肥厚型心肌病患者的临床特征和长期预后
Clinical characteristics and long-term outcomes in patients with apical hypertrophic cardiomyopathy.
作者信息
Guo Meng, Liu Chuanfen, Ye Jingjing, Liu Jian
机构信息
Department of Cardiology, Peking University People's Hospital, Beijing, China.
Beijing Key Laboratory of Early Prediction and Intervention of Acute Myocardial Infarction, Peking University People's Hospital, Beijing, China.
出版信息
ESC Heart Fail. 2025 Aug;12(4):2887-2897. doi: 10.1002/ehf2.15298. Epub 2025 Apr 13.
AIMS
As a special type of hypertrophic cardiomyopathy (HCM), apical HCM (ApHCM) has different clinical characteristics while its nature history and prognosis are not well recognized. We aimed to describe the characteristics and outcomes of ApHCM and identify predictors of adverse outcomes.
METHODS
In this single-centre retrospective study, we included 479 patients with HCM and divided them into ApHCM and non-ApHCM groups. Clinical, electrocardiographic, echocardiographic and survival data were compared between the groups. The primary outcome was major adverse cardiac events in hospital and during follow-up. A two-sided P-value < 0.05 was considered statistically significant.
RESULTS
A total of 109 ApHCM patients and 370 non-ApHCM patients were analysed and 379 patients completed the follow-up among them. The age of enrolled patients was 61.0 (50.0-69.0) years, and 289 (60.3%) were male. Compared with non-ApHCM patients, ApHCM patients were older at diagnosis [55.0 (45.0-64.0) vs. 50.0 (40.0-61.0) years, P = 0.006] and had less positive family history for HCM [3 (2.8%) vs. 34 (9.2%), P = 0.027], more electrocardiographic abnormalities [101 (92.7%) vs. 287 (77.6%), P < 0.001], lower brain natriuretic peptide level [135.5 (60.8-272.8) vs. 422.5 (182.8-888.2) pg/mL, P < 0.001] and better left ventricular ejection fraction (LVEF) [69.00 (64.00-73.87) vs. 67.00 (60.24-73.45) %, P = 0.048] at baseline. During a median follow-up of 5.59 (2.33-10.30) years, the primary outcome occurred less frequently in ApHCM patients [11.4% vs 27.2%; hazard ratio (HR) 0.360 (95% confidence interval, CI: 0.187-0.696), P = 0.002; log rank P = 0.001]. Specifically, ApHCM was characterized by fewer all-cause death (HR 0.545, 95% CI: 0.305-0.975; P = 0.041) and fatal ventricular arrhythmia or appropriate implantable cardioverter defibrillator intervention (HR 0.099, 95% CI: 0.013-0.724; P = 0.023). LVEF (HR 0.861, 95% CI: 0.763-0.971; P = 0.015) and age (HR 1.247, 95% CI: 1.095-1.419; P = 0.001) were identified as independent predictors of the composite outcome in ApHCM.
CONCLUSIONS
Patients with ApHCM may have better prognosis. LVEF and age were independent predictors of long-term outcomes in ApHCM.
目的
作为肥厚型心肌病（HCM）的一种特殊类型，心尖部肥厚型心肌病（ApHCM）具有不同的临床特征，但其自然病史和预后尚未得到充分认识。我们旨在描述ApHCM的特征和预后，并确定不良预后的预测因素。
方法
在这项单中心回顾性研究中，我们纳入了479例HCM患者，并将他们分为ApHCM组和非ApHCM组。比较两组的临床、心电图、超声心动图和生存数据。主要结局是住院期间和随访期间的主要不良心脏事件。双侧P值<0.05被认为具有统计学意义。
结果
共分析了109例ApHCM患者和370例非ApHCM患者，其中379例患者完成了随访。入选患者的年龄为61.0（50.0 - 69.0）岁，男性289例（60.3%）。与非ApHCM患者相比，ApHCM患者诊断时年龄更大[55.0（45.0 - 64.0）岁 vs. 50.0（40.0 - 61.0）岁，P = 0.006]，HCM家族史阳性率更低[3例（2.8%） vs. 34例（9.2%），P = 0.027]，心电图异常更多[101例（92.7%） vs. 287例（77.6%），P < 0.001]，脑钠肽水平更低[135.5（60.8 - 272.8） vs. 422.5（182.8 - 888.2）pg/mL，P < 0.001]，基线时左心室射血分数（LVEF）更好[69.00（64.00 - 73.87）% vs. 67.00（60.24 - 73.45）%，P = 0.048]。在中位随访5.59（2.33 - 10.30）年期间，ApHCM患者主要结局的发生频率较低[11.4% vs 27.2%；风险比（HR）0.360（95%置信区间，CI：0.187 - 0.696），P = 0.002；对数秩检验P = 0.001]。具体而言，ApHCM的特征是全因死亡较少（HR 0.545，95% CI：0.305 - 0.975；P = 0.041）以及致命性室性心律失常或合适的植入式心脏复律除颤器干预较少（HR 0.099，95% CI：0.013 - 0.724；P = 0.023）。LVEF（HR 0.861，95% CI：0.763 - 0.971；P = 0.015）和年龄（HR 1.247，95% CI：1.095 - 1.419；P = 0.001）被确定为ApHCM复合结局的独立预测因素。
结论
ApHCM患者可能具有更好的预后。LVEF和年龄是ApHCM长期结局的独立预测因素。
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