Douglas John S
Division of Cardiology, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia, USA.
Cardiovasc Diagn Ther. 2020 Feb;10(1):36-44. doi: 10.21037/cdt.2019.07.02.
Hypertrophic cardiomyopathy is a genetically determined disorder resulting in left ventricular hypertrophy. In a majority of the estimated 20 million people affected worldwide, left ventricular outflow obstruction is present at rest or with provocation. The presence and degree of obstruction influence the symptomatic presentation, treatment strategies and prognosis of affected individuals. Pharmacologic therapy with beta-adrenergic blocking drugs and calcium channel blockers is the principal treatment strategy in symptomatic patients with left ventricular outflow obstruction but is ineffective in many patients. When symptoms of exertional shortness of breath, chest pain and/or syncope prove refractory to medical therapy and there is persisting left ventricular outflow obstruction, or when there is drug intolerance, septal reduction strategies (surgical myectomy and alcohol septal ablation) are quite effective. Selection of the optimal septal reduction strategy for a given patient has become controversial and is determined largely by the medical system providing treatment strategies for the patient. Regretably, there are no randomized trials comparing myectomy and ablation and none are anticipated. The comprehensive Hypertrophic Cardiomyopathy Guideline Statements published in 2011 and 2014 differ significantly with the earlier statement favoring surgical myectomy and the more recent statement giving equal class I status to the two septal reduction strategies in adult patients with drug-refractory symptoms. Recently published studies of long-term follow-up of patients after alcohol septal ablation in Europe, where surgical myectomy is rarely performed, confirm long-term safety and effectiveness with survival free of cardiac events exceeding 96% at 15 years. The lesser degree of discomfort and more rapid recovery associated with the minimally invasive catheter-based alcohol ablation procedure coupled with the recently published long-term safety data favor an increased use of this strategy in symptomatic adult patients with hypertrophic obstructive cardiomyopathy (HOCM).
肥厚型心肌病是一种由基因决定的疾病,可导致左心室肥厚。在全球估计受影响的2000万人中,大多数人在静息或激发状态下存在左心室流出道梗阻。梗阻的存在和程度会影响患者的症状表现、治疗策略及预后。对于有左心室流出道梗阻的有症状患者,使用β-肾上腺素能阻滞剂和钙通道阻滞剂进行药物治疗是主要的治疗策略,但对许多患者无效。当劳力性气短、胸痛和/或晕厥症状经药物治疗无效且存在持续性左心室流出道梗阻时,或存在药物不耐受情况时,减隔策略(外科室间隔心肌切除术和酒精室间隔消融术)非常有效。为特定患者选择最佳的减隔策略已颇具争议,很大程度上取决于为该患者提供治疗策略的医疗体系。遗憾的是,尚无比较心肌切除术和消融术的随机试验,也没有此类试验的预期。2011年和2014年发布的肥厚型心肌病综合指南声明与早期声明有显著差异,早期声明倾向于外科室间隔心肌切除术,而最新声明赋予这两种减隔策略在药物难治性症状成年患者中同等的I类地位。最近在欧洲发表的关于酒精室间隔消融术后患者长期随访的研究(欧洲很少进行外科室间隔心肌切除术)证实了其长期安全性和有效性,15年时无心脏事件生存超过96%。与基于导管的微创酒精消融术相关的不适程度较轻且恢复较快,再加上最近公布的长期安全性数据,有利于在有症状的肥厚型梗阻性心肌病(HOCM)成年患者中更多地使用这种策略。