283 Ch 282. Pulmonary Hypertension (PH)

肺動脈高壓 (PH) = mean pulmonary artery pressure (mPAP) > 20 mmHg 於 RHC（2018 WSPH revised, 從 > 25 改 > 20）；分 5 大組（WHO classification）：(1) PAH (pulmonary arterial), (2) Left heart disease, (3) Lung disease/hypoxia, (4) CTEPH (chronic thromboembolic), (5) Multifactorial/unclear；診斷靠 RHC + echo + V/Q scan + CT + 6MWT + BNP；PAH 治療 以三大途徑為主 (endothelin, NO/cGMP, prostacyclin)：ERAs (bosentan, ambrisentan, macitentan), PDE5i (sildenafil, tadalafil) ± sGC stimulator (riociguat), prostanoids (epoprostenol, treprostinil, iloprost, selexipag)；2022-2024 重大突破：sotatercept (activin signaling 修飾) — STELLAR trial 改善 6MWT、降低 hospitalization；CTEPH 治療：pulmonary endarterectomy (PEA) 為 cure；inoperable / persistent → riociguat + BPA (balloon pulmonary angioplasty)。