ESC心肌病2023年指南解读与心肌病心脏性猝死的防治策略

ESC Guidelines in 2023 for the Management of Cardiomyopathy and Strategies for Prevention and Treatment of Sudden Cardiac Death in Cardiomyopathy

心肌病是一类以心肌结构和功能异常为特征的异质性疾病,病因复杂,原因不明特别是基因相关者,称为原发性心肌病,包括肥厚型、扩张型、非扩张型左室心肌病,限制性和致心律失常性右室心肌病及未定型心肌病等类型,编码心肌细胞的基因突变在心肌病发病中起着重要作用。心肌病患者的预后很大程度上取决于病因类型、严重程度、管理、治疗策略以及病人本身的健康状态。2023年8月发布的《2023 ESC心肌病管理指南》是包含所有心肌病类型的指南,对各种类型心肌病,特别是非扩张型左室心肌病和儿童心肌病做了诊断和治疗指导性意见,并对抗凝治疗和心脏性猝死的预防更新了指导建议。本文将结合新指南对心肌病的诊断及治疗进展及心肌病合并心脏性猝死问题进行讨论。

Cardiomyopathy is a complex condition characterized with structural and functional abnormalities of the myocardium.The etiology is multifaceted,and primary cardiomyopathy is related to those with unknown causes,esp.genetic mutations.The category encompasses hypertrophic,dilated,non-dilated left ventricular myocardial diseases,restrictive and arrhythmogenic right ventricular cardiomyopathy,as well as unclassified myocardial diseases.Mutations of genes encoding myocardial cells are important in the pathogenesis of cardiomyopathy.The prognosis of cardiomyopathy relies on the etiology,severity,treatment strategies,and the overall health status of patients.In August 2023,the"2023 ESC Guidelines for the management of myocardial diseases"were published.The guidline includes all types of cardiomyopathy and offers diagnostic and therapeutic guidance for various cardiomyopathy,emphasizing non-dilated left ventricular and pediatric cardiomyopathy.Diagnosis and treatment of cardiomyopathy based on this guideline,as well as sudden cardiac death associated with cardiomyopathy are reviewed.