Unsolved Issue in Left Ventricular Noncompaction: Is the Strange Form of Myocardium Congenital or Acquired?
Unsolved Issue in Left Ventricular Noncompaction: Is the Strange Form of Myocardium Congenital or Acquired?
复制标题
左心室致密化不全的未解决问题：奇怪的心肌形态是先天性的还是后天性的？
DOI：
10.1159/000501585
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发表时间：
2019
期刊：
影响因子：
1.9
通讯作者：
Murohara T.
中科院分区：
文献类型：
--
作者：
Okumura T;Murohara T.
左心室致密化不全(LVNC)的形态特征是致密层变薄，网状小梁增多，未致密层有较深的间隙[1]。未致密层也可见于正常胎儿早期，粗大的心肌通过与室腔相通的缝隙和正弦波供血。在发育过程中，心肌逐渐获得致密的结构，最终来自冠状动脉的血液供应成为其主要供应。LVNC被认为是由于胎儿心肌的致密过程受损而导致的海绵状胚胎心肌衰竭[2]。换句话说，异常心肌从出生起就已经存在。然而，近年来，随着成像方法的发展，许多无症状病例被报道，其中LVNC样发现是在医学检查中偶然发现的[3]。根据日本的一项调查，大约40%的LVNC患者被怀疑受到遗传因素的影响，并指出了遗传多样性，如常染色体遗传和线粒体基因突变以及X连锁外[4]。此外，随着时间的推移，一些成人病例的报告证实了这种奇怪结构的突然表达和改善。因此，许多研究人员对LVNC是先天性心肌病还是后天综合征感兴趣。Loria等人[5]报告了一例意外发现的成人LVNC病例，并进行了文献复习。在他们的报告中，一名57岁的女性，有蒽环类药物治疗乳腺癌的病史，表现出明显的心肌小梁。尽管过去无心肌病家族史，超声心动图上也未发现LVNC样表现，但彩色多普勒突然偶然发现LVNC样结构改变和椎间隙深陷影。此外，这些发现也得到心脏磁共振成像(MRI)的证实。看起来，类似LVNC的发现似乎突然发展起来了。
Left ventricular noncompaction (LVNC) is morphologically characterized by a thinning of the compacted layer, excess reticulated trabeculae, and deep gaps in the noncompacted layer [1]. The noncompacted layer is also found in the early normal fetal stage, and the rough myocardium is supplied with blood flow via the gaps and sinusoids in communication with the ventricular cavity. In the developmental process, the myocardium gradually acquires a compacted structure, and eventually the blood supply from the coronary artery becomes its main supply. LVNC is considered to be caused by a failure of the sponge-like embryonic myocardium due to the impaired process of compaction in the fetal myocardium [2]. In other words, abnormal myocardium already exists from birth. However, in recent years, many asymptomatic cases have been reported with the development of imaging modalities, in which LVNC-like findings are identified incidentally in a medical checkup [3]. According to a survey in Japan, around 40% of the patients with LVNC are suspected to be affected by genetic factors, and genetic diversity such as autosomal inheritance and mitochondrial gene mutation in addition to X-linkage has been pointed out [4]. Furthermore, some reports on adult cases have confirmed over time the sudden expression and improvement of this strange structure. Thus, many researchers are interested in whether LVNC is a congenital cardiomyopathy or some acquired syndrome.Loria et al.[5] report an adult case of LVNC identified unexpectedly, along with a literature review. In their report, a 57-year-old female with a history of anthracycline treatment for breast cancer showed prominent myocardial trabeculations. Despite having no family history of cardiomyopathy and not having been noted for LVNC-like findings on echocardiography in the past, LVNC-like structural changes and deep depression with perfusion of the intervertebral space were suddenly and incidentally observed with color Doppler. Furthermore, these findings were also confirmed by cardiac magnetic resonance imaging (MRI). It seems as if the LVNC-like findings suddenly developed.