Moreover, LMVECs isolated from patients with APAH with congenital cardiac shunts, often accompanied by hypoxemia that suppresses endothelial PHD2 activity, showed a trend toward increased STAT1/3 activation compared with control LMVECs from FD subjects (P = 0.085 for pSTAT1, P = 0.036 for the combined pSTAT1 and pSTAT3 effect; Fig. 5E). Here, STAT1 is linked to Fabry disease.