Oneliner_159

OneLiner

Hereditary PAH – Due to inactivating mutation in BMPR2. AD with variable penetrance. Can be detected by loud S2 heart sound and clear lungs (secondary PAH will have pulmonary edema). Disfunction endothelial & smooth muscle proliferation. Increased levels of vasoconstrictive, proliferative mediators (e.g. endothelin) & decreased levels of vasodilative, antiproliferative mediators (eg NO, prostacyclin)