Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
J Pediatr. 2012 Jun;160(6):1039-43.e1. doi: 10.1016/j.jpeds.2011.11.038. Epub 2011 Dec 21.
To evaluate the utility of transthoracic contrast echocardiography (TTCE) as a screening tool for pulmonary arteriovenous malformations (PAVMs) in children with hereditary hemorrhagic telangiectasia (HHT).
This was a single-center study of children who underwent baseline screening for PAVMs using both TTCE and chest computed tomography (CT) for evaluation of HHT. The CT and TTCE results were prospectively reviewed independently by 2 radiologists and 2 cardiologists blinded to the study results.
Both intraobserver and interobserver agreement for interpreting TTCE results were excellent (κ = 0.97 and 0.92, respectively) and higher than the interobserver agreement for CT interpretation (κ = 0.75). The sensitivity and specificity of TTCE to predict PAVMs were 1 and 0.82, respectively, and the positive predictive and negative predictive values were 0.39 and 1, respectively.
TTCE is a sensitive test for PAVMs in children with suspected HHT and can be a useful initial screening tool in pediatric HHT.
评估经胸对比超声心动图（TTCE）作为遗传性出血性毛细血管扩张症（HHT）患儿肺动静脉畸形（PAVMs）筛查工具的效用。
这是一项单中心研究，对接受 HHT 基线筛查的患儿同时使用 TTCE 和胸部计算机断层扫描（CT）评估 PAVMs。CT 和 TTCE 结果由 2 名放射科医生和 2 名心脏病专家独立进行前瞻性审查，他们对研究结果不知情。
TTCE 结果的观察者内和观察者间一致性均极佳（κ 值分别为 0.97 和 0.92），高于 CT 解释的观察者间一致性（κ = 0.75）。TTCE 预测 PAVMs 的灵敏度和特异性分别为 1 和 0.82，阳性预测值和阴性预测值分别为 0.39 和 1。
TTCE 是疑似 HHT 患儿 PAVMs 的敏感检测方法，可作为儿科 HHT 的有用初始筛查工具。
