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主动脉弓离断罕有单独发生的,常伴有各种心脏病,其中包括室间隔缺损、左心梗阻性病变、左室发育不良和单心室等。作者认为主动脉弓离断常伴有单一动脉弓或主肺动脉间隔缺损的临床表现,心血管造影是相似的,死前罕能作出诊断。本文叙述了心血管造影的表现并复习了有关文献。文内5例主动脉弓离断中,3例伴单一动脉干;2例伴主肺动脉间隔缺损。4例有尸检材料。年龄均在81/2月以下。3例有严重的心力衰竭伴轻至中度的紫绀,2例未见紫绀。主动脉弓离断伴发单一动脉干或主肺动脉间隔缺损是罕见的。复习文献收集到60例主动脉弓离断伴单一动脉干;25例主动脉弓离断伴主肺动脉间隔缺损。
Aortic arch segregation rarely occurs alone, often accompanied by a variety of heart diseases, including ventricular septal defect, left heart obstructive lesions, left ventricular dysplasia and single ventricle and so on. The author believes that aortic arch disconnection often accompanied by a single arch or main pulmonary septal defect clinical manifestations, cardiovascular angiography is similar to rare diagnosis before death. This article describes the performance of cardiovascular angiography and reviewed the literature. Five cases of aortic arch segmental in the text, 3 cases with a single trunk; 2 cases with main pulmonary septal defect. 4 cases of autopsy material. Age below 81/2 months. 3 cases had severe heart failure with mild to moderate cyanosis, 2 cases without cyanosis. Aortic arch disconnection with a single trunk or main pulmonary septal defect is rare. A review of the literature collected 60 cases of aortic arch off with a single trunk; 25 cases of aortic arch off with the main pulmonary septal defect.