Clinical Characteristics and Natural History of Hypertrophic Cardiomyopathy With Midventricular Obstruction
Clinical Characteristics and Natural History of Hypertrophic Cardiomyopathy With Midventricular Obstruction
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DOI：
10.1253/circj.cj-12-1561
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发表时间：
2013-09-01
影响因子：
3.3
通讯作者：
Styliadis, Ioannis H.
中科院分区：
文献类型：
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作者：
Efthimiadis, Georgios K.;Pagourelias, Efstathios D.;Styliadis, Ioannis H.
背景：肥厚型心肌病 (HCM) 和心室中段梗阻 (MVO) 患者的患病率、临床特征和自然史尚未得到充分研究。方法和结果：对 423 名患者（平均年龄 49.3 +/- 17.2 岁；66.2% 男性）组成的单中心队列进行了彻底随访，中位随访时间为 84 个月（7 年；范围， 6-480 个月）。 MVO 的特征是超声心动图显示心室中肌并置，同时中腔梯度 >= 30 mmHg，在 34 名患者 (8%) 中被发现。与 HCM 队列中的其他患者相比，MVO 患者在初次评估期间往往症状更严重（>90% 的 NYHA 分级 >= II）。超过四分之一的 MVO 患者 (26.5%) 发现心尖动脉瘤形成，这是该群体的一个特征。在多变量 Cox 回归风险分析中，MVO 的存在强烈预测进展为终末期（精疲力竭）HCM 和相关心力衰竭 (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死性心律失常事件（HR，3.3；95% CI： 1.26-8.85； P=0.016）。结论：MVO 是 HCM 的一种独特表型，与终末期 HCM、猝死和致命性心律失常事件的不良预后相关。高不良结果发生率需要及早识别 MVO 并采取适当的治疗干预措施。
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 >= 30 mmHg, 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.