马凡综合征MSCT血管成像表现及临床价值  

Imaging manifestations and clinical value of MSCT angiography of Marfan＇s syndrome

作　　者：熊青峰[1] 马小静[1] 鲁锦国[1] 陈艳[1] 张雪莲[1] 陈浩[1] 彭志远[1] 

机构地区：[1]武汉亚洲心脏病医院放射科,湖北武汉430022

出　　处：《实用放射学杂志》2013年第11期1777-1779,共3页Journal of Practical Radiology

摘　　要：目的 探讨马凡综合征MSCTA表现及临床价值.方法 回顾性分析行MSCT增强扫描马凡综合征44例,观察CT表现,其中30例行手术治疗,观察术后CT表现.结果 44例马凡综合征,主动脉根部呈瘤样扩张,直径约42～100 mm.主动脉夹层22例,主动脉窦部内膜撕脱2例,心脏瓣膜病12例,2例合并室间隔缺损.骨骼畸形21例,骶管扩张29例,骶管蛛网膜囊肿4例,骶脊膜膨出1例.22例行Bentall手术,8例行Bentall＋全弓置换术＋降主动脉支架象鼻术,其中同时行左前降支搭桥1例.1例胸主动脉壁内血肿支架植入术后行升主动脉人工血管置换术,术后2年出现腹主动脉穿透性溃疡,1例Bentall术后3年出现腹主动脉瘤合并主动脉壁内血肿,1例行室间隔缺损修补及升主动脉成形术,术后5年出现Debakey typeⅠ型夹层及二尖瓣重度反流,行Bentall术及二尖瓣置换术.2例Bentall术后2年再发Debakey type Ⅲ型夹层.1例行Bentall术后1年出现主动脉根部假性动脉瘤.1例Debakey type Ⅲ型夹层,3年后发展为Debakey typeⅠ型夹层.结论 主动脉MSCTA扫描可准确评价主动脉病理改变,明确主动脉根部形态、心脏合并畸形及脊柱畸形,是诊断马凡综合征一种重要方法.Objective To evaluate the imaging manifestations and clinical values of MSCTA in diagnosis of Marfan＇s syndrome. Methods The imaging manifestations and clinical values were retrospectively analyzed in 44 cases with MaHan＇s syndrome, which performed the process of contrast-enhanced MSCT. There were 30 cases which received operation, and the postoperative imaging manifestations were assessed again. Results The aortic roots of all of the 44 cases were dilated with a diameter of 42 100 mm. There were 22 cases with aortic dissection, including 2 cases with intimae avulsing, 12 cases with valvular heart disease, and 2 of them accompanying with ventricular septal defect. 21 cases with skeletal deformity, 29 cases with sacral canal dilation, 4 cases with sacral canal arachnoid cyst, and 1 case with saero-spinal meninges bulging. In the 30 cases which received operation, 22 cases received Bentall operation, 8 cases received Bentall operation and total arch replacement combined with implantation of stented elephant trunk into the descending aorta, 1 case received coronary artery bypass grafting at the anterior descending branch in the operation. 1 case received ascending aorta replacement operation after stent implantation into the thoracic aorta with intramural hematoma, which appeared abdominal aorta penetrating ulcer 2 years later. 1 case appeared abdominal aorta aneurysm with intramural hematoma 3 years after Bentall operation. 1 case received ventricular septal repairing, ascending aorta forming, which appeared Debakey type Ⅰ aorta dissection and heavy regurgitation of mitral valve 5 years later. 2 cases appeared Debakey type Ⅲ dissection 2 years after Bentall operation. 1 case appeared pseudoaneurysm at the aortic root 1 year after Bentall operation. 1 case was Debakey type Ⅲ dissection, which developed Debakey type Ⅰ dissection 3 years later. Conclusion MSCT can accurately evaluate the morphology of the whole aorta, including the aortic root, accompanying cardiovascular malformations, and the spinal defo

关 键 词：心血管疾病 马凡综合征 动脉瘤 计算机体层成像 心血管造影术 

分 类 号：R543[医药卫生—心血管疾病] R732.21[医药卫生—内科学]