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一例罕见的副二尖瓣组织导致左心室流出道梗阻,合并降落伞样二尖瓣、室间隔缺损、二叶式主动脉瓣、未破裂的主动脉窦瘤:病例报告。

A rare case of accessory mitral valve tissue causing left ventricular outflow tract obstruction associated with parachute mitral valve, ventricular septal defect, bicuspid aortic valve, unruptured aneurysm of aortic sinus: a case report.

作者信息

Li Yanan, Hu Yanbin, Wang Jiaxiang, Liu Lin

机构信息

Department of Cardiovascular Ultrasound, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, China.

Department of Cardiac Surgery, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, China.

出版信息

Eur Heart J Case Rep. 2018 Jul 16;2(3):yty082. doi: 10.1093/ehjcr/yty082. eCollection 2018 Sep.

Abstract

BACKGROUND

Accessory mitral valve tissue rarely causes left ventricular outflow tract obstruction in adults. It is often associated with other cardiac and vascular congenital malformations. Here, we report the rarest presentation of accessory mitral valve tissue (AMVT) causing left ventricular outflow tract obstruction.

CASE SUMMARY

A 22-year-old female patient presented with a history of shortness of breath and chest pain for more than 5 years. A diagnosis of AMVT with parachute mitral valve, ventricular septal defect (VSD), bicuspid aortic valve, unruptured aneurysm of aortic sinus, and left ventricular outflow tract obstruction was made. Successful closure of VSD with mitral valve replacement, excision of AMVT, and repair of the aortic sinus were performed. The post-operative course was uneventful, and an echocardiogram showed complete resection of the accessory mitral valve, no residual shunt and no left ventricular outflow gradient. Additionally, the peak gradient of rapid filling phase and atrial systolic phase across the prosthetic mitral valve were 16 mmHg and 4 mmHg, respectively. The peak velocity across left ventricular outflow tract was 1.4 m/s.

DISCUSSION

Accessory mitral valve tissue is associated with other cardiac abnormalities and is usually diagnosed in the first or second decade of life. It is responsible for left ventricular outflow tract obstruction. The obstruction can occur in the early period of life due to continued deposition of fibrous tissues within left ventricular outflow tract. Accessory mitral valve tissue should be considered a rare but important cause of left ventricular outflow tract obstruction.

摘要

背景

二尖瓣附属组织在成人中很少引起左心室流出道梗阻。它常与其他心脏和血管先天性畸形相关。在此,我们报告二尖瓣附属组织(AMVT)导致左心室流出道梗阻的最罕见表现。

病例摘要

一名22岁女性患者有5年多的呼吸急促和胸痛病史。诊断为AMVT合并降落伞样二尖瓣、室间隔缺损(VSD)、二叶式主动脉瓣、主动脉窦未破裂动脉瘤及左心室流出道梗阻。成功进行了室间隔缺损闭合术、二尖瓣置换术、AMVT切除术及主动脉窦修复术。术后过程顺利,超声心动图显示二尖瓣附属组织完全切除,无残余分流且无左心室流出梯度。此外,经人工二尖瓣快速充盈期和心房收缩期的峰值梯度分别为16 mmHg和4 mmHg。左心室流出道的峰值速度为1.4 m/s。

讨论

二尖瓣附属组织与其他心脏异常相关,通常在生命的第一个或第二个十年被诊断出来。它是左心室流出道梗阻的原因。由于左心室流出道内纤维组织的持续沉积,梗阻可在生命早期发生。二尖瓣附属组织应被视为左心室流出道梗阻的一种罕见但重要的原因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3311/6177097/4bf21bd89c03/yty082f1.jpg

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