Argirò Alessia, Zampieri Mattia, Berteotti Martina, Marchi Alberto, Tassetti Luigi, Zocchi Chiara, Iannone Luisa, Bacchi Beatrice, Cappelli Francesco, Stefàno Pierluigi, Marchionni Niccolò, Olivotto Iacopo
Cardiomyopathy Unit, Careggi University Hospital, 50134 Florence, Italy.
Cardiac Surgery, Careggi University Hospital, 50134 Florence, Italy.
J Clin Med. 2021 Mar 1;10(5):951. doi: 10.3390/jcm10050951.
Hypertrophic cardiomyopathy (HCM) is a common myocardial disease characterized by otherwise unexplained left ventricular hypertrophy. The main cause of disabling symptoms in patients with HCM is left ventricular outflow tract (LVOT) obstruction. This phenomenon is multifactorial, determined both by anatomical and functional abnormalities: myocardial hypercontractility is believed to represent one of its major determinants. The anatomical anomalies are targeted by surgical interventions, whereas attenuating hypercontractility is the objective of old and new drugs including the novel class of allosteric myosin inhibitors. This review summarizes the current treatment modalities and discusses the emerging therapeutical opportunities focusing on the recently developed cardiac myosin ATPase inhibitors Mavacamten and CK-274. Novel surgical and interventional approaches are also discussed.
肥厚型心肌病（HCM）是一种常见的心肌疾病，其特征为左心室肥厚且病因不明。HCM患者出现致残症状的主要原因是左心室流出道（LVOT）梗阻。这种现象是多因素的，由解剖和功能异常共同决定：心肌过度收缩被认为是其主要决定因素之一。解剖异常是手术干预的目标，而减弱过度收缩则是包括新型变构肌球蛋白抑制剂在内的新旧药物的目标。本综述总结了当前的治疗方式，并讨论了聚焦于最近开发的心肌肌球蛋白ATP酶抑制剂马伐卡坦和CK-274的新兴治疗机会。还讨论了新型手术和介入方法。
