Wieland Staab MD, Presenter: Nothing to Disclose

Jan Martin Sohns MD, Abstract Co-Author: Nothing to Disclose

Martin Fasshauer MD, Abstract Co-Author: Nothing to Disclose

Christian Sohns, Abstract Co-Author: Nothing to Disclose

Joachim Lotz MD, Abstract Co-Author: Research Cooperation, Siemens AG

Christina Unterberg-Buchwald, Abstract Co-Author: Nothing to Disclose

Alexander Schwarz, Abstract Co-Author: Nothing to Disclose

This study sought to determine the clinical influence of right and left ventricular findings in pediatric patients undergoing cardiac magnetic resonance imaging (CMRI) ≤ 18 years with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC).

In a consecutive series between September 2010 and December 2013 (38 months), 79 (14.0 ± 3.9 years, 46 male) young patients ≤ 18 underwent contrast-enhanced magnetic resonance imaging (CMRI) and genetic analysis after biopsy for evaluation clinically suspected ARVC.

Overall, 5 patients showed major criteria due to a combination of moderate to severe RV dysfunction and dilation as well as regional akinesia. Applying the revised TFC, 6 patients showed minor abnormalities such as mild RV dilatation, dys-synchronus RV contraction or regional akinesia. Overall 11 out of 12 (92%) patients with positive genetic characteristics were found to have major or minor abnormalities applying the revised Task Force Criteria. Here, positive predictive value (PPV) was 100%, negative predictive value (NPV) was 93%, sensitivity was 93% and specificity was 100%. Mean RVEDVI/BSA was 80 ± 16 and mean EF was 51 ± 8 in the whole study population. A subgroup analysis revealed a significantly (p = 0.01) decreased mean EF of 36 ± 9 and an increased RVEDVI/BSA of 101 ± 10 in 11 patients with major or minor abnormalities according to the revised TFC.

This is the first study applying the revised Task Force Criteria (TFC) regarding the detection of ARVC in young patient’s ≤ 18. In the current study, CMRI revealed 11 out of 12 patient`s (major and minor TFC) with positive findings in genetics with perfect positive predictive value and specificity.

Applying the revised Task Force Criteria (TFC) regarding the detection of ARVC in young patient’s ≤ 18 may increase the diagnostic value of CMR  in this context.

Staab, W, Sohns, J, Fasshauer, M, Sohns, C, Lotz, J, Unterberg-Buchwald, C, Schwarz, A, Cardiac Magnetic Resonance Imaging in Pediatric Patient’s ≤ 18 Years with Suspected Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC): A Correlation to Genetics.  Radiological Society of North America 2014 Scientific Assembly and Annual Meeting, - ,Chicago IL. http://archive.rsna.org/2014/14014677.html