Pathophysiological Mechanisms in PH

Elevated EDN1 levels in PH patients trigger several pathogenic processes:

Vasoconstriction: Persistent ETA activation increases pulmonary arterial pressure.

Vascular Remodeling: EDN1 stimulates smooth muscle proliferation, fibroblast activation, and collagen deposition, thickening vessel walls.

Endothelial Dysfunction: Overactive EDN1 signaling reduces nitric oxide and prostacyclin bioavailability, impairing vasodilatory responses.

Right Ventricular Overload: Chronically elevated pulmonary pressures increase right ventricular workload, promoting hypertrophy and eventual failure.

These mechanisms underscore EDN1’s central role in both disease onset and progression.