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

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

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Case Reports

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

Ghadeer F Bu Saeed et al. Cureus. .

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.

Keywords: aortic aneurysm; aortic regurgitation; bentall procedure; ejection fraction; marfan syndrome.

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Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. CT angiogram showing (A) axial view with a dilated ascending aorta and (B) coronal view displaying a dilated aortic annulus (2.7 cm) along with a proximal ascending aortic aneurysm (7 cm).
CT: computed tomography
Figure 2
Figure 2. (A,B) TEE showing a dilated aortic root (7.3 cm) with a small intimal flap localized at the aortic root.
TEE: transesophageal echocardiography

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