Volume 14 Supplement 1
MRI in childhood Arrhythmogenic Right Ventricular Cardiomyopathy and proposed modification of the Task Force Criteria for children
© Grosse-Wortmann et al; licensee BioMed Central Ltd. 2012
Published: 1 February 2012
ARVC is a genetically determined cardiomyopathy which typically manifests clinically during the second to fourth decade of life. The diagnosis is made using a scoring system of signs and symptoms, known as the revised task force criteria (rTFC). MRI has recently been shown to be of little added value in the diagnosis of ARVC in adults. Its role in the pediatric age group is unclear. We sought assess the usefulness of magnetic resonance imaging (MRI) in the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC).
We retrospectively reviewed the MRI studies of all pediatric patients who were referred to MRI for signs of ARVC between 2005 and 2009. Following exclusion of serial studies in the same patient and those with poor image quality, 145 studies were analyzed for wall motion abnormalities (WMA), fibrofatty infiltration, and right ventricular (RV) volume. A diagnosis of possible, borderline, or definitive ARVC was made on the basis of the rTFC.
Unlike in adults, MRI is a useful and important tool in the diagnostic work-up of ARVC in children and adolescents. The reason lies within the more subtle degree of WMA in this age group which are not detected by echocardiography but found on MRI. In the pediatric age group, fibrofatty degeneration is found rarely and never without wall motion abnormalities. The respective sequences should be omitted in this population. Our data strengthen the concept that ARVC is a global, biventricular disease, rather than an isolated RV cardiomyopathy. Based on our data, we are proposing a modification of the rTFC to exclude certain criteria and incorporate non-rTFC MRI findings, leading to a novel scoring system for pediatric ARVC.
No external funding.
This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.