Cardiovascular Magnetic Resonance in Arrhythmogenic Right Ventricular Cardiomyopathy Revisited: Comparison With Task Force Criteria and Genotype

摘要: OBJECTIVES We sought to assess the utility of cardiovascular magnetic resonance (CMR) in evaluation arrhythmogenic right ventricular cardiomyopathy (ARVC) relation diagnostic criteria and genotype. BACKGROUND Timely diagnosis ARVC is difficult as clinical findings may be subtle nonspecific early disease. The role CMR controversial owing absence a standardized protocol, insufficient experience with modality, inherent difficulties imaging ventricle. METHODS Comprehensive examination was performed 232 patients undergoing for suspected ARVC. outcomes were compared with: 1) prospective using Task Force guidelines, without proposed modifications familial ARVC; 2) gene-carrier status 35 individuals from genotyped families. RESULTS studies positive all 64 who prospectively fulfilled criteria, resulting 100% sensitivity. Specificity low (29%). Of 119 apparent false positives detected by CMR, however, 63 modified 7 obligate gene carriers, suggesting that frequently identifies disease, whom are relatively insensitive. This borne out (26 gene-positive 9 gene-negative), had sensitivity 96% specificity 78%. CONCLUSIONS valuable component workup when dedicated protocol specialists analysis volumes, wall motion, delayed-enhancement imaging.