289 Ch 288. Tetralogy of Fallot, Coarctation of Aorta, Transposition + Eisenmenger Syndrome

TOF 為最常見 cyanotic CHD(4 特徵:RVOT obstruction + VSD + overriding aorta + RVH),現代兒童期手術後存活 > 90%;post-TOF complicationspulmonic regurgitation (PR) 是最常見,RV 擴張 → VT/SCD、QRS > 180 ms 是 SCD 標記;TPVR (Melody / Harmony) 為現代解方;Coarctation = 主動脈狹窄(preductal infantile vs postductal adult,後者多在 L subclavian 後);HTN + radial-femoral delay;balloon angioplasty + stent 是成人首選;TGA 分 D-TGA(complete)+ ccTGA(congenitally corrected, L-TGA),現代用 arterial switch(Jatene),older patients 為 atrial switch (Mustard/Senning),long-term 風險為 systemic RV failure + arrhythmia;Eisenmenger syndrome = L→R 反轉成 R→L from progressive PAH,治療 = PAH-specific therapy(BREATHE-5 bosentan)+ supportive care;absolutely AVOID pregnancy in Eisenmenger(maternal mortality 30-50%)。