Extensive loss of endothelial CAV1 accompanied by enhanced expression of CAV1 in vascular smooth muscle cells (VSMC) was reported in IPAH, in HPAH, in PAH associated with congenital heart defect and drug toxicity [22,28,29,30,31], and in infants with bronchopulmonary dysplasia and evidence of inflammation [29]. Here, CAV1 is linked to pulmonary arterial hypertension.