Mutation-positive arrhythmogenic right ventricular dysplasia/ cardiomyopathy: The triangle of dysplasia displaced

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

Research output: Contribution to journalArticle

Abstract

ARVD/C: The Triangle of Dysplasia Displaced 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.

Original languageEnglish (US)
Pages (from-to)1311-1320
Number of pages10
JournalJournal of cardiovascular electrophysiology
Volume24
Issue number12
DOIs
StatePublished - Dec 1 2013

Keywords

  • arrhythmogenic right ventricular dysplasia/cardiomyopathy
  • electroanatomic mapping
  • genetics
  • implantable cardioverter defibrillator
  • magnetic resonance imaging
  • phenotype
  • ventricular tachcardia

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

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  • Cite this

    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., & Tandri, H. (2013). Mutation-positive arrhythmogenic right ventricular dysplasia/ cardiomyopathy: The triangle of dysplasia displaced. Journal of cardiovascular electrophysiology, 24(12), 1311-1320. https://doi.org/10.1111/jce.12222