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[儿童心肌病]

[Cardiomyopathies in childhood].

作者信息

Schmaltz A A, Apitz J

出版信息

Klin Padiatr. 1986 Jan-Feb;198(1):1-7. doi: 10.1055/s-2008-1026842.

Abstract

Primary cardiomyopathies are heart muscle diseases of unknown cause. In hypertrophic cardiomyopathy (HCM) a hypertrophy of unknown aetiology results either in asymmetric septal hypertrophy (mostly with obstruction) or in concentric hypertrophy of the left ventricle (mostly without obstruction). This condition is rare in childhood, partly inherited and sometimes observed already in newborns. Sudden cardiac death is a main complication in the second decade. In non-obstructive HCM endomyocardial biopsy is the tool to differentiate secondary forms. Dilated cardiomyopathy is recognized by aetiologically unclear dilatation of left or right or both ventricles. In childhood this condition has to be differentiated from coronary anomalies, endocardial fibroelastosis, and myocarditis. Also rare secondary forms have to be considered because of possible therapeutic consequences. From the very few histologically proven reports a reliable prognosis for this disease in childhood cannot be deduced.

摘要

原发性心肌病是病因不明的心肌疾病。在肥厚型心肌病(HCM)中,病因不明的心肌肥厚可导致不对称性室间隔肥厚(大多伴有梗阻)或左心室向心性肥厚(大多无梗阻)。这种疾病在儿童期罕见,部分为遗传性,有时在新生儿期即可观察到。心源性猝死是第二个十年的主要并发症。在非梗阻性HCM中,心内膜心肌活检是鉴别继发性类型的手段。扩张型心肌病的特征是左心室或右心室或双心室病因不明的扩张。在儿童期,这种疾病必须与冠状动脉异常、心内膜弹力纤维增生症和心肌炎相鉴别。由于可能的治疗后果,也必须考虑罕见的继发性类型。从极少的组织学证实的报告中无法推断出这种疾病在儿童期的可靠预后。

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