馬凡氏症患者併發主動脈剝離之案例探討

Marfan Syndrome Complicated Aortic Dissection: A Case Report

黃微儀(Wei-Yi Huang) ； 陳名聖(Ming-Sheng Cheng) ； 鄭則有(Ze-You Zheng) ； 李基訓(Ji-Syun Li) ； 林新傑(Sin-Jie Lin) ； 洪嘉亨(Jia-Heng Hong) ； 詹前毅(Gian-Yi Zhan)

《北市醫放雜誌》 12卷1期 (2022/12) Pp. 1-9

https://doi.org/10.6663/TPART.202212_12(1).0001

摘要

馬凡氏症候群（Marfan syndrome，簡稱MFS）為一種遺傳性結締組織疾病，常伴有骨骼、眼睛、心血管系統異常，其中升主動脈剝離破裂更是造成猝死主因。本文探討一位患有MFS的55歲男性病人，經術後電腦斷層血管攝影追蹤檢查，診斷為復發性A型主動脈剝離；藉由影像上的表現，可清楚顯示出主動脈剝離程度、真假腔的血栓是否形成或是出現心包積液，對於病程與預後提供可靠的資訊。因此，在無創情況下，臨床醫師可藉CT輔助立即做診斷，並盡速給予醫藥與手術治療，來提升馬凡氏症患者之存活率。

關鍵字

馬凡氏症候群 ； 主動脈剝離 ； 電腦斷層血管攝影

並列摘要

Marfan syndrome (MFS) is a hereditary connective tissue disorder characterized by abnormalities in the bones, eyes, and cardiovascular system, especially dissection and rupture of the ascending aorta, which usually lead to sudden death. This article describes the case of a 55-year-old male patient with MFS who was diagnosed with chronic type A aortic dissection after postoperative computed tomography angiography. The imaging manifestations demonstrated the degree of aortic dissection, thrombus formation in the true and false lumens, or pericardial effusion, and hence, provided reliable information on the course and prognosis of the disease. Therefore, under non-invasive conditions, clinicians should be immediately assisted in making a diagnosis, and medical and surgical treatment should be administered as soon as possible to improve the survival rate of MFS patients.

並列關鍵字

Marfan syndrome ； Aortic dissection ； Computed Tomography Angiography