289.4 📋 章末速記 Summary

289.4.1 🔑 一句話總結

TOF 是最常見 cyanotic CHD（4 特徵：RVOT obstruction + VSD + overriding aorta + RVH），現代兒童期手術後存活 > 90%；後遺症最常見為 pulmonic regurgitation (PR) → RV 擴張 → VT/SCD；TPVR (Melody/Harmony) 是新利器；Coarctation = 主動脈狹窄常在 L subclavian 後；HTN in arms + radial-femoral delay + “rib notching”；adult 用 balloon + stent；TGA D-TGA arterial switch / Mustard-Senning atrial switch / ccTGA 都有 systemic RV 問題；Eisenmenger = L→R 反轉成 R→L from PAH；治療 = PAH-specific therapy；absolutely contraindicate pregnancy (30-50% maternal mortality)。

289.4.2 💊 治療精要

  • TOF post-op：PVR for severe PR with RV dilation / dysfunction / arrhythmia (RVEDV index > 150)；TPVR or surgical
  • TOF SCD risk：QRS > 180、↓ RV/LV、syncope、inducible VT → ICD primary prevention
  • Coarctation adult：balloon + stent；BP < 130/80；lifelong CMR follow-up
  • D-TGA：arterial switch (Jatene) — 兒童期；late coronary + neo-AV 問題
  • Mustard/Senning + ccTGA：systemic RV → GDMT；severe TR → 手術；最終 transplant
  • Eisenmenger：bosentan (BREATHE-5)、sildenafil、selexipag、prostanoid；no pregnancy

289.4.3 🎯 盧醫師的考前提醒

  1. TOF 4 components：RVOT obstruction + VSD + overriding aorta + RVH（記憶 = ROVR）
  2. TOF SCD risk factors：QRS > 180 ms、RVEDV indexed > 150、LV EF < 40%、syncope、inducible VT
  3. boot-shaped heart = TOF；egg on side = TGA；snowman = TAPVR；rib notching + figure 3 = coarctation
  4. TPVR (Melody, Harmony) 是 post-TOF severe PR 的新標準（avoid open heart re-do surgery）
  5. coarctation 多在 L subclavian 之後（postductal adult type）；BAV 共存 50-80%；Turner syndrome 共存 ~ 30%
  6. D-TGA arterial switch (Jatene) 是現代做法，long-term 注意 coronary ostia re-implantation 問題 + neo-AV regurgitation
  7. Mustard/Senning + ccTGA 都有 systemic RV problem → 終身 GDMT + 最終 transplant
  8. Eisenmenger 鐵則：pregnancy contraindicated（maternal mortality 30-50%）+ IV filter + 補鐵（despite polycythemia）+ Hct > 65% AND 症狀才 phlebotomy
  9. BREATHE-5 bosentan 在 Eisenmenger 是 landmark trial；MAESTRO (macitentan) 略弱；STELLAR (sotatercept) subgroup 看好
  10. 22q11 deletion (DiGeorge) 在 15-20% TOF 患者 — 記得 cardiac panel testing