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文莱达鲁萨兰国肥厚型心肌病患者的临床特征及心脏磁共振成像结果

Clinical Characteristics and Cardiac Magnetic Resonance Findings in Patients With Hypertrophic Cardiomyopathy in Brunei Darussalam.

作者信息

Tong Kah Cheong, Bilgrami Nazar Luqman

机构信息

Acute Medical Unit, Stepping Hill Hospital, Manchester, GBR.

Cardiology, Heart of Melbourne, Melbourne, AUS.

出版信息

Cureus. 2025 Jun 18;17(6):e86303. doi: 10.7759/cureus.86303. eCollection 2025 Jun.

Abstract

Background Hypertrophic cardiomyopathy (HCM) is a genetic heart disease that poses a risk for sudden cardiac death. It has not been studied systematically in Southeast Asian countries. The purpose of this study was to investigate the characteristics and patterns of fibrosis in HCM, as well as to determine the risk of sudden cardiac death in relation to age, sex, and myocardial fibrosis in Brunei Darussalam. Methods All patients diagnosed with HCM from 2011 to 2017 were studied in Brunei Darussalam. Data was acquired from clinical notes of these patients and studied for the demographic profile of the disease, clinical manifestations, and risks for sudden cardiac death. Results A total of 39 patients with HCM were identified, with a mean age at diagnosis of 47.46±12.27 years. Roughly half of HCM patients (51.3%) were found to be asymptomatic. A total of 30 patients underwent cardiac magnetic resonance (CMR). Myocardial fibrosis was found in 53.8%. This was more prevalent and significant in apical HCM compared to asymmetrical septal HCM, in 10 (83.3%) and nine (60%) patients (p=0.032), respectively. Apical and asymmetrical septal HCM were the most common types of HCM in the population, 15 (38.5%) and 16 (41%), respectively. Asymmetrical septal HCM presented with more murmurs than apical HCM patients, nine (56.3%) and one (6.7%) patients (p=0.001), respectively. Asymmetrical septal HCM has a higher median risk score compared to apical HCM, with medians of 2.05 (IRQ: 1.78) and 1.44 (IRQ: 0.85; p=0.025), respectively. There was no statistical difference in terms of clinical and imaging characteristics between male and female HCM patients. Conclusion Demographics and clinical presentation of the disease are comparable to those of other studies. However, the risk for sudden cardiac death was higher in asymmetrical septal HCM compared to apical HCM. We also found that myocardial fibrosis on CMR is more significant in apical HCM.

摘要

背景

肥厚型心肌病(HCM)是一种遗传性心脏病,存在心脏性猝死风险。东南亚国家尚未对其进行系统研究。本研究旨在调查文莱达鲁萨兰国HCM患者的纤维化特征和模式,以及确定与年龄、性别和心肌纤维化相关的心脏性猝死风险。方法:对文莱达鲁萨兰国2011年至2017年期间诊断为HCM的所有患者进行研究。从这些患者的临床记录中获取数据,并研究疾病的人口统计学特征、临床表现和心脏性猝死风险。结果:共识别出39例HCM患者,诊断时的平均年龄为47.46±12.27岁。约一半(51.3%)的HCM患者无症状。共有30例患者接受了心脏磁共振成像(CMR)检查。发现53.8%的患者存在心肌纤维化。与非对称性室间隔肥厚型心肌病相比,心尖肥厚型心肌病的心肌纤维化更为普遍且显著,分别为10例(83.3%)和9例(60%)患者(p = 0.032)。心尖肥厚型心肌病和非对称性室间隔肥厚型心肌病是该人群中最常见的HCM类型,分别为15例(38.5%)和16例(41%)。非对称性室间隔肥厚型心肌病患者出现杂音的比例高于心尖肥厚型心肌病患者,分别为9例(56.3%)和1例(6.7%)患者(p = 0.001)。非对称性室间隔肥厚型心肌病的中位风险评分高于心尖肥厚型心肌病,分别为2.05(四分位间距:1.78)和1.44(四分位间距:0.85;p = 0.025)。HCM男性和女性患者在临床和影像学特征方面无统计学差异。结论:该疾病的人口统计学特征和临床表现与其他研究相当。然而,与心尖肥厚型心肌病相比,非对称性室间隔肥厚型心肌病的心脏性猝死风险更高。我们还发现,CMR显示的心尖肥厚型心肌病的心肌纤维化更为显著。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ff18/12274072/e2d634e8620c/cureus-0017-00000086303-i01.jpg

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