Giuseppe Femia1, Chijen Hsu1, Suresh Singarayar1, Raymond W Sy1, Michael Kilborn1, Geoffrey Parker2, Mark McGuire1, Chris Semsarian3, Rajesh Puranik4. 1. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia. 2. Department of Radiology, Royal Prince Alfred Hospital, Sydney, Australia. 3. 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. 4. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia. Electronic address: raj.puranik@cmrs.org.au.
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.
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: Domenico Corrado; Peter J van Tintelen; William J McKenna; Richard N W Hauer; Aris Anastastakis; Angeliki Asimaki; Cristina Basso; Barbara Bauce; Corinna Brunckhorst; Chiara Bucciarelli-Ducci; Firat Duru; Perry Elliott; Robert M Hamilton; Kristina H Haugaa; Cynthia A James; Daniel Judge; Mark S Link; Francis E Marchlinski; Andrea Mazzanti; Luisa Mestroni; Antonis Pantazis; Antonio Pelliccia; Martina Perazzolo Marra; Kalliopi Pilichou; Pyotr G A Platonov; Alexandros Protonotarios; Alessandra Rampazzo; Jeffry E Saffitz; Ardan M Saguner; Christian Schmied; Sanjay Sharma; Hari Tandri; Anneline S J M Te Riele; Gaetano Thiene; Adalena Tsatsopoulou; Wojciech Zareba; Alessandro Zorzi; Thomas Wichter; Frank I Marcus; Hugh Calkins Journal: Eur Heart J Date: 2020-04-07 Impact factor: 29.983
Authors: Giuseppe Femia; Christopher Semsarian; Mark McGuire; Raymond W Sy; Rajesh Puranik Journal: J Cardiovasc Magn Reson Date: 2019-12-12 Impact factor: 5.364
Authors: Annina S Vischer; David J Connolly; Caroline J Coats; Virginia Luis Fuentes; William J McKenna; Silvia Castelletti; Antonios A Pantazis Journal: Acta Myol Date: 2017-09-01