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孤立性左心室异常肌小梁形成是神经肌肉疾病的一种心脏表现。

Isolated left ventricular abnormal trabeculation is a cardiac manifestation of neuromuscular disorders.

作者信息

Stöllberger C, Finsterer J, Blazek G

机构信息

Krankenanstalt Rudolfstiftung, Wien, Osterreich.

出版信息

Cardiology. 2000;94(1):72-6. doi: 10.1159/000007050.

Abstract

OBJECTIVES

Isolated left ventricular abnormal trabeculation (ILVAT) is defined as >3 coarse trabeculations of the left ventricular wall, apically to the papillary muscles, in hearts without congenital malformations. The aims of the study were to assess by echocardiography the prevalence of ILVAT, look for cardiac findings in ILVAT and find out if ILVAT is associated with neuromuscular disorders.

DESIGN AND RESULTS

In the course of 3 years, ILVAT was found in 15/17, 648 patients (0.08%). Twelve patients had heart failure, all had ECG abnormalities. All patients had neuromuscular disorders. A definite diagnosis could be established in 10 patients (metabolic myopathy, n = 9; Becker's muscular dystrophy, n = 1). The remaining 5 patients refused further neurological investigations.

CONCLUSIONS

ILVAT is a rare disorder associated with ECG abnormalities, heart failure and neuromuscular disorders, which is a reason why both cardiologists and neurologists should be involved.

摘要

目的

孤立性左心室异常肌小梁化(ILVAT)定义为在无先天性畸形的心脏中,左心室壁在乳头肌尖端上方有大于3条粗大肌小梁。本研究的目的是通过超声心动图评估ILVAT的患病率,寻找ILVAT中的心脏表现,并确定ILVAT是否与神经肌肉疾病相关。

设计与结果

在3年的时间里,17648例患者中有15例(0.08%)发现有ILVAT。12例患者有心力衰竭,所有患者均有心电图异常。所有患者都有神经肌肉疾病。10例患者可明确诊断(代谢性肌病,9例;贝克肌营养不良症,1例)。其余5例患者拒绝进一步的神经学检查。

结论

ILVAT是一种罕见的疾病,与心电图异常、心力衰竭和神经肌肉疾病相关,这就是心脏病学家和神经学家都应参与其中的原因。

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