289.2 🩺 國考版

289.2.1 高頻考點

289.2.1.1 TOF

  • 4 features: RVOT obstruction + VSD + overriding aorta + RVH
  • Most common cyanotic CHD beyond infancy
  • “Boot-shaped heart”
  • Post-op: PR (most common complication), VT, SCD
  • TPVR (Melody, Harmony)

289.2.1.2 TOF SCD Risk Factors

  • QRS > 180 ms
  • RV dilation (RVEDV indexed > 150 mL/m²)
  • LV dysfunction (EF < 40%)
  • Syncope
  • Inducible VT
  • Late repair age

289.2.1.3 Coarctation

  • HTN in arms, ↓ BP in legs (> 20 mmHg gradient)
  • Radial-femoral delay
  • “Rib notching” CXR (intercostal collaterals)
  • Balloon + stent first-line in adults

289.2.1.4 Transposition

  • D-TGA: aorta from RV, PA from LV; “egg on side”; arterial switch in newborns
  • Mustard/Senning: older atrial switch; systemic RV → late failure; arrhythmia, baffle issues
  • ccTGA: doubly transposed; physiologically corrected; eventual systemic RV failure

289.2.1.5 Eisenmenger

  • Reversed shunt from PAH
  • Treatment: bosentan (BREATHE-5), sildenafil, prostanoid
  • Pregnancy contraindicated (maternal mortality 30-50%)
  • IV filter, iron supplementation, hydration

289.2.1.6 Key Trials

  • BREATHE-5: bosentan in Eisenmenger
  • MAESTRO: macitentan in Eisenmenger (modest)
  • STELLAR: sotatercept (CHD-PAH sub)

289.2.2 易混淆比范

Lesion Cyanosis CXR ECG Long-Term Issue
TOF Yes (variable) Boot-shaped, ↓ pulm vasc RVH, RAD, RBBB PR, VT, SCD
Coarctation No Rib notching, figure 3 LVH HTN, dissection, aneurysm
D-TGA Yes Egg on side RVH (newborn) Post-arterial switch: coronary, neoaortic
Mustard/Senning No (post-op) Variable Atrial arrhythmia Systemic RV failure, baffle
ccTGA No Variable Position changes Systemic RV failure, TR
Eisenmenger Yes (acquired) Enlarged PA RVH PAH-CHD; pregnancy banned

289.2.2.1 TOF Repair Pearls

  • Modern: full repair in infancy (3-12 mo)
  • Historical: Blalock-Taussig palliative shunt
  • Late presentation rare in developed; common in developing world