Moreira Fábio Cañellas, Miglioransa Marcelo Haertel, Mautone Marcela Pozo, Müller Karen Reetz, Lucchese Fernando
Echocardiography Laboratory, Hospital São Francisco, Complexo Santa Casa, Porto Alegre, Rio Grande do Sul, Brazil.
Sao Paulo Med J. 2006 Jan 5;124(1):31-5. doi: 10.1590/s1516-31802006000100007. Epub 2006 Apr 3.
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 patients family upon such diagnosis, and today this can be achieved using echocardiographic criteria.
左心室心肌致密化不全是一种形态发生异常，涉及子宫内生活期间心肌纤维网络致密化的丧失。这是一种极其罕见的病症，仅占数据库评估病例的0.05%。在不同性别、许多种族群体和不同年龄段均有报道。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。由于对该综合征相关表现了解不足，迄今为止许多病例被误诊。由于诊断后需要对患者家族进行调查，因此需要尽早进行准确诊断，而如今使用超声心动图标准即可实现这一点。
