Department of Cardiology, Klinikum Würzburg-Mitte, Juliusspital, Würzburg; Department of Thoracic and Cardiovascular Surgery, Saarland University Hospital, Homburg/Saar; University of Paris Descartes, Sorbonne Paris Cité, Paris, France.
Dtsch Arztebl Int. 2019 Jan 25;116(4):47-53. doi: 10.3238/arztebl.2019.0047.
Hypertrophic cardiomyopathy (HCM) is caused by mutations in a number of genes. Its prevalence is 0.2% to 0.6%.
This review is based on publications retrieved by a selective literature search and on the authors' clinical experi- ence.
70% of patients with HCM suffer from the obstructive type of the condition, clinically characterized by highly dynamic and variable manifestations in the form of dyspnea, angina pectoris, and stress-dependent presyncope and syn- cope. Younger patients are at particular risk of sudden cardiac death; thus, all patients need not only symptomatic treatment, but also risk assessment, which can be difficult in individual cases. Left ventricular obstruction, which usually causes symptoms, is treated medically at first, with either a beta- blocker or verapamil. If medical treatment fails, two invasive treatments are available, surgical myectomy and percu- taneous septum ablation. Both of these require a high level of expertise. If performed successfully, they lead to sustained gradient reduction and clinical improvement. Septum ablation is associated with low perioperative and peri-interventional mortality but necessitates permanent pacemaker implantation in 10-20% of patients.
In the absence of evidence from randomized comparison trials, a suitable method of reducing the gradient should be determined by an HCM team in conjunction with each individual patient. Important criteria for decision-making include the anatomical findings and any accompanying illnesses.
肥厚型心肌病（HCM）是由许多基因的突变引起的。其患病率为 0.2%至 0.6%。
本综述基于选择性文献检索和作者的临床经验中获得的出版物。
70%的 HCM 患者患有梗阻型疾病，临床上表现为呼吸困难、心绞痛和与压力相关的晕厥和晕厥，表现高度动态和多变。年轻患者尤其有发生心源性猝死的风险；因此，所有患者不仅需要对症治疗，还需要进行风险评估，而在某些情况下这可能很困难。通常引起症状的左心室梗阻首先进行药物治疗，使用β受体阻滞剂或维拉帕米。如果药物治疗失败，有两种侵入性治疗方法，即外科心肌切除术和经皮间隔消融术。这两种方法都需要高度的专业知识。如果成功实施，它们可导致梯度持续降低和临床改善。间隔消融术与低围手术期和围介入期死亡率相关，但需要在 10-20%的患者中永久植入起搏器。
在没有随机对照试验证据的情况下，HCM 团队应与每位患者一起确定适当的降低梯度的方法。决策的重要标准包括解剖学发现和任何伴随的疾病。
