283.2 🩺 國考版

283.2.1 高頻考點

283.2.1.1 5 WHO Groups

  1. PAH (idiopathic, heritable, drug, CTD, CHD, portal, HIV, schisto)
  2. Left heart disease (most common)
  3. Lung disease / hypoxia
  4. CTEPH
  5. Multifactorial

283.2.1.2 RHC Findings

  • Pre-capillary PH: mPAP > 20, PCWP ≀ 15, PVR ≥ 2 WU
  • Post-capillary: mPAP > 20, PCWP > 15
  • Mixed: PVR > 2 in post-capillary

283.2.1.3 Vasoreactivity Test (IPAH Only)

  • Inhaled NO or IV epoprostenol
  • Positive: ↓ mPAP ≥ 10 + final < 40 + CO unchanged
  • ~ 10% positive → CCB (amlodipine, diltiazem, nifedipine)

283.2.1.4 Three Pathways

  • Endothelin: bosentan, ambrisentan, macitentan
  • NO/cGMP: sildenafil, tadalafil, riociguat (sGC stimulator)
  • Prostacyclin: epoprostenol, treprostinil, iloprost, selexipag

283.2.1.5 Key Trials

  • AMBITION (2015): initial combo (ambrisentan + tadalafil) > mono
  • SERAPHIN: macitentan composite endpoint benefit
  • GRIPHON: selexipag added to standard ↓ disease progression
  • STELLAR (2023): sotatercept improved 6MWT in PAH
  • CHEST-1: riociguat for CTEPH
  • PATENT-1: riociguat for PAH
  • INCREASE (2021): inhaled treprostinil for ILD-PH

283.2.1.6 CTEPH Cure

  • PEA (pulmonary endarterectomy) at experienced center
  • Inoperable: riociguat + BPA

283.2.2 易混淆比范

Group Cause RHC Treatment
1 (PAH) Plexiform, BMPR2 mut Pre-capillary PAH drugs + sotatercept
2 (LH) HFrEF/HFpEF, valve Post-capillary Treat LH disease (no PAH drugs)
3 (Lung) COPD, ILD Mild PH Oxygen, treat lung dz
4 (CTEPH) Post-PE Pre-capillary, V/Q defects PEA, riociguat, BPA
5 Hemolysis, sarcoid, MPN Variable Treat underlying

283.2.2.1 Group 1 Subtypes

  • Idiopathic (no cause)
  • Heritable (BMPR2 most common)
  • Drug-induced (anorexigens, methamphetamine, dasatinib)
  • CTD (scleroderma > SLE > MCTD > RA)
  • CHD (Eisenmenger)
  • Portal HTN
  • HIV
  • Schistosomiasis
  • PVOD / PCH