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孤立性左心室心肌致密化不全合并心室预激:一例报告

Isolated left ventricular non-compaction cardiomyopathy associated with ventricular preexcitation: a case report.

作者信息

Bozić Ivo, Fabijanić Damir, Carević Vedran, Jurilj Radoslav, Zekanović Drazen, Bonacin Damir

机构信息

Department of Medicine, University Hospital Center Split, Split, Croatia.

出版信息

Coll Antropol. 2009 Dec;33(4):1415-9.

Abstract

Isolated left ventricular non-compaction (ILVNC) is one of the most misclassified cardiomyopathies. It is caused by failure of normal embryonic development of the myocardium from loosely arranged muscle fibers to the mature compacted form of myocardium, but it seems that etiology is not exclusively congenital. Diagnosis of ILVNC is mostly missed because of lack of awareness and knowledge. The recognition of the disease is mandatory, because of its high mortality and morbidity due to the progressive heart failure, thromboembolic events and lethal arrhythmias. We report of a family in which two adult members were found to have ILVNC. A literature review about ILVNC pathogenesis, diagnosing, and treatment was discussed.

摘要

孤立性左心室心肌致密化不全(ILVNC)是最常被误诊的心肌病之一。它是由于心肌在胚胎发育过程中未能从排列松散的肌纤维正常发育为成熟的致密化心肌形式,但病因似乎并非完全是先天性的。由于缺乏认识和了解,ILVNC的诊断大多被漏诊。鉴于其因进行性心力衰竭、血栓栓塞事件和致命性心律失常导致的高死亡率和高发病率,对该疾病的认识至关重要。我们报告了一个家族,其中两名成年成员被发现患有ILVNC。并对有关ILVNC发病机制、诊断和治疗的文献进行了综述。

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