Department of Biomedical Sciences, College of Osteopathic Medicine, University of New England, Biddeford, Maine, USA.
Albany Medical Center, Albany Medical College, Albany, New York, USA.
Physiol Rep. 2024 May;12(9):e16029. doi: 10.14814/phy2.16029.
Left ventricular noncompaction cardiomyopathy (LVNC) is a structural heart defect that has been associated with generation of arrhythmias in the population and is a cause of sudden cardiac death with severe systolic dysfunction and fatal arrhythmias. LVNC has gained increasing acknowledgment with increased prevalence. We conducted a systematic review of reported electrocardiogram (ECG) results for pediatric LVNC patients. EMBASE database query was performed, yielding 4531 articles related to LVNC between 1990 and December 2023. Patient age ranged from prenatal to 18 years of age. Qualitative analyses were performed to characterize individual arrhythmias, and summative interpretation of ECG evaluations was gathered for the entire cohort. Systematic review of 57 LVNC cases and ECG presentation revealed many waveform consistencies, including abnormal left ventricular, atrioventricular node, and interventricular septal patterns, and specifically a high incidence of Mobitz type II and Wolff-Parkinson-White waveforms. This review of ECG analysis reinforces the clinical and etiologic significance of pediatric LVNC. While LVNC in pediatric populations may not always present as acute clinical cases, further investigation into the electrophysiology of the disease supports the need for further evaluation and risk stratification for patients with suspected LVNC and/or ventricular arrhythmia.
左心室心肌致密化不全（LVNC）是一种结构性心脏病，与人群中的心律失常发生有关，是严重收缩功能障碍和致命性心律失常导致心源性猝死的原因。随着患病率的增加，LVNC 得到了越来越多的认可。我们对已发表的 LVNC 儿科患者心电图（ECG）结果进行了系统评价。对 EMBASE 数据库进行了查询，获得了 1990 年至 2023 年 12 月期间与 LVNC 相关的 4531 篇文章。患者年龄从产前到 18 岁不等。对心律失常的特征进行了定性分析，并对整个队列的 ECG 评估进行了综合解释。对 57 例 LVNC 病例和 ECG 表现进行了系统评价，发现了许多波形一致性，包括异常左心室、房室结和室间隔模式，特别是高度发生莫氏 II 型和沃夫-帕金森-怀特波形。这项 ECG 分析的综述加强了儿科 LVNC 的临床和病因学意义。虽然儿科人群中的 LVNC 不一定总是表现为急性临床病例，但对疾病电生理学的进一步研究支持对疑似 LVNC 和/或室性心律失常患者进行进一步评估和风险分层的必要性。
