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

摘要: ARVD/C: The Triangle of Dysplasia Displaced Introduction The traditional description the in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) predates genetic testing and excludes biventricular phenotypes. Methods Results We analyzed Cardiac Magnetic Resonance (CMR) studies 74 mutation-positive ARVD/C patients for regional abnormalities on a 5-segment RV 17-segment LV model. location electroanatomic endo- epicardial scar site successful VT ablation was recorded 11 subjects. Among 54/74 (73%) subjects with abnormal CMR, almost all (96%), 52% had involvement. Isolated were uncommon (4%). Dyskinetic basal inferior wall (94%) most prevalent abnormality, followed by anterior (87%) dyskinesis. Subepicardial fat infiltration posterolateral (80%) frequent abnormality. Similar to CMR data, voltage maps revealed (<0.5 mV) (100%), (64%) (45%). All 16 VTs originated from (50%) or (50%). Of 3 VTs, 2 localized wall. In both modalities, apical involvement never occurred isolation. Conclusion Mutation-positive exhibits previously unrecognized characteristic pattern disease involving RV, LV. apex is only involved advanced ARVD/C, typically as part global These results displace Dysplasia, provide insights into pathophysiology ARVD/C.