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Mutation-Positive Arrhyhmogenic Right Ventricular Dysplasia/Cardiomyopathy: The Triangle of Dysplasia Displaced.
MedLine Citation:
PMID:  23889974     Owner:  NLM     Status:  Publisher    
INTRODUCTION: The traditional description of the Triangle of Dysplasia in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) predates genetic testing and excludes biventricular phenotypes.
METHODS AND RESULTS: We analyzed Cardiac Magnetic Resonance (CMR) studies of 74 mutation-positive ARVD/C patients for regional abnormalities on a 5-segment RV and 17-segment LV model. The location of electroanatomic endo- and epicardial scar and site of successful VT ablation was recorded in 11 ARVD/C subjects. Among 54/74 (73%) subjects with abnormal CMR, the RV was abnormal in almost all (96%), and 52% had biventricular involvement. Isolated LV abnormalities were uncommon (4%). Dyskinetic basal inferior wall (94%) was the most prevalent RV abnormality, followed by basal anterior wall (87%) dyskinesis. Subepicardial fat infiltration in the posterolateral LV (80%) was the most frequent LV abnormality. Similar to CMR data, voltage maps revealed scar (<0.5 mV) in the RV basal inferior wall (100%), followed by the RV basal anterior wall (64%) and LV posterolateral wall (45%). All 16 RV VTs originated from the basal inferior wall (50%) or basal anterior wall (50%). Of 3 LV VTs, 2 localized to the posterolateral wall. In both modalities, RV apical involvement never occurred in isolation.
CONCLUSION: Mutation-positive ARVD/C exhibits a previously unrecognized characteristic pattern of disease involving the basal inferior and anterior RV, and the posterolateral LV. The RV apex is only involved in advanced ARVD/C, typically as a part of global RV involvement. These results displace the RV apex from the Triangle of Dysplasia, and provide insights into the pathophysiology of ARVD/C.
Anneline S J M Te Riele; Cynthia A James; Binu Philips; Neda Rastegar; Aditya Bhonsale; Judith A Groeneweg; Brittney Murray; Crystal Tichnell; Daniel P Judge; Jeroen F VAN DER Heijden; Maarten J M Cramer; Birgitta K Velthuis; David A Bluemke; Stefan L Zimmerman; Ihab R Kamel; Richard N W Hauer; Hugh Calkins; Harikrishna Tandri
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-6-19
Journal Detail:
Title:  Journal of cardiovascular electrophysiology     Volume:  -     ISSN:  1540-8167     ISO Abbreviation:  J. Cardiovasc. Electrophysiol.     Publication Date:  2013 Jun 
Date Detail:
Created Date:  2013-7-29     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9010756     Medline TA:  J Cardiovasc Electrophysiol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2013 Wiley Periodicals, Inc.
Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands; Department of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
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