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马凡综合征合并射血分数降低患者的Bentall手术:病例报告

Bentall Procedure in a Marfan Syndrome Patient With Reduced Ejection Fraction: A Case Report.

作者信息

Bu Saeed Ghadeer F, Alshammari Manal F, Alamoud Abalkhail Dana S, Elghoneimy Yasser A

机构信息

College of Medicine and Surgery, Imam Abdulrahman Bin Faisal University, Dammam, SAU.

Department of Cardiac Surgery, King Fahad University Hospital, Dammam, SAU.

出版信息

Cureus. 2024 Dec 23;16(12):e76238. doi: 10.7759/cureus.76238. eCollection 2024 Dec.

Abstract

Marfan syndrome (MFS) is an autosomal dominant disorder affecting the connective tissue, often leading to aortic root dilation, aneurysm, and dissection. We report on a 35-year-old Bangladeshi female patient with MFS who presented with chest pain, shortness of breath, and a significant aortic root aneurysm, along with a reduced ejection fraction (EF) of 20%-25%. Imaging confirmed significant aortic dilation, and due to the high risk of mortality, an urgent Bentall procedure was performed. Postoperatively, the patient had an EF of 25% and was doing well at discharge. Timely Bentall procedure in MFS with severe aortic root dilation and reduced EF is critical for improving outcomes and reducing morbidity and mortality.

摘要

马凡综合征(MFS)是一种常染色体显性疾病,会影响结缔组织,常导致主动脉根部扩张、动脉瘤和夹层形成。我们报告了一名35岁患有马凡综合征的孟加拉女性患者,她出现胸痛、呼吸急促以及显著的主动脉根部动脉瘤,同时射血分数(EF)降低至20%-25%。影像学检查证实主动脉明显扩张,由于死亡风险高,遂紧急进行了Bentall手术。术后,患者的射血分数为25%,出院时情况良好。对于患有严重主动脉根部扩张且射血分数降低的马凡综合征患者,及时进行Bentall手术对于改善预后、降低发病率和死亡率至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae3e/11693403/35e08b4c48cf/cureus-0016-00000076238-i01.jpg

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