Kubik Marcin, Dąbrowska-Kugacka Alicja, Lewicka Ewa, Daniłowicz-Szymanowicz Ludmiła, Raczak Grzegorz
Department of Cardiology and Electrotherapy, Medical University of Gdańsk, Poland.
Adv Clin Exp Med. 2018 Mar;27(3):415-422. doi: 10.17219/acem/67457.
Left ventricular noncompaction (LVNC) is a unique inherited cardiomyopathy, characterized by an increased risk of adverse cardiovascular events such as heart failure, arrhythmia or sudden cardiac death. Although in comparison to dilated cardiomyopathy, the number of clinical studies concerning LVNC is still small, it is quickly increasing, which reflects a huge effort of the cardiovascular society to develop data to improve understanding of this cardiomyopathy. However, the predictors of adverse outcomes in LVNC are not well established. The aim of this review is to systematize the available data obtained from the medical literature in order to establish a proper prognosis, so that affected patients can receive the most appropriate treatment. The review considers issues connected with various areas of risk in LVNC, referring to its incidence and prevalence, comorbidity, genetics, morphology, symptoms, thromboembolic events, incidence of arrhythmia, sudden cardiac death, and mortality. Beginning with a genetic approach to the disease, passing through diagnostic tools, and finishing with issues relating to invasive methods of treatment, the article points out the most important and valuable clues for predicting a poor prognosis in LVNC. The review confirms that LVNC is not a disease, but a type of cardiac abnormality laden with a variety of prognostic factors of poor outcomes in terms of life-threatening ventricular arrhythmia and progression of heart failure. Thus, establishing a proper prognosis for individual patients is crucial for implementing the most appropriate treatment, and it should be based on the outcomes of a variety of clinical tests.
左心室心肌致密化不全（LVNC）是一种独特的遗传性心肌病，其特征是发生心力衰竭、心律失常或心源性猝死等不良心血管事件的风险增加。尽管与扩张型心肌病相比，关于LVNC的临床研究数量仍然较少，但正在迅速增加，这反映了心血管学界为收集数据以增进对这种心肌病的了解所做出的巨大努力。然而，LVNC不良结局的预测因素尚未完全明确。本综述的目的是对从医学文献中获得的现有数据进行系统整理，以便建立恰当的预后评估，从而使受影响的患者能够接受最合适的治疗。该综述考虑了与LVNC各个风险领域相关的问题，涉及发病率和患病率、合并症、遗传学、形态学、症状、血栓栓塞事件、心律失常发生率、心源性猝死和死亡率。文章从对该疾病的遗传学研究方法入手，经过诊断工具，最后论述与侵入性治疗方法相关的问题，指出了预测LVNC不良预后的最重要和最有价值的线索。该综述证实，LVNC并非一种单一疾病，而是一种心脏异常类型，就危及生命的室性心律失常和心力衰竭进展而言，它带有多种不良预后因素。因此，为个体患者建立恰当的预后评估对于实施最合适的治疗至关重要，并且应该基于各种临床检查的结果。
