Vatta Matteo
Department of Pediatrics (Cardiology) and Molecular Physiology and Biophysics, Baylor College of Medicine and Texas Children's Hospital, Houston, TX 77030, USA.
G Ital Cardiol (Rome). 2010 Oct;11(10):746-52.
Cardiomyopathies represent a significant clinical and social issue due to their high morbidity and mortality. In addition to myocardial dysfunction, the often associated arrhythmias are an additional risk factor for morbidity and mortality in heart failure individuals. Arrhythmias are frequently correlated to cardiac pump failure, but they may develop in asymptomatic heart failure subjects with preserved ejection fraction and stable hemodynamic performance. Up to date, arrhythmias have been explained by the occurrence of cardiac valve diseases or myocardial morphological alterations. However, recent evidence suggests a tight structural and functional link, at the molecular level, between ion channels and cytoskeletal proteins involved in the structural alterations that lead to heart failure. Furthermore, mutations in these structural proteins may cause ion channel dysfunction resulting in a higher risk of arrhythmias. These new elements of investigation may allow a better understanding of the arrhythmogenic phenomenon in heart failure patients and facilitate alternative research approaches and innovative clinical applications.
心肌病因其高发病率和死亡率而成为一个重大的临床和社会问题。除心肌功能障碍外，常伴发的心律失常是心力衰竭患者发病和死亡的另一个危险因素。心律失常常与心脏泵衰竭相关，但也可能在射血分数保留且血流动力学表现稳定的无症状心力衰竭患者中发生。迄今为止，心律失常一直被解释为心脏瓣膜疾病或心肌形态改变的结果。然而，最近的证据表明，在分子水平上，参与导致心力衰竭的结构改变的离子通道与细胞骨架蛋白之间存在紧密的结构和功能联系。此外，这些结构蛋白的突变可能导致离子通道功能障碍，从而增加心律失常的风险。这些新的研究要素可能有助于更好地理解心力衰竭患者的心律失常现象，并促进替代研究方法和创新临床应用的发展。
