Faber Lothar
Department of Cardiology, Heart and Diabetes Center North Rhine-Westphalia, University Hospital of the Ruhr University Bochum, Georgstraße 11, 32545 Bad Oeynhausen, Germany.
Adv Med. 2014;2014:464851. doi: 10.1155/2014/464851. Epub 2014 May 6.
Hypertrophic cardiomyopathy (HCM) is one of the more common hereditary cardiac conditions. According to presence or absence of outflow obstruction at rest or with provocation, a more common (about 60-70%) obstructive type of the disease (HOCM) has to be distinguished from the less common (30-40%) nonobstructive phenotype (HNCM). Symptoms include exercise limitation due to dyspnea, angina pectoris, palpitations, or dizziness; occasionally syncope or sudden cardiac death occurs. Correct diagnosis and risk stratification with respect to prophylactic ICD implantation are essential in HCM patient management. Drug therapy in symptomatic patients can be characterized as treatment of heart failure with preserved ejection fraction (HFpEF) in HNCM, while symptoms and the obstructive gradient in HOCM can be addressed with beta-blockers, disopyramide, or verapamil. After a short overview on etiology, natural history, and diagnostics in hypertrophic cardiomyopathy, this paper reviews the current treatment options for HOCM with a special focus on percutaneous septal ablation. Literature data and the own series of about 600 cases are discussed, suggesting a largely comparable outcome with respect to procedural mortality, clinical efficacy, and long-term outcome.
肥厚型心肌病(HCM)是较为常见的遗传性心脏疾病之一。根据静息或激发状态下有无流出道梗阻,该病较常见的(约60 - 70%)梗阻型(HOCM)必须与较不常见的(30 - 40%)非梗阻型表型(HNCM)相区分。症状包括因呼吸困难、心绞痛、心悸或头晕导致的运动受限;偶尔会发生晕厥或心源性猝死。在肥厚型心肌病患者管理中,关于预防性植入植入式心脏复律除颤器(ICD)的正确诊断和风险分层至关重要。有症状患者的药物治疗在HNCM中可表现为对射血分数保留的心力衰竭(HFpEF)的治疗,而HOCM中的症状和梗阻压差可用β受体阻滞剂、丙吡胺或维拉帕米来处理。在对肥厚型心肌病的病因、自然史和诊断进行简短概述后,本文回顾了HOCM的当前治疗选择,特别关注经皮室间隔消融术。讨论了文献数据及自身约600例病例系列,提示在手术死亡率、临床疗效和长期预后方面结果大致相当。