Al-Mohamad Hussein, Stout Kara, Bolling Taryn, Walsh Ronald
Department of Cardiology, Largo Medical Center, Largo, FL, USA.
Department of Internal Medicine, Largo Medical Center, Largo, FL, USA.
Case Rep Cardiol. 2020 Feb 23;2020:1704150. doi: 10.1155/2020/1704150. eCollection 2020.
. Marfan syndrome (MFS) is a rare connective tissue disorder attributed to a defect in the fibrillin-1 gene. Aortic aneurysms and dissection are common causes of morbidity and mortality in Marfan syndrome. . A 43-year-old female with a history of MFS and a 4.0 cm dilated ascending aorta presented to her cardiologist reporting that since a C-section two years prior, the left side of her abdomen painlessly protruded when standing. An outpatient CT scan of the abdomen/pelvis noted a 5.5 cm abdominal aortic dissection, and she was directed to the hospital. Repeat CT scan of the abdomen/pelvis revealed a 5.6 cm dissecting aneurysm of the infrarenal abdominal aorta. The patient was admitted to the ICU and started on a nitroglycerin drip to maintain systolic blood pressure less than 110 mmHg. The patient underwent repair of her abdominal aortic dissection via a retroperitoneal approach, and she tolerated the procedure well. She was started on metoprolol tartrate 12.5 mg BID and aspirin 81 mg postoperatively. She was safely discharged with follow-up care. . This case stresses the importance of having a low threshold to obtain imaging in a MFS patient with protruding abdomen, even though the patient may not have pain and be hemodynamically stable.
马凡综合征(MFS)是一种罕见的结缔组织疾病,归因于原纤蛋白-1基因缺陷。主动脉瘤和主动脉夹层是马凡综合征发病和死亡的常见原因。一名43岁女性,有马凡综合征病史,升主动脉扩张4.0厘米,她向心脏病专家报告称,自两年前剖宫产以来,站立时左侧腹部无痛性突出。门诊腹部/盆腔CT扫描发现腹主动脉夹层5.5厘米,随后她被送往医院。腹部/盆腔重复CT扫描显示肾下腹主动脉夹层动脉瘤5.6厘米。患者入住重症监护病房,开始静脉滴注硝酸甘油以维持收缩压低于110毫米汞柱。患者通过腹膜后入路接受了腹主动脉夹层修复术,手术耐受良好。术后开始服用酒石酸美托洛尔12.5毫克,每日两次,阿司匹林81毫克。她在接受随访护理后安全出院。该病例强调,对于有腹部突出的马凡综合征患者,即使患者可能没有疼痛且血流动力学稳定,也应保持较低的影像学检查门槛。