title: Echocardiography as a Screening Test for Myocardial Scarring in Children with Hypertrophic Cardiomyopathy.
abstract: Introduction. 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.Methods. 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.Results. 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%).Conclusions. 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.
authors.affiliation: Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, Canada.
authors.forename: Gregory
authors.identifier: 0000-0002-5915-8537
authors.initials: G
authors.lastname: Compton
authors.affiliation: Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, Canada.
authors.forename: Lynne
authors.initials: L
authors.lastname: Nield
authors.affiliation: Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, Canada.
authors.forename: Andreea
authors.initials: A
authors.lastname: Dragulescu
authors.affiliation: Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, Canada.
authors.forename: Lee
authors.initials: L
authors.lastname: Benson
authors.affiliation: 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.
authors.forename: Lars
authors.identifier: 0000-0002-1185-3805
authors.initials: L
authors.lastname: Grosse-Wortmann