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一名患有马凡综合征的年轻男性出现从升主动脉至腹主动脉的无症状性大主动脉夹层:一例挑战传统诊断模式的病例报告。

Asymptomatic large aortic dissection from ascending to abdominal aorta in a young man with Marfan syndrome: A case report challenging conventional diagnostic paradigms.

作者信息

Makhloof Mohammad, Alkheder Ahmad, Mazloum Abdaljawad, Muhammad Tareq, Alshara Mohammad, Baddour Firas

机构信息

Department of Cardiology, Almaza Military Hospital, Damascus, Syria.

Department of Otorhinolaryngology, Al Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria; Faculty of Medicine, Syrian Private University, Damascus, Syria.

出版信息

Int J Surg Case Rep. 2024 Oct;123:110292. doi: 10.1016/j.ijscr.2024.110292. Epub 2024 Sep 19.

Abstract

INTRODUCTION

Aortic dissection, a serious medical condition characterized by a tear in the aorta's inner layer leading to the establishment of a false channel within the vessel wall, in this report, we present a very rare case of asymptomatic substantial aortic dissection spanning from the ascending aorta to the abdominal aorta.

CASE PRESENTATION

A 36-year-old male with fever and cough was found to have clinical features suggestive of Marfan syndrome. Examination revealed bilateral fine crackles and a diastolic heart murmur. Imaging showed bilateral infiltrates, hyperinflation, and aortic dissection. Echocardiography confirmed severe aortic valve regurgitation and Stanford type A dissection. Despite recommended surgery, the patient opted for medical therapy due to financial constraints, showing stable cardiac anomalies after 6 months.

DISCUSSION

Aortic dissection, a critical cardiovascular emergency, affects mainly the elderly, exacerbated by hypertension, atherosclerosis, and connective tissue disorders. Early detection via CT and biomarkers is crucial. Type A dissections typically require surgical repair, while endovascular repair is used for complex Type B cases, reducing long-term mortality. The TEM system aids in categorization, guiding treatment. Conditions like Marfan syndrome significantly contribute to aortic wall degeneration, requiring close monitoring and intervention.

CONCLUSION

The coalescence of aortic dissections with Marfan syndrome underscores the need for tailored monitoring in high-risk populations and highlights the silent complexities of cardiovascular pathologies, urging refined diagnostics and therapeutic approaches.

摘要

引言

主动脉夹层是一种严重的医学病症,其特征是主动脉内层撕裂,导致血管壁内形成假腔。在本报告中,我们呈现了一例非常罕见的无症状性大量主动脉夹层病例,病变范围从升主动脉延伸至腹主动脉。

病例介绍

一名36岁发热、咳嗽的男性被发现具有提示马凡综合征的临床特征。检查发现双侧有细湿啰音及舒张期心脏杂音。影像学检查显示双侧浸润、肺过度充气及主动脉夹层。超声心动图证实存在严重主动脉瓣反流及A型主动脉夹层。尽管建议进行手术,但由于经济限制,患者选择了药物治疗,6个月后心脏异常情况保持稳定。

讨论

主动脉夹层是一种严重的心血管急症,主要影响老年人,高血压、动脉粥样硬化和结缔组织疾病会使其病情加重。通过CT和生物标志物进行早期检测至关重要。A型夹层通常需要手术修复,而复杂的B型病例则采用血管内修复,以降低长期死亡率。TEM系统有助于分类,指导治疗。马凡综合征等病症会显著导致主动脉壁退变,需要密切监测和干预。

结论

主动脉夹层与马凡综合征并存凸显了对高危人群进行针对性监测的必要性,并突出了心血管疾病隐匿的复杂性,促使采用更精细的诊断和治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/870d/11424808/bd4f621a513a/gr1.jpg

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