Management of a giant aortic root aneurysm in a young patient with Marfan syndrome: a case report
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(2020) 15:264
CASE REPORT
Open Access
Management of a giant aortic root aneurysm in a young patient with Marfan syndrome: a case report Jiayu Shen†, Changping Gan†, R. D. T. Rajaguru, Dou Yuan and Zhenghua Xiao*
Abstract Introduction: Marfan syndrome (MFS) is a common heritable connective tissue disease involving multiple organs. Even though the clinical manifestations of MFS can be various, aortic root aneurysm is estimated as one of the most serious complications. We herein describe an individualized treatment decision-making process for a 23-yearold male with MFS, suffering from a giant but stable aortic root aneurysm which is extremely rare at his age. Case: The patient, a 23-year-old male with a family history of MFS, presented to our cardiovascular department because of progressive exertional chest distress, fatigue and occasional precordial pain. Physical examinations revealed 190.5 cm of height, high myopia, and a diastolic murmur at the aortic valve area. Laboratory examinations for systemic vasculitis and infectious diseases were negative. Transthoracic echocardiography and enhanced thoracic computed tomography (CT) scan revealed the existence of a giant aortic root aneurysm (125.1 mm in short-axis), severe aortic valve regurgitation, cardiac dilatation (LV; 99 mm in diastolic diameter) and a poor ejection fraction (EF; 18%). Considering the risk of rupture or dissection of the dilated aortic root, we performed Bentall procedure based on the results of multidisciplinary team discussion and intraoperative exploration. Postoperative thoracic CT scan revealed a normal sized reconstructed aortic root, and the patient was discharged uneventfully 7 days later. Conclusion: It is extremely rare to report such a giant aortic root aneurysm in a young patient. In the treatment decision-making process, the patient’s specific situation should be taken into consideration. A mechanical Bentall procedure seems to be an acceptable option for some selected cases. Keywords: Aortic aneurysm, Cardiac surgery, Dilated aortic root, Marfan syndrome
Introduction Marfan syndrome (MFS) is an autosomal dominant disorder of connective tissues, which is primarily associated with the mutation of FBN-1 gene on chromosome 15q21 encoding fibrillin-1, an essential glycoprotein in the extracellular matrix [1]. Aortic root aneurysm and ectopia lentis are considered as the cardinal features of MFS [2]. Indeed, it is the progressive dilation of the * Correspondence: [email protected] † Jiayu Shen and Changping Gan contributed equally to this work. Department of Cardiovascular Surgery, West China Hospital of Sichuan University, No.37 Guo Xue Alley, Chengdu 610041, Sichuan, China
aorta leading to aortic rupture or dissection that affects the prognosis. Meanwhile, aortic regurgitation secondary to aortic root dilation leads to ventricular volume overload, left ventricular dilation and eventually cardiac function impairment [3]. In order to reduce the risk of aortic dissection or secondary cardiac dysfunction, replacement of the dilated
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