先天性主动脉缩窄病理形态及合并心脏缺损分析

Pathology and Associated Defects of Congenital Coarctation of the Aorta

目的主动脉缩窄(CoA)属于左室流出道梗阻疾病谱之一,本研究目的旨在分析CoA的病理形态和合并心脏缺损。方法回顾分析2008年10月-2009年12月手术治疗的74例CoA的临床资料。结果导管旁型26例(35.1%),导管前型17例(23.0%),导管后型2例(2.7%),弓和/或峡部发育不良25例。简单型CoA14例(18.9%),复杂型CoA60例(81.1%)。合并畸形包括室间隔缺损42例(56.8%)、动脉导管未闭39例(52.7%)、二叶式主动脉瓣18例(24.3%)、继发孔型房间隔缺损15例(20.3%)、主动脉瓣狭窄8例(10.8%)、二尖瓣狭窄3例(4.1%)、主动脉瓣下狭窄1例(1.4%)、主动脉瓣上狭窄1例(1.4%)。结论CoA以导管旁型最常见,也常表现为弓和/或峡部发育不良。室间隔缺损和动脉导管未闭是最常见的合并缺损。CoA常和其他左室流出道梗阻病变(二叶式主动脉瓣、主动脉瓣狭窄)合并发生,提示它们在发生上具有共同的机制。

Objective To analyze the pathology and associated defects of congenital coarctation of the aorta（CoA）,a common lesion among left ventricular outflow tract obstruction.Methods The clinical data of 74 CoA patients who received operation from October 2008 to December 2009 were retrospectively analyzed.Results Of the 74 CoA patients,juxtaductal type was found in 26 cases（35.1%）,preductal type in 17 cases（23.0%）,postductal type in 2 cases（2.7%）,and arch and/or isthmus hypoplasia in 25 cases（33.8%）.Simple coarctation was 14 cases（18.9%）,and complex coarctation 60 cases（81.1%）.Associated defects included ventricular septal defect 42 cases（56.8%）,patent ductus arteriosus 39 cases（52.7%）,bicuspid aortic valve 18 cases（24.3%）,secundum atrial septal defect 15 cases（20.2%）,aortic valve stenosis 8 cases（10.8%）,mitral stenosis 3 cases（4.1%）,subaortic stenosis 1 case（1.4%）,and supravalvar aortic stenosis 1 case（1.4%）.Conclusion Juxtaductal in location was the most common pathology of the CoA in our study.Arch and/or isthmus hypoplasia were also common pathology.Ventricular septal defect and patent ductus arteriosus were the most common associated lesions.CoA and other left ventricular outflow tract obstruction lesions including AVS and BAV often coexisted,suggesting they may share a common developmental cause.