title: Impact of new task force criteria in the diagnosis of arrhythmogenic right ventricular cardiomyopathy.
abstract: BACKGROUND
  Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy that can lead to sudden cardiac death. The diagnostic criterion has recently been revised and through the use of cardiac magnetic resonance (CMR) imaging this study aimed to assess the clinical impact of comparing the original 1994 task force (TF) criterion to the revised 2010 criterion.
  
  
  METHODS
  We evaluated 173 consecutive CMR scans of patients referred with clinical suspicion of ARVC between 2008 and 2011. We then compared the prevalence of major and minor CMR criteria by applying the two criteria.
  
  
  RESULTS
  Using the 1994 TF criterion, 13 (7.5%) patients had definite, 11 (6.4%) had borderline, and 39 (22.5%) had possible ARVC. Using the 2010 TF criterion, 10 (5.8%) patients had definite, 1 had borderline, and 7 had (0.04%) possible ARVC. With the 1994 criterion, 81 patients satisfied CMR criterion, of which 36 (44%) had major and 45 (56%) had minor criteria. Upon reclassification with the revised criterion, 61 of the 81 patients were not assigned any criteria, even though many patients had significant risk factors. The negative predictive values (NPV) for both CMR criteria were 100% but the positive predictive values (PPV) for combined CMR major or minor criteria improved from 23% to 55%.
  
  
  CONCLUSIONS
  Revision of the criterion has enhanced the diagnostic capabilities of CMR but has resulted in a large cohort of patients not classified. In these patients, there is presently no official consensus on imaging or clinical strategy for surveillance of the evolution of pathology over time.
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Femia
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Chijen
authors.initials: C
authors.lastname: Hsu
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Suresh
authors.initials: S
authors.lastname: Singarayar
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Raymond W
authors.initials: RW
authors.lastname: Sy
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Michael
authors.initials: M
authors.lastname: Kilborn
authors.affiliation: Department of Radiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Geoffrey
authors.initials: G
authors.lastname: Parker
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Mark
authors.initials: M
authors.lastname: McGuire
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia; Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Sydney, Australia; Sydney Medical School, University of Sydney, Australia.
authors.forename: Chris
authors.initials: C
authors.lastname: Semsarian
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia. Electronic address: raj.puranik@cmrs.org.au.
authors.forename: Rajesh
authors.initials: R
authors.lastname: Puranik
mesh_terms: D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000145:classification / Q000175:diagnosis*; D057791:Cardiac Imaging Techniques / Q000379:methods*; D003953:Diagnosis-Related Groups; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D018487:Ventricular Dysfunction, Left / Q000145:classification / Q000175:diagnosis*; D018497:Ventricular Dysfunction, Right / Q000145:classification / Q000175:diagnosis*
keywords: Arrhythmogenic right ventricular cardiomyopathy; Cardiac magnetic resonance; RV dysplasia