BACKGROUND Left ventricular noncompaction cardiomyopathy (LVNC) is associated with poor clinical outcome in childhood. Standard diagnostic criteria are still controversial, especially in young patients. Recent studies in adults demonstrated that left ventricular (LV) twist is abnormal in LVNC, but it has not been investigated in pediatric patients to date. Our aim was to assess LV cardiac mechanics, LV twist, and the prevalence of rigid body rotation, using 2-dimensional speckle tracking echocardiography, in young patients with LVNC and LV hypertrabeculation. METHODS Forty-seven children (age range: 0-18 years) were assessed for suspected LVNC. All patients underwent 2-dimensional speckle tracking echocardiography and cardiovascular magnetic resonance imaging at 1.5 Tesla (T). Twenty-three patients fulfilled the cardiovascular magnetic resonance imaging diagnostic criteria for LVNC (LVNC group), while the remaining 24 did not and were included in the LV hypertrabeculation group. Forty-seven age- and sex-matched healthy volunteers were used as controls. RESULTS The average LV twist was significantly reduced in LVNC compared with control and LV hypertrabeculation. Rigid body rotation was recognized in 13 (56%) children with LVNC and in 1 (4%) child with LV hypertrabeculation and a strong family history for LVNC. Multivariable analysis demonstrated that LV twist is an independent predictor of LVNC ( P=0.006; coefficient=0.462). The receiver operating characteristics curve showed that LV twist had optimal predictive value to discriminate patients with LVNC (cutoff value <5.8°; sensitivity, 82%; specificity, 92%; area under the curve=0.914). CONCLUSIONS LV twist has good predictive value in diagnosing LVNC in young patients. Our findings strongly support the routine use of 2-dimensional speckle tracking echocardiography in the evaluation of young patients with suspected LVNC.

中文翻译：

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左心室扭转力学，以识别童年时期的左心室非紧致。

背景技术左心室非致密性心肌病（LVNC）与儿童期临床预后不良有关。标准诊断标准仍然存在争议，尤其是在年轻患者中。成年人的最新研究表明左心室扭转在LVNC中是异常的，但迄今为止尚未在儿科患者中进行过研究。我们的目的是使用二维散斑跟踪超声心动图技术评估年轻的LVNC和LV超小梁患者的LV心脏力学，LV扭转和刚体旋转的患病率。方法评估了47名儿童（年龄范围：0-18岁）的疑似LVNC。所有患者均在1.5特斯拉（T）处进行了二维斑点跟踪超声心动图和心血管磁共振成像。23例患者符合LVNC的心血管磁共振成像诊断标准（LVN​​C组），而其余24例未达到标准，被纳入LV超小梁形成组。47名年龄和性别匹配的健康志愿者被用作对照。结果与对照组和左小梁超切术相比，LVNC的平均左左扭转明显降低。刚体旋转在13例（56％）LVNC儿童和1例（4％）LV超小梁并有LVNC家族史的儿童中被确认。多变量分析表明，左心室扭转是左心室狭窄的独立预测因子（P = 0.006；系数= 0.462）。接受者的操作特征曲线表明，左心室扭转具有最佳的预测价值，可区分左室神经节患者（临界值<5.8°；敏感度为82％；特异性为92％；特异度为92％。曲线下面积= 0.914）。结论LV扭转在年轻患者LVNC的诊断中具有良好的预测价值。我们的发现强烈支持二维散斑跟踪超声心动图在评估疑似LVNC的年轻患者中的常规使用。