The authors wish to acknowledge funding from the Dutch Heart Foundation (to ASJMtR), the Alexandre Suerman Stipend (to ASJMtR), the Interuniversity Cardiology Institute of the Netherlands (to JAG), the National Heart, Lung, and Blood Institute (K23HL093350 to HT), the St. Jude Medical Foundation, and Medtronic Inc. The Johns Hopkins ARVD/C Program (ARVD.com) is supported by the Bogle Foundation, the Healing Hearts Foundation, the Campanella family, and Wilmerding Endowments, and the Dr. Francis P. Chiaramonte Private Foundation.
Mutation-Positive Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: The Triangle of Dysplasia Displaced
Article first published online: 25 JUL 2013
© 2013 Wiley Periodicals, Inc.
Journal of Cardiovascular Electrophysiology
Volume 24, Issue 12, pages 1311–1320, December 2013
How to Cite
TE RIELE, A. S.J.M., JAMES, C. A., PHILIPS, B., RASTEGAR, N., BHONSALE, A., GROENEWEG, J. A., MURRAY, B., TICHNELL, C., JUDGE, D. P., VAN DER HEIJDEN, J. F., CRAMER, M. J.M., VELTHUIS, B. K., BLUEMKE, D. A., ZIMMERMAN, S. L., KAMEL, I. R., HAUER, R. N.W., CALKINS, H. and TANDRI, H. (2013), Mutation-Positive Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: The Triangle of Dysplasia Displaced. Journal of Cardiovascular Electrophysiology, 24: 1311–1320. doi: 10.1111/jce.12222
Dr. Calkins received research support from Medtronic and St. Jude Medical. Other authors: No disclosures.
- Issue published online: 2 DEC 2013
- Article first published online: 25 JUL 2013
- Accepted manuscript online: 19 JUN 2013 11:43AM EST
- Manuscript Accepted: 12 JUN 2013
- Manuscript Received: 11 JUN 2013
- Dutch Heart Foundation (to ASJMtR)
- Alexandre Suerman Stipend (to ASJMtR)
- Interuniversity Cardiology Institute of the Netherlands (to JAG)
- National Heart, Lung, and Blood Institute. Grant Number: K23HL093350 to HT
- arrhythmogenic right ventricular dysplasia/cardiomyopathy;
- magnetic resonance imaging;
- electroanatomic mapping;
- ventricular tachcardia;
- implantable cardioverter defibrillator
ARVD/C: The Triangle of Dysplasia Displaced
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.
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.