复杂型先天性心脏病大型主-肺动脉侧支血管的形态学特征

Morphological characteristics of major aortopulmonary collateral arteries in complex congenital heart disease

目的探讨复杂型先天性心脏病（先心病）患儿大型主一肺动脉侧支血管（major aortopul—monary collatera larteries，MAPCAs）的形态学特征。方法回顾性分析伴MAPCAs的复杂型先心病患儿的MAPCAs的影像学资料，总结MAPCAs发布规律、数量及形态学特征。本组51例伴MAPCAs的复杂型先心病患儿中肺动脉闭锁／室间隔缺损33例（64．71％），法洛四联症11例（21．57％），右心室双出口2例（3．92％），完全性大动脉转位1例（1．96％），完全性房室问隔缺损1例（1．96％），肺动脉狭窄1例（1．96％），肺动脉吊带1例（1．96％），肺动脉缺如1例（1．96％）。另外，合并动脉导管未闭16例（31．37％）。结果51例患儿中存在中央共汇43例（84．31％），MPCAs共117支，每例1～6支，平均2．29支。MAPCAs起始直径2．0～18．0mm，平均4．3mm。MAPCAs起源于胸降主动脉75支（64．10％），主动脉弓16支（13．68％），左锁骨下动脉12支（10．26％），右锁骨下动脉11支（9．40％），头臂干2支（1．71％），其他1支（0．85％）。本组3支（2．56％）MAPCAs开口狭窄及狭窄后扩张，57支（48．72％）MAPCAs在进入肺脏前出现迂回、扭曲；8例（15．69％）患儿MAPCAs为惟一血源供应相应肺段。另有13支（11例，均为肺动脉闭锁／室间隔缺损）较粗大的MAPCAs可见分支，其分支为2～4支，多为2支。结论MAPCAs多见于某些肺血减少型先心病，且分布较广泛，其起源、数量、大小、走行以及分支等方面变异较大，主要影响手术方式选择及疗效，了解MAPCAs的分布规律及形态学特征具有十分重要的临床意义。

Objective To investigate the mox＇phological characteristics of major aortopulmonary collateral arteries （MAPCAs） in complex congenital heart disease （CHD）. Methods Analyzed the imaging data of CHD with MAPCAs. The distribution, number and the morphological characteristics of MAPCAs were summarized. These patients cohort included 51 pa- tients of CHD with MAPCAs. Among them, 33 patients （64.71%） were pulmonary atrsia/ventricular septal defect, 11 （21.57%） were tetralogy of Fallot, 2 （3.92%） were double outlet right ventricle, 1 （ 1.96% ） was complete transposition of the great arteries, 1 （ 1.96% ） was complete atrloventricular septal defect, 1 （ 1.96% ） was puhnonary stenosis, 1 （ 1.96% ） was puhnonary artery sling, 1 （ 1.96% ） was absence of puhnonary artery. Besides, 16 patients （31.37%） had patent duetus arteriosus. Results In this group, central puhnonary arteries and confluent were present in 43 patients （ 84.31% ）. Totally, 117 MAPCAs were founded in 51 patients, 1 -6 per patients, mean 2.29 per patients. Theirs opening diameter were 2.0 - 18.0 ram, mean 4.3 mm. There were 16 MAPCAs （ 13.68% ） from aortic arch, 75 （64. 10% ） from thoracic descending aor- ta, 2 （1.71%） from braehioeephalic artery, 11 （9.40%） from right subclavian artery, 12 （10.26%） from left subclavian artery, and 1 （0. 85 % ） from other rare arteries. Three MAPCAs （2.56%） had narrowed in opening and followed by dilation, and 57 MAPCAs （48.72%） had squirm twist before turned into lungs. MAPCAs were the only supply of relative lobes in 8 pa- tients （15.96%）. Besides, 13 MAPCAs （ 11 patients with pulmonary artesia./ventricular septal defect） had their branches,with larger opening diameter. The number of MAPCAs branches were 2 - 4, usually 2. Conclusion MAPCAs were highly variable in their origin, number, size, course and arborization. It mainly impacted on the choice of operations. It was very im- portant to know about the distribution and morphological characteristics of MAPCAs.