Chungsomprasong Paweena, Hamilton Robert, Luining Wietske, Fatah Meena, Yoo Shi-Joon, Grosse-Wortmann Lars
Department of Paediatrics, Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Department of Paediatrics, Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Am J Cardiol. 2017 Mar 1;119(5):778-784. doi: 10.1016/j.amjcard.2016.11.020. Epub 2016 Dec 2.
The aim of this study was to determine if left ventricular (LV) contractility is reduced in children with arrhythmogenic right ventricular cardiomyopathy (ARVC). For this retrospective study, children and adolescents undergoing a workup for ARVC were characterized according to the revised Task Force Criteria (rTFC). LV strain, rotation, and torsion were measured by feature-tracking cardiovascular magnetic resonance imaging (CMR). Of 142 pediatric patients, 41% had no, 23% possible, 20% borderline, and 16% definite ARVC. LV ejection fraction (EF) did not differ between rTFC categories. Patients in higher rTFC categories had lower right ventricular (RV) EF z-scores (Z-), higher Z-RV end-diastolic volumes (EDVs) and larger Z-LVEDVs (p <0.001, p = 0.002 and 0.013, respectively). LV global circumferential strain was lower in higher rTFC categories (p = 0.018). Z-LVEDV correlated with Z-RVEDV, and Z-LVEF correlated with Z-RVEF (r = 0.69 and r = 0.55, both p <0.001). Z-LVEF and Z-RVEF correlated with LV global circumferential strain (r = 0.48 and r = 0.46, both p <0.001). Forty-eight patients (34%) underwent follow-up CMR investigations after a mean of 3.2 ± 1.9 (0.4 to 8.4) years. A decrease of Z-LVEF over time correlated with that of Z-RVEF (r = 0.35), and Z-LVEDV increase correlated with Z-RVEDV increase (r = 0.57). In conclusion, LV myocardial dysfunction is present in young patients with suspected ARVC. Progressive LV dysfunction assessed by conventional CMR and feature-tracking and enlargement over time parallel adverse remodeling of the RV.
本研究的目的是确定致心律失常性右室心肌病（ARVC）患儿的左心室（LV）收缩力是否降低。在这项回顾性研究中，根据修订的工作组标准（rTFC）对接受ARVC检查的儿童和青少年进行了特征描述。通过特征跟踪心血管磁共振成像（CMR）测量左室应变、旋转和扭转。在142例儿科患者中，41%无ARVC，23%可能患有ARVC，20%为临界状态，16%确诊为ARVC。rTFC各分类之间的左室射血分数（EF）无差异。rTFC分类较高的患者右心室（RV）EF z评分（Z-）较低，Z-RV舒张末期容积（EDV）较高，Z-LVEDV较大（分别为p<0.001、p = 0.002和0.013）。rTFC分类较高的患者左室整体圆周应变较低（p = 0.018）。Z-LVEDV与Z-RVEDV相关，Z-LVEF与Z-RVEF相关（r = 0.69和r = 0.55，均p<0.001）。Z-LVEF和Z-RVEF与左室整体圆周应变相关（r = 0.48和r = 0.46，均p<0.001）。48例患者（34%）在平均3.2±1.9（0.4至8.4）年后接受了随访CMR检查。Z-LVEF随时间的下降与Z-RVEF的下降相关（r = 0.35），Z-LVEDV的增加与Z-RVEDV的增加相关（r = 0.57）。总之，疑似ARVC的年轻患者存在左室心肌功能障碍。通过传统CMR和特征跟踪评估的进行性左室功能障碍以及随时间的扩大与右室的不良重塑平行。
