pulmonary vascular development; 2) pulmonary vascular disease accompanying fetal development and perinatal life; 3) properties of pulmonary vascular endothelial cells; 4) role of bone marrow cells in pulmonary vascular disease; 5) insights into pulmonary thromboembolic disease; 6) role of pathology in the assessment of pulmonary vascular disease; and 7) considerations of tissue banking for research in pulmonary hypertension. These important goals provide a blueprint for future research that may significantly impact our present and future understanding of pulmonary hypertension.

Key Words

pulmonary hypertension
pulmonary circulation
angiogenesis
endothelial cell progenitor
pathology

Abbreviations and Acronyms

BMP
bone morphogenetic protein
BMPR
bone morphogenetic protein receptor
BPD
bronchopulmonary dysplasia
CDH
congenital diaphragmatic hernia
CTEPH
chronic thromboembolic pulmonary hypertension
eNOS
endothelial nitric oxide synthase
EPC
endothelial progenitor cell
IPAH
idiopathic pulmonary arterial hypertension
PAH
pulmonary arterial hypertension
PCH
pulmonary capillary hemangiomatosis
PH
pulmonary hypertension
PPHN
persistent pulmonary hypertension of the newborn
PVOD
pulmonary veno-occlusive disease
TGF
transforming growth factor
WHO
World Health Organization

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