Even though clinically distinct, PAH, HHT, Marfan- and Loeys-Dietz-syndrome may be driven by a similarly occurring biomechanical stressor because they show dysregulation of the same molecular players found in BMPR2-deficient ECs (i.e. TGFβ, BMP9, endoglin, ALK1, Alk5, fibrillin and SMADs). The gene discussed is BMPR2; the disease is pulmonary arterial hypertension.