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首页> 外文期刊>Circulation journal >Clinical Characteristics and Natural History of Hypertrophic Cardiomyopathy With Midventricular Obstruction
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Clinical Characteristics and Natural History of Hypertrophic Cardiomyopathy With Midventricular Obstruction

机译：肥厚型心肌病合并室间隔梗阻的临床特征和自然病史

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Background: ?The prevalence, clinical characteristics and natural history of patients with hypertrophic cardiomyopathy (HCM) and midventricular obstruction (MVO) have not been adequately studied. Methods and Results: ?A single-center cohort consisting of 423 patients (mean age, 49.3±17.2 years; 66.2% male) was thoroughly followed up for a median of 84 months (7 years; range, 6–480 months). MVO, characterized by the echocardiographic appearance of midventricular muscular apposition with a simultaneous mid-cavitary gradient ≥30mmHg, was identified in 34 patients (8%). Patients with MVO tended to be more symptomatic during their initial evaluation (>90% presented with NYHA class ≥II) compared to the rest of the HCM cohort. Apical aneurysm formation was identified in more than one-fourth of patients with MVO (26.5%), being a characteristic of the group. On multivariate Cox regression hazard analysis, presence of MVO strongly predicted progression to end-stage (burnt out) HCM and related heart failure (HF) deaths (hazard ratio, [HR], 2.62; 95% confidence interval [CI]: 1.2–8.8; P=0.047), as well as sudden death and associated lethal arrhythmic events (HR, 3.3; 95% CI: 1.26–8.85; P=0.016). Conclusions: ?MVO is a distinct phenotype of HCM associated with unfavorable prognosis in terms of end-stage HCM, sudden death and lethal arrhythmic events. The high adverse outcome rate necessitates early recognition of MVO and appropriate therapeutic interventions.??( Circ J ?2013; 77: 2366–2374)
机译：背景：？对肥厚型心肌病（HCM）和脑室中部梗阻（MVO）的患病率，临床特征和自然病程尚未进行充分的研究。方法和结果：①对一个包括423名患者（平均年龄49.3±17.2岁;男性66.2％）的单中心队列进行了彻底的随访，平均中位时间为84个月（7年;范围6–480个月）。在34例患者（8％）中发现了MVO，其特征是室中肌并发超声心动图，同时腔内梯度同时≥30mmHg。与HCM队列的其余部分相比，MVO患者在其初始评估期间倾向于出现症状（> 90％的NYHA≥II级患者）。在该组的特征中，超过四分之一的MVO患者（26.5％）发现了根尖动脉瘤的形成。在多因素Cox回归风险分析中，MVO的存在强烈预测了晚期HCM（倦怠）HCM的进展以及相关的心力衰竭（HF）死亡（风险比[HR]，2.62; 95％置信区间[CI]：1.2– 8.8; P = 0.047），以及猝死和相关的致命性心律失常事件（HR，3.3; 95％CI：1.26-8.85; P = 0.016）。结论：在终末期HCM，猝死和致死性心律失常方面，ΔMVO是HCM的独特表型，预后不良。较高的不良预后率要求及早发现MVO和采取适当的治疗干预措施。（Circ J？2013; 77：2366-2374）

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