Compton Gregory, Nield Lynne, Dragulescu Andreea, Benson Lee, Grosse-Wortmann Lars
Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, Canada.
Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, Canada.
Int J Pediatr. 2016;2016:1980636. doi: 10.1155/2016/1980636. Epub 2016 Nov 15.
. Hypertrophic cardiomyopathy (HCM) is burdened with morbidity and mortality including tachyarrhythmias and sudden cardiac death. These complications are attributed in part to the formation of proarrhythmic scars in the myocardium. The presence of extensive LGE is a risk factor for adverse outcomes in HCM. Late gadolinium enhancement (LGE) cardiac magnetic resonance imaging (cMRI) is the standard for the noninvasive evaluation of myocardial scars. However, echocardiography represents an attractive screening tool for myocardial scarring. The aim of this study was to compare the suitability of echocardiography to detect myocardial scars to the standard of cMRI-LGE. . The cMRI studies and echocardiograms from 56 consecutive children with HCM were independently evaluated for the presence of cMRI-LGE and echocardiographic evidence of scarring by expert readers. . Echocardiography had a high sensitivity (93%) and negative predictive value (94%) in comparison to LGE. The false positive rate was high, leading to a low specificity (37%) and a low positive predictive value (35%). . Given the poor specificity and positive predictive value, echocardiography is not a suitable screening test for the presence of myocardial scarring in children with HCM. However, children without echocardiographic evidence of myocardial scarring may not need to undergo cardiac magnetic resonance imaging to "rule in" LGE.
肥厚型心肌病（HCM）存在包括快速性心律失常和心源性猝死在内的发病和死亡负担。这些并发症部分归因于心肌中促心律失常瘢痕的形成。广泛的延迟强化（LGE）的存在是HCM不良结局的一个危险因素。钆延迟增强（LGE）心脏磁共振成像（cMRI）是心肌瘢痕无创评估的标准。然而，超声心动图是一种有吸引力的心肌瘢痕筛查工具。本研究的目的是比较超声心动图检测心肌瘢痕的适用性与cMRI-LGE标准。对连续56例HCM患儿的cMRI研究和超声心动图进行独立评估，由专业人员判断是否存在cMRI-LGE及瘢痕形成的超声心动图证据。与LGE相比，超声心动图具有较高的敏感性（93%）和阴性预测值（94%）。假阳性率较高，导致特异性较低（37%）和阳性预测值较低（35%）。鉴于特异性和阳性预测值较差，超声心动图不是HCM患儿心肌瘢痕存在与否的合适筛查试验。然而，没有超声心动图心肌瘢痕证据的儿童可能无需进行心脏磁共振成像来“确诊”LGE。
