A recent study developed a new model of severe experimental pulmonary hypertension that recapitulates pulmonary vascular remodeling, occlusion, cardiac remodeling, and heart failure in mice by disrupting HIF prolyl hydroxylases, which stabilizes HIF-2α specifically in pulmonary endothelial cells and hematopoietic cells [127]. Here, EPAS1 is linked to pulmonary hypertension.