Letter by Maron et al Regarding Article, "Genotype and Lifetime Burden of Disease in Hypertrophic Cardiomyopathy: Insights From the Sarcomeric Human Cardiomyopathy Registry (SHaRe)".
Letter by Maron et al Regarding Article, "Genotype and Lifetime Burden of Disease in Hypertrophic Cardiomyopathy: Insights From the Sarcomeric Human Cardiomyopathy Registry (SHaRe)".
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DOI：
10.1161/circulationaha.118.038189
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发表时间：
2019-03
期刊：
影响因子：
37.8
通讯作者：
B. Maron;E. Rowin;M. Maron
中科院分区：
文献类型：
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作者：
B. Maron;E. Rowin;M. Maron
Barry J. Maron, MD Ethan J. Rowin, MD Martin S. Maron， MD致编辑：作为经验丰富的肥厚性心肌病（HCM）临床研究者，我们不得不问Ho等人：HCM是如何突然又变成一种糟糕的疾病的？我们非常惊讶地发现，来自《循环》杂志上的SHaRe登记处（肌性人类心肌病登记处）的数据，促进了我们不再认识的HCM的愿景（即，严峻，无情，不完全治疗，预后差）。事实上，很难将Ho等人报道的HCM的高死亡率1与与良好生活质量相关的极低死亡率（每年0.5%）2相一致，也很难将高效的当代治疗干预措施（包括用于预防猝死的植入式除颤器、低风险：高效益的手术肌瘤切除术以逆转进行性心力衰竭）对HCM产生的重大影响的数据相一致。房颤导管消融（和迷宫手术）和抗凝用于预防卒中。因此，我们提出两个具体问题供考虑。首先，对同一疾病的预后和结果的两种截然相反的观点的解释是什么？ Ho等人1的结果数据是否因使用不太有力的治疗措施而受到不利的影响？例如，SHaRe数据似乎可以追溯到近60年前HCM开始出现的时候，在许多有效的治疗方法出现之前（尽管，矛盾的是，作者报告的中位随访期只有2.9年）。对于一种经常被患者错误描述的疾病，这些数据可能会造成混淆，这可能会对当代执业心血管社区的临床医生的态度产生不利影响，在这个HCM的现代治疗时代，这是一个特别不幸的可能性。其次，SHaRe登记中专门针对阻塞性HCM的管理措施尚不清楚，尽管几乎三分之一的患者有流出梯度（包括许多有限制症状的患者）。值得注意的是，对有症状的阻塞性HCM的治疗，如手术肌瘤切除术（或酒精性室间隔消融），未被提及，尽管这些干预措施已知可以可靠地逆转心力衰竭症状并延长寿命因此，Ho等人报告的高HCM死亡率能否部分归因于作者对阻塞性HCM的治疗不积极？本文提出这些问题是为了使临床医生能够充分理解SHaRe数据的局限性，而当代以治疗为导向的综合研究已经为hcm患者的临床病程、治疗和预后创造了更加现实和乐观的评估
March 19, 2019 1557 Barry J. Maron, MD Ethan J. Rowin, MD Martin S. Maron, MD To the Editor: As experienced hypertrophic cardiomyopathy (HCM) clinical investigators, we are compelled to ask Ho et al1: How did HCM suddenly become a bad disease...again? We were very surprised to encounter data from the SHaRe registriy (Sarcomeric Human Cardiomyopathy Registry) in Circulation1 that promote a vision of HCM we no longer recognize (ie, grim, unrelenting, incompletely treated, and with poor outcomes). Indeed, it is difficult to align the high mortality reported for HCM by Ho et al1 with the very low mortality rates (0.5% per year)2 associated with good quality of life and the data reporting the substantial impact that highly effective contemporary treatment interventions have made in this disease, including implantable defibrillators for sudden death prevention, low risk:high benefit surgical myectomy to reverse progressive heart failure, and atrial fibrillation catheter-ablation (and Maze procedure) and anticoagulation for stroke prevention.2–5 Therefore, we present 2 specific questions for consideration. First, what is the explanation for 2 polar opposite views of prognosis and outcome for the same disease, and could the outcome data of Ho et al1 have been skewed unfavorably by using less robust therapeutic initiatives? For example, the SHaRe data seem to extend back almost 60 years to the inception of HCM and well before many effective treatments were available (although, paradoxically, the authors report a median follow-up period of only 2.9 years). The confusion potentially created by these data for a disease too often mischaracterized to patients could adversely affect attitudes of clinicians in the contemporary practicing cardiovascular community, a particularly unfortunate possibility in this modern treatment era for HCM. Second, the management initiatives specifically for obstructive HCM in the SHaRe registry are unclear, although almost one-third of all patients had outflow gradients (including many with limiting symptoms). Remarkably, treatments for symptomatic obstructive HCM, such as surgical myectomy (or alternatively, alcohol septal ablation), are not mentioned, even though these interventions are known to reliably reverse heart failure symptoms and extend longevity.5 Hence, could the high HCM mortality reported by Ho et al1 be attributable in part to the authors’ less aggressive treatment of obstructive HCM? These questions are posed here so that clinicians may fully understand the limitations of the SHaRe data, in contrast to the comprehensive contemporary treatment-oriented studies that have created a much more realistic and optimistic appraisal of clinical course, treatment, and prognosis for patients with HCM.2–5