Left Ventricular Cardiomyopathy in Mitral Valve Prolapse: Fact or Fiction?

C. Jost, M. Greutmann, H. Connolly, B. Naegeli, A. Faeh-Gunz, C. Scharf, R. Candinas, E. V. Buechel, R. Weber, C. Binggeli, O. Franzen, A. Medeiros-Domingo
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Abstract

In most patients with mitral valve prolapse (MVP) without severe mitral regurgitation (MR), left ventricular (LV) function is preserved. There are, however, patients with MVP who have unexplained LV dilatation and/or decreased LV function. An association between MVP and sudden cardiac death has also been reported. LV size and function may be affected by the type of MVP, severity of regurgitation, and cause of MVP (myxomatous degeneration versus fibroelastic deficiency). There is increasing evidence suggesting an intrinsic cardiomyopathy associated with MVP. The cardiomyopathy associated with MVP can also affect the right ventricle (RV). Although the impact on ventricular dimensions and function are usually subtle, these abnormalities can affect clinical and echocardiographic estimation of the severity of MR and may thus have an impact on therapeutic decisions. Particularly in patients with the most extreme forms of MVP (Barlow disease), and in patients with Marfan syndrome or other connective tissue disorders, a cardiomyopathy affecting the LV and RV may thus occur occasionally. A better understanding of LV impairment associated with MVP is important for risk assessment and clinical decision-making.
二尖瓣脱垂引起的左室心肌病:事实还是虚构?
在大多数没有严重二尖瓣反流(MR)的二尖瓣脱垂(MVP)患者中,左心室(LV)功能得以保留。然而,也有MVP患者有无法解释的左室扩张和/或左室功能下降。MVP与心源性猝死之间的关联也有报道。左室大小和功能可能受到MVP类型、反流严重程度和MVP原因(粘液瘤变性与纤维弹性缺乏)的影响。越来越多的证据表明内源性心肌病与MVP相关。MVP相关的心肌病也可影响右心室(RV)。虽然对心室尺寸和功能的影响通常是微妙的,但这些异常可以影响临床和超声心动图对MR严重程度的估计,从而可能影响治疗决策。特别是在最极端形式的MVP (Barlow病)患者,以及马凡氏综合征或其他结缔组织疾病患者中,影响左室和右室的心肌病可能偶尔发生。更好地了解与MVP相关的左室损伤对风险评估和临床决策很重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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