Department of Pediatric Cardiology, Istanbul University, Istanbul Faculty of Medicine, Turkey.
Cardiol J. 2011;18(2):176-84.
Left ventricular non-compaction (LVNC) is a specific cardiomyopathy that occurs following a disruption of endomyocardial morphogenesis. This study presents clinical findings, diagnostic features, treatment and follow-up of pediatric patients diagnosed with LVNC.
Patients with LVNC who were followed from January 2006 to March 2010 were included in this study. Diagnosis was made with the use of characteristic findings of magnetic resonance imaging and echocardiography. Holter electrocardiography and metabolic screening tests were also performed in all patients.
A total of 24 patients were studied (18 male, six female). Patient age at diagnosis was 50 ± 60 months (eight days to 15 years). Average follow-up period was 22 ± 12 months (four months to four years). Findings at diagnosis were as follows: eight (33%) patients had heart failure, five (20%) had rhythm abnormalities, five (20%) had cardiomegaly, two had murmurs, two had cyanosis, and two presented with fatigue. Ten (41%) patients had been followed previously with other diagnoses. In 21 (87.5%) patients, electrocardiographic abnormalities were noted, especially left ventricular hypertrophy and ST-T changes. Patients had an average ejection fraction of 46% (18-73%) and three of them had additional congenital heart disease (patent ductus arteriosus, aortopulmonary window and complex cyanotic heart disease). Scanning for metabolic diseases revealed fatty acid oxidation disorder in one patient, and mitochondrial disease in another. During follow-up, a permanent pacemaker was implanted in a patient with severe bradycardia and ventricular dysfunction, and three patients died.
LVNC can be diagnosed at any age from newborn to adolescent and has a variable clinical course. Closer study of patients with cardiomegaly and heart failure can reduce delays in diagnosis of LVNC.
左心室心肌致密化不全（LVNC）是一种特定的心肌病，发生于心内膜心肌形态发生中断之后。本研究介绍了经诊断患有 LVNC 的儿科患者的临床发现、诊断特征、治疗和随访情况。
本研究纳入了 2006 年 1 月至 2010 年 3 月期间接受随访的 LVNC 患者。使用磁共振成像和超声心动图的特征性发现做出诊断。还对所有患者进行了动态心电图和代谢筛查试验。
共研究了 24 例患者（18 例男性，6 例女性）。诊断时患者年龄为 50±60 个月（8 天至 15 岁）。平均随访时间为 22±12 个月（4 个月至 4 年）。诊断时的发现如下：8 例（33%）患者存在心力衰竭，5 例（20%）患者存在节律异常，5 例（20%）患者存在心脏扩大，2 例有杂音，2 例有发绀，2 例表现为疲劳。10 例（41%）患者之前曾被诊断为其他疾病。21 例（87.5%）患者心电图异常，尤其是左心室肥厚和 ST-T 改变。患者的平均射血分数为 46%（18%至 73%），其中 3 例患者还患有其他先天性心脏病（动脉导管未闭、主肺动脉窗和复杂紫绀型心脏病）。代谢疾病筛查发现 1 例患者存在脂肪酸氧化障碍，另 1 例患者存在线粒体疾病。在随访期间，1 例严重心动过缓和心室功能障碍患者植入了永久性起搏器，3 例患者死亡。
LVNC 可在从新生儿到青少年的任何年龄段诊断，且临床表现差异较大。对存在心脏扩大和心力衰竭的患者进行更密切的研究，可以减少 LVNC 诊断的延迟。
