Department of Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom (J.S., G.D.S., S.K., A.F., C.P., V.B., M.J., J.P., D.S., I.V., S.P., P.D.).
National Heart and Lung Institute, Imperial College, London, United Kingdom (G.D.S., S.P., A.F., P.D.).
Circ Cardiovasc Imaging. 2019 Apr;12(4):e007805. doi: 10.1161/CIRCIMAGING.118.007805.
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.
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.
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).
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.
左心室心肌致密化不全（LVNC）与儿童期不良临床预后相关。目前，标准诊断标准仍存在争议，尤其是在年轻患者中。最近的成人研究表明，LVNC 患者的左心室（LV）扭转异常，但迄今为止尚未在儿科患者中进行研究。我们的目的是使用二维斑点追踪超声心动图评估 LV 心脏力学、LV 扭转以及刚体旋转的发生率，这些在 LVNC 和 LV 心肌肥厚的年轻患者中进行。
评估了 47 例疑似 LVNC 的儿童患者。所有患者均在 1.5T 磁共振（T）上接受二维斑点追踪超声心动图和心血管磁共振成像检查。23 例患者符合心血管磁共振成像诊断 LVNC 的标准（LVNC 组），而其余 24 例不符合标准，被纳入 LV 心肌肥厚组。47 例年龄和性别匹配的健康志愿者作为对照组。
与对照组和 LV 心肌肥厚组相比，LVNC 患者的平均 LV 扭转明显降低。在 13 例（56%）LVNC 患儿和 1 例（4%）LV 心肌肥厚且有 LVNC 家族史的患儿中发现了刚体旋转。多变量分析表明，LV 扭转是 LVNC 的独立预测因子（P=0.006；系数=0.462）。ROC 曲线显示，LV 扭转对区分 LVNC 患者具有最佳预测价值（截断值<5.8°；敏感性为 82%，特异性为 92%，曲线下面积为 0.914）。
LV 扭转对诊断年轻患者的 LVNC 具有良好的预测价值。我们的研究结果强烈支持在评估疑似 LVNC 的年轻患者时常规使用二维斑点追踪超声心动图。
