Pulmonary hypertension due to lung disease and/or hypoxia

Pulmonary hypertension may complicate the course of patients with many forms of advanced lung disease. The cause is likely multifactorial with pathogenic pathways both common and unique to the specific disease entities. The occurrence of pulmonary hypertension is associated with worse outcomes, but whether this is an adaptive or maladaptive phenomenon remains unknown. The treatment of pulmonary hypertension with vasoactive medications in lung disease remains unproved. Specific disease phenotypes that might benefit, and those in which such therapies might be deleterious, remain to be determined.