伴有或不伴有左心室流出道（LVOT）梗阻的肥厚型心肌病（HCM）是一种常见的原发性心肌疾病，患病率为1:500。其特征是心肌增厚。其诊断评估包括病史采集和体格检查、基因研究、经胸超声心动图和心脏 MRI。如果接受最佳治疗，每年的死亡率低于 1%。本综述基于选择性文献检索检索到的相关出版物，包括当前的指南。在有症状的高 LVOT 梯度 (≥ 50 mm Hg) 患者中，首选治疗是使用非血管舒张性β受体阻滞剂或非二氢吡啶型钙通道拮抗剂的药物治疗。常见的副作用包括心动过缓和低血压，并且存在房室结阻滞的风险。两种物质类别都会降低 LVOT 梯度。 β-受体阻滞剂可缓解呼吸困难并改善患者的生活质量。维拉帕米可以增强身体恢复能力。另一种选择是 mavacamten，这是一种肌球蛋白抑制剂，于 2023 年中期在德国获得批准：它也可以降低 LVOT 梯度并改善生活质量。 7-10% 的患者左心室射血分数可逆性降低至 50% 以下。如果药物治疗失败，可以考虑鼻中隔复位治疗。还必须注意房颤、恶性心律失常和二尖瓣关闭不全等后遗症的处理。如果早期诊断并按照指南进行治疗，HCM 患者的预期寿命将接近正常。 HCM 和 HOCM（肥厚性梗阻性心肌病）的治疗仅在少数临床试验中进行过研究，还需要具有临床终点的随机研究。

Hypertrophic cardiomyopathy (HCM) with or without left ventricular outflow tract (LVOT) obstruction is a common primary myocardial disease, with a prevalence of 1:500. It is characterized by thickening of the myocardium. Its diagnostic evaluation includes history-taking and physical examination, genetic studies, transthoracic echocardiography, and cardiac MRI. When optimally treated, it carries a mortality of less than 1% per year.This review is based on pertinent publications retrieved by a selective literature search, including the current guidelines.In symptomatic patients with high LVOT gradients (≥ 50 mm Hg), the treatment of first choice is pharmacotherapy with non-vasodilating beta-blockers or non-dihydropyridine-type calcium channel antagonists. Common side effects include bradycardia and hypotension, and there is a risk of AV nodal blockade. Both substance classes lower the LVOT gradient. Beta-blockers alleviate dyspnea and improve patients' quality of life. Verapamil can increase physical resilience. A further option is mavacamten, a myosin inhibitor that gained approval in Germany in mid-2023: it, too, lowers the LVOT gradient and improves quality of life. In 7-10% of patients, there is a reversible reduction of the left ventricular ejection fraction to less than 50%. Septal reduction treatments can be considered if drug therapy fails. Attention must also be paid to the management of sequelae such as atrial fibrillation, malignant arrhythmias, and mitral valve insufficiency.Patients with HCM have a near-normal life expectancy if the disease is diagnosed early and treated according to the guidelines. The treatment of HCM and HOCM (hypertrophic obstructive cardiomyopathy) have been studied in no more than a few clinical trials, and randomized studies with clinical endpoints are needed.