Risk of Death in Long-Term Follow-Up of Patients With Apical Hypertrophic Cardiomyopathy

Risk of Death in Long-Term Follow-Up of Patients With Apical Hypertrophic Cardiomyopathy
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DOI:
10.1016/j.amjcard.2013.02.040
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发表时间:
2013-06-15
影响因子:
2.8
通讯作者:
Ommen, Steve R.
Ommen, Steve R.
中科院分区:
医学3区
文献类型:
--
作者:
Klarich, Kyle W.;Jost, Christine H. Attenhofer;Ommen, Steve R.

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顶端肥厚性心肌病(HC)一直被认为是一种“良性”形式的HC,但关于长期预后的数据有限。我们比较了顶端HC患者与非HC患者,年龄和性别匹配的明尼苏达州白人人群,以确定结果和预后因素。1976年至2006年间,我们诊所共收治了193例根尖HC患者(62%为男性)。回顾了他们最近的超声心动图检查。首次发病时的平均+/- SD年龄为58 +/- 17岁。43例(22%)患者报告有HC或心源性猝死家族史;22例(11%)有冠状动脉疾病。29例患者出现根尖袋,包括6例根尖动脉瘤和23例根尖扩张伴运动不足。中位随访(187例[97%])为78个月(范围1-350)。55名患者(29%;33名女性)因各种原因死亡,平均年龄为72岁(范围20-92岁)。在随访期间,女性发生心力衰竭(p = 0.001)、房颤(p = 0.009)或死亡(p < 0.001)的人数多于男性。生存率低于预期(p = 0.001);观察到的20年生存率和预期的20年生存率分别为47%和60%。随访期间,11例(6%)患者出现SCD、复苏性心脏骤停和/或除颤器放电。生存率降低的多因素预测因子为基线时较高的年龄(p < 0.001)、女性(p < 0.001)和基线时房颤(p = 0.06)。总之,这一人群的根尖HC与死亡率增加有关,尤其是女性。由于根尖HC不像以前怀疑的那样是良性的,因此需要仔细的纵向护理。(C) 2013爱思唯尔公司版权所有。
Apical hypertrophic cardiomyopathy (HC) has been considered a "benign" form of HC, with limited data on long-term outcome. We compared apical HC patients with a non-HC, age- and gender-matched Minnesota white population to identify outcomes and prognostic factors. Between 1976 and 2006, 193 patients (62% men) with apical HC were seen at our clinic. Their most recent echocardiographic examinations were reviewed. Mean +/- SD age at first presentation was 58 +/- 17 years. A family history of HC or sudden cardiac death (SCD) was reported by 43 patients (22%); coronary artery disease was known in 22 (11%). An apical pouch was present in 29 patients, including an apical aneurysm in 6 and apical dilatation with hypokinesis in 23. Median follow-up (187 patients [97%]) was 78 months (range, 1-350). Death from all causes occurred in 55 patients (29%; 33 women) at a mean age of 72 years (range, 20-92). During follow-up, more women had heart failure (p = 0.001), atrial fibrillation (p = 0.009), or died (p < 0.001) than men. Survival was worse than expected (p = 0.001); the observed versus expected 20-year survival was 47% versus 60%. SCD, resuscitated cardiac arrest, and/or defibrillator discharge was observed in 11 patients (6%) during follow-up. Multivariate predictors of decreased survival were higher age at baseline (p < 0.001), female gender (p < 0.001), and atrial fibrillation at baseline (p = 0.06). In conclusion, apical HC in this population was associated with increased mortality, especially in women. Because apical HC is less benign than previously suspected, careful longitudinal care is warranted. (C) 2013 Elsevier Inc. All rights reserved.