296.4 📋 章末速記 Summary

296.4.1 🔑 一句話總結

系統性疾病心臟侵犯 跨多個族群：autoimmune（SLE pericarditis + Libman-Sacks、RA pericarditis、scleroderma PAH + 心肌纖維化、APS valve abnormalities）、vasculitis（Takayasu aortitis、Churg-Strauss myocarditis）、infiltrative（AL amyloid daratumumab + CyBorD、ATTR-wild type tafamidis、sarcoid + ICD、hemochromatosis chelation、Fabry ERT）、endocrine（thyroid、acromegaly、Cushing、pheo、DM diabetic cardiomyopathy）、infectious（syphilis aortitis、TB pericarditis、HIV cardiomyopathy、Chagas apical aneurysm、Lyme AVB）、hematologic（sickle cell PH、thalassemia iron overload）、genetic / neuromuscular（Friedreich HCM、Duchenne DCM、myotonic AVB、Emery-Dreifuss DCM+AVB）、COVID-19 post-acute sequelae。

296.4.2 💊 治療精要

  • autoimmune：disease control（HCQ、steroids、DMARDs、biologics）+ aggressive CV RF management
  • Takayasu：steroids + MTX + tocilizumab
  • AL amyloid：daratumumab + CyBorD（ANDROMEDA）
  • ATTR：tafamidis（ATTR-ACT）；patisiran + vutrisiran（gene silencers）；acoramidis（2024 新 stabilizer）
  • sarcoid：steroids + 免疫抑制 + ICD + pacemaker
  • hemochromatosis：phlebotomy or chelation；CMR T2*
  • Fabry：ERT (agalsidase α/β)、migalastat（chaperone）、pegunigalsidase
  • diabetic cardiomyopathy：SGLT2i + GLP-1 + ACEi + statin
  • Chagas：benznidazole + HF therapy + ICD

296.4.3 🎯 盧醫師的考前提醒

  1. SLE 心臟侵犯 #1 = pericarditis（~30%）；Libman-Sacks endocarditis = APS overlap 的 sterile MV vegetation
  2. scleroderma 死因 #1 = PAH — 年度 DETECT screening 是 standard of care
  3. vasculitis 區分 vessel size：large (Takayasu, GCA) / medium (PAN, Kawasaki) / small (ANCA-associated, Churg-Strauss = EGPA)
  4. AL vs ATTR amyloid 區分：AL 從 plasma cell (FLC + 免疫固定)；ATTR 從 transthyretin（wild-type elderly 或 hereditary 有突變）；PYP scan + biopsy + mass spec
  5. tafamidis (ATTR-ACT 2018) = ATTR-CM gold standard；patisiran + vutrisiran 為 gene silencers；acoramidis 2024 為新 stabilizer
  6. AL amyloid 新標準：daratumumab + bortezomib + cyclophosphamide + dexamethasone (CyBorD) — ANDROMEDA trial
  7. sarcoid 心臟最常見 = AV block；CMR + PET + EMB；steroids + 免疫抑制 + ICD 為治療
  8. hemochromatosis cardiomyopathy：DCM 為主；T2* CMR 量化心臟鐵含量（< 20 abnormal、< 10 severe）；hereditary 用 phlebotomy，transfusion-dependent 用 chelation
  9. Fabry disease：X-linked GLA 缺乏；HCM phenotype + renal failure + neuropathy + angiokeratomas；ERT (agalsidase) / migalastat / pegunigalsidase
  10. 特殊鑑別：Chagas (apical aneurysm)、syphilis (ascending aorta + AR + ostial)、Lyme (acute AV block)、Duchenne (DCM 年少)、myotonic (AVB + SCD)、Emery-Dreifuss (DCM + AVB, LMNA)、Friedreich (HCM, GAA repeats)