• Suhail Al-Saleh, Andreea Dragulescu, David Manson, Fraser Golding, Jeffrey Traubici, Meir Mei-Zahav, Ian B Maclusky, Marie E Faughnan, Susan Carpenter, and Felix Ratjen.
• Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
• J. Pediatr. 2012 Jun 1; 160 (6): 1039-43.e1.

ObjectiveTo evaluate the utility of transthoracic contrast echocardiography (TTCE) as a screening tool for pulmonary arteriovenous malformations (PAVMs) in children with hereditary hemorrhagic telangiectasia (HHT).Study DesignThis 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.ResultsBoth 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.ConclusionTTCE is a sensitive test for PAVMs in children with suspected HHT and can be a useful initial screening tool in pediatric HHT.Copyright © 2012 Mosby, Inc. All rights reserved.

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