巴洛氏病临床一例

E. Reznik, YuYu. . Golubev, N.V. Yurtaeva, D.V. Zayats, G.Yu. Ishchenko, A. I. Katkov, D. V. Puzenko, E. Novikova, P. V. Lednev, G. Golukhov
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摘要

Barlow's disease (BD)是退行性二尖瓣(MV)疾病的主要形式之一,其特征是结缔组织的形态学改变触发二尖瓣小叶适应失败的机制。该病的特点是由脊索断裂或伸长引起的中枢轴脱垂,小叶面积扩大和环扩张。24%的二尖瓣脱垂(MPV)患者有典型的双相障碍症状。需要注意的是,双相障碍是一种遗传性疾病。双相障碍患者多为年轻女性(小于40岁),长期无症状。双相障碍通常是在筛查检查中偶然诊断出来的。根治性的纠正这种中压功能障碍只能通过外科技术来实现。基于超声心动图的发现,可以区分两种类型的Barlow病:典型的有中压小叶增厚的Barlow病和非典型的没有增厚的Barlow病(可能与结缔组织发育不良有关)。本文重点介绍了符号学的基础和BD患者管理的主要原则。它提供了MVP和二尖瓣功能不全的原因的回顾,并描述了手术治疗BD的临床病例。关键词:二尖瓣反流,二尖瓣整形手术,Barlow病,二尖瓣脱垂,遗传性结缔组织疾病,发育不良。引文:Reznik e.v., Golubev Yu.Yu。, Yurtaeva N.V.等。巴洛氏病临床一例。俄罗斯医学调查。2023;7(1):41-48(俄文)。DOI: 10.32364 / 2587-6821-2023-7-1-41-48。
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Barlow's disease illustrated by a clinical case
Barlow's disease (BD) is one of the dominant forms of degenerative mitral valve (MV) disease characterized by morphological changes of the connective tissue triggering the mechanism of mitral valve leaflet coaptation failure. The disease is characterized by MV prolapse driven by chordal rupture or elongation, leaflet area enlargement and annular ectasia. Typical BD symptoms are found in 24% of patients with mitral valve prolapse (MPV). It should be noted that BD is a heritable disorder. Patients with BD are typically young women (less than 40-year-old), who remain asymptomatic for a long time. BD is often diagnosed incidentally, during the screening examination. Radical correction of such MV dysfunction can be achieved only by using surgical techniques. Based on echocardiography findings, two types of Barlow's disease are distinguished: a classical one with MV leaflet thickening and a non-classical — without its thickening (presumably associated with dysplasia of the connective tissue). This article is focused on the foundations of semiotics and the main principles of BD patient management. It provides a review of MVP and mitral incompetence causes and describes a clinical case of surgical BD treatment. KEYWORDS: mitral regurgitation, plastic surgery of the mitral valve, Barlow's disease, mitral valve prolapse, heritable disorders of connective tissue, dysplasia. FOR CITATION: Reznik E.V., Golubev Yu.Yu., Yurtaeva N.V. et al. Barlow's disease illustrated by a clinical case. Russian Medical Inquiry. 2023;7(1):41–48 (in Russ.). DOI: 10.32364/2587-6821-2023-7-1-41-48.
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