先天性主动脉-左心室通道四例的影像诊断

Imaging diagnoses of four cases with congenital aortic-left ventricular tunnel

目的 探讨先天性主动脉 左心室通道的影像表现和诊断特征。方法 4例病人 ,男 3例 ,女 1例 ;年龄 4～ 30岁 ,经超声心动图和心血管造影确诊为先天性主动脉 左心室通道。结果 升主动脉造影显示主动脉根部有异常的囊袋样突出管道进入左心室腔内。 1例拒绝手术 ,3例手术治疗 ,术中见升主动脉扩张 ,主动脉根部有一管状膨出形成隧道引流入左心室腔 ,2例合并有主动脉瓣关闭不全 ,同时行隧道修补和主动脉瓣替换术 ,治愈出院。结论 先天性主动脉 左心室通道是极为罕见的先天性心血管畸形 ,需早期诊断 ,尽早手术治疗 ,能避免出现主动脉瓣关闭不全和心力衰竭。

Objective To assess the diagnosis and characteristics of aortic-left ventricular tunnel.Methods Four cases (3 males and 1 female) of congenital aortic-left ventricular tunnel were studied,and the age ranged from 4 to 30 years.All patients were diagnosed by echocardiography and angiocardiography.Results The aortogram showed an unusual saccular aneurismal opacification of the tunnel that bypassed the aortic valve and terminated within the left ventricle.One case was discharged without surgery.Three cases were operated with good functional results.The operation showed dilated ascending aorta with an aneurismal bulge occupying the area of the Valsalva sinus.Aortic incompetence was also detected in 2 cases.Aortic-left ventricular tunnel repair and the aortic valve replacement were performed.Conclusion Aortic-left ventricular tunnel is a rare congenital abnormal communication between the aorta and the left ventricle.The disease has rarely been reported in the literature.Aortic-left ventricular tunnel needs early diagnosis and operation.Surgery is the only treatment and should be performed before the development of aortic incompetence or congestive heart failure.