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肥厚型心肌病的心尖段功能障碍:进展为终末期心力衰竭并伴有心源性猝死。

Apical segmental dysfunction in hypertrophic cardiomyopathy: progression into end-stage heart failure with sudden cardiac death.

作者信息

Ko Y L, Lin J L, Lei M H, Chen J J, Tsou S S, Kuan P, Lien W P

机构信息

Department of Internal Medicine, National Taiwan University Hospital, Taipei, Republic of China.

出版信息

Cardiology. 1995;86(5):436-40. doi: 10.1159/000176917.

DOI:10.1159/000176917
PMID:7585750
Abstract

Apical segmental dysfunction is an unusual finding in hypertrophic cardiomyopathy (HCM). It is characterized by a poor clinical course and a high incidence of malignant ventricular tachyarrhythmias. Long-term prognosis of patients with HCM and apical segmental dysfunction is still unclear. We report 2 cases of apical segmental dysfunction in HCM in whom progressive apical dilatation and congestive heart failure developed. Both patients died suddenly, and intractable ventricular tachyarrhythmias were documented in 1 case during resuscitation. This report provides further evidence that apical segmental dysfunction might predict a subgroup of patients with HCM who are likely to develop end-stage heart failure and are at high risk of sudden cardiac death.

摘要

心尖段功能障碍在肥厚型心肌病(HCM)中是一种不常见的表现。其特点是临床病程不佳且恶性室性心律失常发生率高。HCM合并心尖段功能障碍患者的长期预后仍不明确。我们报告2例HCM合并心尖段功能障碍患者,这2例患者均出现进行性心尖部扩张和充血性心力衰竭。2例患者均猝死,其中1例在复苏过程中记录到顽固性室性心律失常。本报告进一步证明,心尖段功能障碍可能预示着HCM患者中的一个亚组,这些患者可能发展为终末期心力衰竭且心脏性猝死风险高。

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1
Apical segmental dysfunction in hypertrophic cardiomyopathy: progression into end-stage heart failure with sudden cardiac death.肥厚型心肌病的心尖段功能障碍:进展为终末期心力衰竭并伴有心源性猝死。
Cardiology. 1995;86(5):436-40. doi: 10.1159/000176917.
2
[Clinical characteristics and cardiac events in elderly patients with apical hypertrophic cardiomyopathy].
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Clinical Characteristics and Outcomes in Patients With Apical and Nonapical Hypertrophic Cardiomyopathy.心尖肥厚型和非心尖肥厚型肥厚型心肌病患者的临床特征和结局。
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引用本文的文献

1
Malignant familial hypertrophic cardiomyopathy in a family with a 453Arg-->Cys mutation in the beta-myosin heavy chain gene: coexistence of sudden death and end-stage heart failure.一个家族中因β-肌球蛋白重链基因发生453Arg→Cys突变导致的恶性家族性肥厚型心肌病:猝死与终末期心力衰竭并存
Hum Genet. 1996 May;97(5):585-90. doi: 10.1007/BF02281865.