Screening and diagnosis of pulmonary hypertension associated with chronic lung disease (PH-CLD): A consensus statement from the pulmonary vascular research institute's innovative drug development initiative—group 3 pulmonary hypertension

Long-term hypoxia is one of the main causes of pulmonary vascular remodeling in pulmonary hypertension (PH) associated with congenital heart disease (CHD) children. Endothelial to mesenchymal transition (EndMT) is an important pathological basis of pulmonary vascular remodeling in PH. 

To the editor,
Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease characterized by alveolar surfactant accumulation, progressive dyspnea, and hypoxemic respiratory insufficiency, and in some patients, secondary infections, pulmonary fibrosis, respiratory failure, and death.1 GM-CSF (granulocyte/macrophage colony-stimulating factor) autoantibodies cause aPAP by blocking GM-CSF signaling, which impairs alveolar macrophage functions including surfactant clearance.

The PERFECT study, a randomized, controlled, double-blind study of inhaled treprostinil in patients with COPD and associated pulmonary hypertension (PH-COPD) was a negative trial that was terminated early. The reason(s) for the negative outcome remains uncertain.

Tuberculosis (TB) may cause significant long-term cardiorespiratory complications, of which pulmonary vascular disease is most under-recognized. TB is rarely listed as a cause of pulmonary hypertension (PH) in most PH guidelines, yet PH may develop at various stages in the time course of TB, from active infection through to the post-TB period.