Weisz Sara H, Limongelli Giuseppe, Pacileo Giuseppe, Calabro Paolo, Russo Maria G, Calabro Raffaele, Vatta Matteo
Department of Cardiology, Second University of Naples, Monaldi Hospital, Naples, Italy.
Congenit Heart Dis. 2010 Sep-Oct;5(5):384-97. doi: 10.1111/j.1747-0803.2010.00446.x.
Left ventricular non compaction (LVNC) is a myocardial disease characterized by a hypertrabeculated myocardium. The hypertrabeculations in the left ventricular wall define deep recesses communicating with the left ventricular chamber where blood penetrates with increased risk of blood clots in the meshwork of the prominent trabeculations. The left ventricular apex and the free wall are particularly affected. During in utero ventriculogenesis, myocardial blood supply is initially linked to the presence of sinusoids, in which blood penetrates and diffuses nutriments and oxygen to myocardial cells. Progressively, with the development of the heart and the increase of cells demand of blood, coronary arteries system develops. This step is associated with myocardial modification that leads to compaction of hypertrabeculated myocardial net. Probably, the premature interruption of this process leads to ventricular noncompaction. Many studies have been conducted in adults with hypertrabeculated myocardium. To date, data regarding childhood LVNC are sparse. The aim of this review is to summarize the clinical and preclinical knowledge about LVNC in children.
左心室心肌致密化不全（LVNC）是一种以心肌小梁增多为特征的心肌病。左心室壁的心肌小梁形成与左心室腔相通的深陷凹，血液渗入其中，在明显的小梁网中形成血栓的风险增加。左心室心尖和游离壁尤其受累。在子宫内心室形成过程中，心肌血液供应最初与血窦的存在有关，血液通过血窦渗透并将营养物质和氧气扩散到心肌细胞。随着心脏的发育和细胞对血液需求的增加，冠状动脉系统逐渐发育。这一步骤与心肌改变有关，导致心肌小梁网致密化。可能是这个过程的过早中断导致了心室致密化不全。许多研究已经在患有心肌小梁增多的成年人中进行。迄今为止，关于儿童LVNC的数据很少。这篇综述的目的是总结关于儿童LVNC的临床和临床前知识。
