complete ransposition of Great Artery(D-TGA) 完全大動脈轉位
2018/04/20 07:22
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Background:
- TGA accounts for approximately 3 percent of all congenital heart disease (CHD) disorders 大動脈轉位約佔3%先天性心臟病
- Almost 20 percent of all cyanotic CHD defects 20%的發紺性心臟病源自於TGA
- 80 percent of patients with DiGeorge syndrome(22q11 deletion) 80%TGA伴有DiGeorge syndrome
Physicopathology:
- Unknown cause 病因未知
- Risk factors:
- Maternal: diabetes, rubella, poor nutrition, alcohol, >40y/o 包含了糖尿病、德國麻疹、營養不良、酗酒、四十歲以上產婦為高風險
- Abnormal growth and development of the bilateral subarterial conus 和動脈圓錐的異常發育有關
- Transposition of pulmonary artery and aorta 大動脈與肺動脈轉位
- Separated to 2 isolated circuit 原本正常的循環系統被分為兩個獨立的循環
- RA and RV are full of unsaturated oxygen; LA and LV are full of saturated oxygen 造成右心循環充滿缺氧血;而左心循環充滿充氧血(不交流)

Pic from:OB images, https://www.obimages.net/fetal-heart/dextro-transposition-of-the-great-arteries-d-tga/post-delivery-graphic-anatomy-d-tga/
Clinical presentation:
- Asymptom before birth(covered by foramen ovale, ductus arteriousus and ductus venous) 右心室充氧血可藉由軟圓孔、動脈導管以及靜脈導管流至體循環,故少見症狀
- After birth, to keep alive must have foramen ovale, ductus arteriosus and VSD to keep alive 為了保持胎兒存活,
- Cyanosis 發紺
- Congestive heart failure (RV pumps to aorta, too high pressure) 鬱血心心臟病(右心室需打入體循環,作功過大)
- RV hypertrophy and LV atrophy 作功大而造成右心肥大;而左心萎縮
Treatment and prevention:
- Prostaglandin therapy(PGE1)(i.v)→maintain ductal patency 給予PGE1維持動脈導管打開
- Balloon atrial septostomy (cardiac catheterization) 經心導管以氣球擴張
- Surgical repair
- Arterial switch operation (ASO) 手術-動脈置換術
Reference:
- Uptodate
- OB images
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