TY - JOUR
T1 - Mutation-positive arrhythmogenic right ventricular dysplasia/ cardiomyopathy
T2 - The triangle of dysplasia displaced
AU - Te Riele, Anneline S.J.M.
AU - James, Cynthia A.
AU - Philips, Binu
AU - Rastegar, Neda
AU - Bhonsale, Aditya
AU - Groeneweg, Judith A.
AU - Murray, Brittney
AU - Tichnell, Crystal
AU - Judge, Daniel P.
AU - Van Der Heijden, Jeroen F.
AU - Cramer, Maarten J.M.
AU - Velthuis, Birgitta K.
AU - Bluemke, David A.
AU - Zimmerman, Stefan L.
AU - Kamel, Ihab R.
AU - Hauer, Richard N.W.
AU - Calkins, Hugh
AU - Tandri, Harikrishna
PY - 2013/12
Y1 - 2013/12
N2 - 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.
AB - 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.
KW - arrhythmogenic right ventricular dysplasia/cardiomyopathy
KW - electroanatomic mapping
KW - genetics
KW - implantable cardioverter defibrillator
KW - magnetic resonance imaging
KW - phenotype
KW - ventricular tachcardia
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U2 - 10.1111/jce.12222
DO - 10.1111/jce.12222
M3 - Article
C2 - 23889974
AN - SCOPUS:84889085540
SN - 1045-3873
VL - 24
SP - 1311
EP - 1320
JO - Journal of cardiovascular electrophysiology
JF - Journal of cardiovascular electrophysiology
IS - 12
ER -