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首页> 美国卫生研究院文献>Discoveries >The mechanisms diagnosis and management of mitral regurgitation in mitral valve prolapse and hypertrophic cardiomyopathy
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The mechanisms diagnosis and management of mitral regurgitation in mitral valve prolapse and hypertrophic cardiomyopathy

机译：二尖瓣脱垂和肥厚型心肌病的二尖瓣关闭不全的机制诊断和处理

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Valvular disease is a frequent cardiac pathology leading to heart failure and, ultimately, death. Mitral regurgitation, defined as the inability of the two mitral leaflets to coapt, is a common valvular disease and a self sustained pathology. A better understanding of the mitral valve histological layers provides a better understanding of the leaflet and chordae changes in mitral valve prolapse.Mitral valve prolapse may occur in myxomatous degenerative abnormalities, connective tissue disorders or in sporadic isolated cases. It is the most common mitral abnormality of non-ischemic cause leading to severe surgery-requiring mitral regurgitation. In addition to standard echocardiographic investigations, newly implemented three-dimensional techniques are being used and they permit a better visualisation, from the so-called ‘surgical view’, and an improved evaluation of the mitral valve.Hypertrophic cardiomyopathy is the most frequent inherited myocardial disease caused by mutations in various genes encoding proteins of the cardiac sarcomere, leading to a marked left ventricular hypertrophy unexplained by other comorbidities. The pathological echocardiographic hallmarks of hypertrophic cardiomyopathy are left ventricular hypertrophy, left ventricular outflow tract obstruction and systolic anterior motion of the mitral valve. The systolic anterior motion of the mitral valve contributes to the development of mitral regurgitation and further narrows the left ventricular outflow tract, leading to more severe symptomatology. Cardiac magnetic resonance imaging accurately measures the left ventricular mass, the degree of diastolic function and it may also be used to distinguish phenotypic variants.The clinical outcome of patients with these pathologies is mostly determined by the selected option of treatment. The purpose of surgical correction regarding mitral valve involvement is to restore valvular competence. Surgery has proven to be the only useful treatment in preventing heart failure, improving symptomatology and reducing mortality. Our approach wishes to enhance the understanding of the mitral valve’s involvement in hypertrophic cardiomyopathy and mitral valve prolapse from genetic, haemodynamic and clinical perspectives, as well as to present novelties in the grand field of treatment.
机译：瓣膜疾病是导致心脏衰竭甚至最终死亡的常见心脏病理。二尖瓣关闭不全（定义为两个二尖瓣小叶无法接合）是一种常见的瓣膜疾病，是一种自我持续的病理。更好地了解二尖瓣组织学层可以更好地了解二尖瓣脱垂的小叶和腱索变化。二尖瓣脱垂可能发生在粘液性变性异常，结缔组织疾病或零星的孤立病例中。这是非缺血性疾病最常见的二尖瓣异常，导致需要手术的严重二尖瓣关闭不全。除了标准的超声心动图检查外，还使用了新近实施的三维技术，这些技术可从所谓的“手术视野”更好地显示影像，并改善二尖瓣的评估。肥厚型心肌病是最常见的遗传性心肌病，由编码心肌肌节蛋白的各种基因突变引起，导致明显的左心室肥大，其他合并症无法解释。肥厚型心肌病的病理超声心动图标志是左心室肥大，左心室流出道梗阻和二尖瓣收缩前移。二尖瓣的收缩前运动有助于二尖瓣反流的发展，并进一步缩小左心室流出道，导致更严重的症状。心脏磁共振成像可准确测量左心室质量，舒张功能程度，也可用于区分表型变异。具有这些病变的患者的临床结局主要取决于所选择的治疗方案。有关二尖瓣受累的手术矫正的目的是恢复瓣膜功能。事实证明，手术是预防心力衰竭，改善症状和降低死亡率的唯一有用方法。我们的方法希望从遗传，血液动力学和临床角度增进对二尖瓣参与肥厚型心肌病和二尖瓣脱垂的了解，并提出治疗领域的新颖性。

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