NON- DISSECTING LARGE AORTIC ARCH ANEURYSM LEADING TO CHRONIC AORTIC INSUFFICIENCY PRESENTING AS ACUTE HEART FAILURE REVEALING MARFAN SYNDROME

Mariam EL Harrak
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Abstract

Introduction: Marfan syndrome is an autosomal dominant disorder of connective tissue which has many clinical symptoms and whose prognosis depends on associated cardiovascular complications, dominated by progressive aortic root dilation, which may lead to aortic dissection, rupture or aortic regurgitation. Prevention of these life threatening complications is of major importance. Case presentation: We report here a case of a 42-year-old man, with a history of two retinal detachments in 2015 and 2018, who presented with severe aortic valve regurgitation and severe heart failure secondary to significant aortic root dilatation revealing Marfan syndrome. Unfortunately, before referring the patient to the surgeon, the patient ended up dying from an unrecovered cardiac arrest. Conclusion: It is very important to recognize Marfan syndrome on time and the preventive actions should be undertaken in order to avoid life-threatening consequences of this disorder. Echocardiography and especially the angioscanner represent the main tools used for the diagnosis of gravity and the follow-up of this condition.
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非剥离性大主动脉弓动脉瘤导致慢性主动脉功能不全,表现为急性心力衰竭,显示马凡氏综合征
马凡氏综合征是一种常染色体显性结缔组织疾病,有许多临床症状,预后取决于相关的心血管并发症,以进行性主动脉根扩张为主,可导致主动脉夹层、破裂或主动脉反流。预防这些危及生命的并发症非常重要。病例介绍:我们在此报告一例42岁男性,2015年和2018年有两例视网膜脱离病史,表现为严重的主动脉瓣反流和严重的心力衰竭,继发于主动脉根明显扩张,显示马凡氏综合征。不幸的是,在将病人转诊给外科医生之前,病人最终死于无法恢复的心脏骤停。结论:及时认识马凡氏综合征,及时采取预防措施,避免发生危及生命的后果是十分重要的。超声心动图,特别是血管扫描仪是用于诊断重力和这种情况的随访的主要工具。
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