主动脉弓离断三联征的放射诊断

Imaging diagnosis of triad of interrupted aortic arch

目的 :分析与评价主动脉弓离断 (IAA)三联征的影像学表现及其诊断价值。方法 :回顾性分析 2 0例患者的X线平片、心导管检查和心血管造影资料 ,其中 7例行电子束CT检查 ,11例行手术治疗 ,逐个分析其X线平片征象并与心血管造影及手术结果对照。结果 :X线胸片显示肺血多 ,肺动脉段明显凸出和心脏增大 (n =2 0 ) ;右上纵隔大血管影变窄 (n =16) ;主动脉结观察不清 (n =16) ;侧位或左前斜位见低位主动脉弓 (n =12 ) ;降主动脉的顶端与主肺动脉处同一水平 (n =8)。心导管检查均提示肺动脉高压、主动脉弓畸形、动脉导管未闭。心血管造影诊断IAA(A型 ) 13例 ,IAA(B型 ) 5例 ,其中合并主肺间隔缺损 4例 ,合并右室双出口 2例 ,右肺动脉起源异常 1例。结论 :X线平片对该畸形的诊断有一定的价值 ,多数病例可作诊断 。

Objective:To analyse and evaluate the imaging findings of triad of interrupted aortic arch. Methods:Data from twenty patients on X ray chest films,cardiac catheterization and angiocardiography were retrospectively analyzed. Among those patients, seven patients performed electron beam computed tomography (EBCT),eleven patients underwent successfully surgical repair.All chest radiographs were studied retrospectively and correlated with angiocardiography and surgical results.Results:X ray chest films showed pulmonary congestion in both lung fields, prominance of pulmonary segment and cardiomegaly ( n=20 ),narrowing of right upper mediastinal shadow ( n=16 ),obscurity of aortic knuckle ( n=16 ), a 'low arch' on lateral or left anterior oblique projections( n=12 ),and upper margin of the decending thoracic aortic shadow at the level of the main pulmonary artery ( n=8 ).Cardiac catheterization suggested pulmonary hypertension, anomaly of aortic arch,and patent ductus arteriosus. All patients were confirmed by angiocardiography including 13 cases IAA(A) and 5 cases IAA(B).Among them ,4 patients associated with aortopulmonary window (APW),2 patients with double outlet of right ventricule,and 1 patient with aortic origin of right pulmonary artery.Conclusion:About 60% cases of IAA can be diagnosed by findings on plain chest film.Angiocardiography is the most reliable method in diagnosis of triad of interrupted aortic arch.