271.4 📌 章末速蚘

271.4.0.0.1 Classification
  • DCM (HFrEF; idiopathic, familial, viral, toxic, tachycardia, peripartum)
  • HCM (asymmetric septal hypertrophy; sarcomere mutations; SCD risk)
  • RCM (HFpEF; amyloid + sarcoid + iron + Fabry)
  • ARVC (RV fibrofatty; desmosome mutations; VT/SCD)
  • Takotsubo (stress-induced, reversible apical ballooning)
  • Peripartum (pregnancy/postpartum; 30-50% recover)
271.4.0.0.2 HCM
  • Mavacamten (FDA 2022) for symptomatic obstructive
  • β-blocker / CCB / disopyramide
  • Septal myectomy / alcohol ablation for refractory
  • ICD for high-risk SCD features (family hx, syncope, NSVT, LVH ≥ 30 mm, abnormal BP exercise, LGE)
  • Family screening + genetic testing
271.4.0.0.3 Cardiac Amyloidosis
  • AL amyloid: chemotherapy (daratumumab + CyBorD; ANDROMEDA)
  • ATTR amyloid: tafamidis (Vyndaqel) + patisiran (TTR silencer)
  • Workup: free light chains + echo + CMR + PYP scan + biopsy for AL
  • Subtype critical for treatment
271.4.0.0.4 ARVC
  • RV fibrofatty replacement
  • Desmosome mutations
  • VT with LBBB morphology
  • Epsilon wave on ECG
  • ICD + exercise restriction
  • Family screening
271.4.0.0.5 Takotsubo
  • Stress-induced (postmenopausal women)
  • Reversible apical ballooning
  • Normal coronaries
  • Supportive treatment
271.4.0.0.6 Peripartum
  • Pregnancy/postpartum
  • Standard HFrEF (avoid ACEi/ARB in pregnancy)
  • 30-50% recover
271.4.0.0.7 Myocarditis
  • Viral most common (Coxsackie B, COVID-19)
  • Immune-mediated: SLE, sarcoid, eosinophilic, giant cell, ICI
  • Drug-induced: ICI, anthracyclines, cocaine
  • Lake Louise CMR criteria (T1/T2 + LGE)
  • Specific treatment:
    • Giant cell: cyclosporine + steroid
    • Eosinophilic + sarcoid: corticosteroids
    • ICI: high-dose steroid + stop ICI + cardio-oncology
  • Supportive HFrEF + mechanical support if severe
271.4.0.0.8 Key Trials/Drugs
  • EXPLORER-HCM: mavacamten for obstructive HCM
  • ATTR-ACT: tafamidis for ATTR amyloidosis
  • ANDROMEDA: daratumumab + CyBorD for AL amyloid
  • STEP-HCM: mavacamten cohort
  • MERIT-HF + CIBIS-II + RALES + EMPHASIS-HF + DAPA-HF + EMPEROR-Reduced (HF — Ch 268)
271.4.0.0.9 盧醫垫 hint
  • HCM + symptomatic obstructive: mavacamten (FDA 2022) — game changer
  • HFpEF + LVH + bilateral CTS + low voltage ECG: cardiac amyloidosis workup
  • ATTR amyloidosis: tafamidis early; significant outcome improvement
  • ICI myocarditis: high-dose steroid + stop ICI urgent
  • Giant cell myocarditis: cyclosporine + steroid; ECMO bridge if severe
  • Takotsubo: supportive + avoid catecholamines + reassurance (reversible)
  • Familial cardiomyopathy: family screening + genetic testing