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克利夫兰诊所关于高嗜酸性粒细胞综合征背景下嗜酸性粒细胞性心肌炎的经验:人口统计学、心脏成像及预后

The Cleveland Clinic experience of eosinophilic myocarditis in the setting of hypereosinophilic syndrome: demographics, cardiac imaging, and outcomes.

作者信息

Reeder Matthew, Okushi Yuichiro, Lo Presti Vega Saberio, Prasad Rohan, Grimm Richard A, Griffin Brian P, Xu Bo

机构信息

Department of Medicine, Cleveland Clinic, Cleveland, OH, USA.

Section of Cardiovascular Imaging, Robert and Suzanne Tomsich Department of Cardiovascular Medicine, Sydell and Arnold Miller Family Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH, USA.

出版信息

Cardiovasc Diagn Ther. 2024 Dec 31;14(6):1122-1133. doi: 10.21037/cdt-24-347. Epub 2024 Dec 2.

Abstract

BACKGROUND

Hypereosinophilic syndrome (HES) represents a group of disorders with eosinophil-mediated end-organ damage. Eosinophilic myocarditis (EM) represents cardiac involvement in HES. Data are limited regarding this rare condition. To better understand contemporary clinical characteristics of EM in HES, we reviewed demographics, cardiac imaging, and outcomes of this condition at our center.

METHODS

We performed a cross-sectional study of all patients aged >18 years with diagnosis of EM in HES at our center between September 1986 and January 2023. Relevant clinical data, including clinical presentation, medical history, medication use, comorbidities, imaging findings, and outcomes, were collected and analyzed.

RESULTS

Of 1,664 patients identified with hypereosinophilia (HE), 36 cases of clinically diagnosed HES were identified. Of the 36 patients diagnosed with HES, 11 patients (30.6%) were diagnosed with EM. Of these, six patients underwent endomyocardial biopsy (EMB). The mean age was 57±12 years and 63.6% were female. Asthma was the most common comorbidity (54.5%). Patients with EM had significantly more dyspnea (63.6%), fatigue (54.5%), and neuropathy (36.4%) compared to those without cardiac involvement. Echocardiography was performed in all patients and cardiac magnetic resonance (CMR) imaging was performed in eight patients. Left ventricular (LV) thrombus was detected more frequently by CMR (5/8, 62.5%) compared to echocardiography (3/10, 30%). Subendocardial pattern of late gadolinium enhancement (LGE) was observed in the majority of patients on CMR (6/7, 85.7%). Steroids were utilized in 90.9% of cases, and aspirin in all patients. Compared to HES patients without cardiac involvement, thromboembolic events occurred significantly more frequently (63.6% 24.0%, P=0.02).

CONCLUSIONS

In a 37-year cohort of HES-associated EM, echocardiography was the first-line imaging modality, while CMR was an essential but still under-utilized imaging modality. Patients with EM had significantly more thromboembolic events compared to HES without cardiac involvement.

摘要

背景

高嗜酸性粒细胞综合征(HES)是一组由嗜酸性粒细胞介导的终末器官损伤的疾病。嗜酸性粒细胞性心肌炎(EM)是HES累及心脏的表现。关于这种罕见疾病的数据有限。为了更好地了解HES中EM的当代临床特征,我们回顾了我院该疾病的人口统计学、心脏影像学及预后情况。

方法

我们对1986年9月至2023年1月期间我院诊断为HES合并EM且年龄大于18岁的所有患者进行了一项横断面研究。收集并分析了相关临床数据,包括临床表现、病史、用药情况、合并症、影像学检查结果及预后。

结果

在1664例确诊为高嗜酸性粒细胞增多症(HE)的患者中,有36例临床诊断为HES。在这36例诊断为HES的患者中,11例(30.6%)被诊断为EM。其中,6例患者接受了心内膜心肌活检(EMB)。平均年龄为57±12岁,63.6%为女性。哮喘是最常见的合并症(54.5%)。与无心脏受累的患者相比,EM患者出现呼吸困难(63.6%)、疲劳(54.5%)和神经病变(36.4%)的比例明显更高。所有患者均进行了超声心动图检查,8例患者进行了心脏磁共振(CMR)成像检查。与超声心动图(3/10,30%)相比,CMR更常检测到左心室(LV)血栓(5/8,62.5%)。CMR检查发现大多数患者(6/7,85.7%)存在晚期钆增强(LGE)的心肌内膜下模式。90.9%的病例使用了类固醇,所有患者均使用了阿司匹林。与无心脏受累的HES患者相比,血栓栓塞事件的发生频率明显更高(63.6%对24.0%,P=0.02)。

结论

在一个为期37年的HES相关EM队列中,超声心动图是一线影像学检查方法,而CMR是一种重要但仍未得到充分利用的影像学检查方法。与无心脏受累的HES患者相比,EM患者发生血栓栓塞事件的频率明显更高。

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