Despite recent evidence that emphasizes the beneficial role of HIF2α inhibition (in endothelial cells) as a therapeutic target for pulmonary hypertension (208–210), thorough selection of patients to test proof-of-concept of these results in humans will be necessary as endothelial HIF2α appears to be crucial for vascular survival and maintenance of a functional alveolar structure (213, 214). This evidence concerns the gene EPAS1 and pulmonary hypertension.