马凡氏病双主动脉夹层1例。病例报告

Christian Millogo Georges Rosa, Koudougou Kologo Jonas, Arthur Seghda Taryètba André, T. Boro, L. Benon, K. Samadoulougou André, P. Zabsonré
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引用次数: 0

摘要

马凡氏综合征是一种显性常染色体遗传病的结缔组织,导致各种并发症。以心血管并发症为主,主动脉扩张和主动脉夹层是主要的死亡原因。文献中很少报道不同分离部位的双重解剖。我们报告了一例22岁的年轻患者入院急性胸腹痛,心源性休克。调查报告双主动脉夹层,最近的一个a型和一个旧的b型。根特诊断标准用于诊断马凡氏病。手术治疗成功地纠正了A型夹层。早期诊断和适当处理主动脉并发症可降低马凡氏综合征患者的高死亡率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Double aortic dissection in a patient with Marfan disease. A case report
Marfan syndrome is a dominant autosomal genetic disease of the connective tissue, leading to various complications. Cardiovascular complications are the main ones, with dilation of the aorta and aortic dissection which is the main cause of death. Double dissection with different sites of departure is scarcely reported by the literature. We reported the case of a 22-year’s-old young patient admitted for acute chest and abdominal pains, and cardiogenic shock. Investigations reported a double dissection of the aorta with a recent one of type A and an old one of type B. Ghent diagnostic criteria were used to allow the diagnosis of Marfan disease. Surgical management corrected successfully the type A dissection. Early diagnostic and adequate management of aortic complications can reduce the high mortality rate of patients with Marfan syndrome.
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