Bu Saeed Ghadeer F, Alshammari Manal F, Alamoud Abalkhail Dana S, Elghoneimy Yasser A
College of Medicine and Surgery, Imam Abdulrahman Bin Faisal University, Dammam, SAU.
Department of Cardiac Surgery, King Fahad University Hospital, Dammam, SAU.
Cureus. 2024 Dec 23;16(12):e76238. doi: 10.7759/cureus.76238. eCollection 2024 Dec.
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.
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。
