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首页> 外文期刊>Sao Paulo Medical Journal >Noncompaction of the left ventricle: a new cardiomyopathy is presented to the clinician
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Noncompaction of the left ventricle: a new cardiomyopathy is presented to the clinician

机译:左心室不紧密:向临床医师提出了一种新的心肌病

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Noncompaction of the left ventricular myocardium is a morphogenetic abnormality involving loss of compaction of the myocardial fiber meshwork during intrauterine life. It is an extremely rare condition, accounting for only 0.05% of the cases evaluated in databanks. It has been described in both genders, in many ethnic groups and at different ages. Recently published studies of case series have shown a high mortality rate among these patients during follow-up of up to 48 months. Many cases have so far been misdiagnosed due to poor knowledge of the findings relating to this syndrome. There needs to be an attempt at early and accurate diagnosis, because of the need to investigate the patient’s family upon such diagnosis, and today this can be achieved using echocardiographic criteria.
机译:左心室心肌的不紧实是一种形态发生异常,涉及子宫内生命期间心肌纤维网的紧密性丧失。这是一种极为罕见的情况,仅占数据库评估案例的0.05%。在许多种族和不同年龄的男女中都有描述。最近发表的病例系列研究表明,在长达48个月的随访期间,这些患者的死亡率很高。迄今为止,由于对与该综合征有关的发现的了解不足,许多病例被误诊。由于需要在诊断时对患者的家人进行调查,因此需要尽早进行准确的诊断,而今天可以使用超声心动图标准来实现。

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