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Background: Among inherited cardiomyopathies involving the left ventricle, whether dilated or not, certain genotypes carry a well-established arrhythmic risk, notably manifested as sustained monomorphic ventricular tachycardia (SMVT). Nonetheless, the precise localization and electrophysiological profile of this substrate remain undisclosed across different genotypes., Methods: Patients diagnosed with cardiomyopathy and left ventricle involvement due to high-risk genetic variants and SMVT treated by electrophysiological study were recruited from 18 European/US centers. Electrophysiological study, imaging, and outcomes data after ablation were assessed in relation to genotype., Results: Seventy-one patients were included (49.6 Q1-Q3 [40-60] years, 76% men). They were divided into 4 groups according to the affected protein: desmosomal ( DSP , PKP2 , DSG2 , and DSC2 ), nuclear membrane ( LMNA and TMEM43 ), cytoskeleton ( FLNC and DES ), and sarcoplasmic reticulum ( PLN ). Desmosomal genes, TMEM43 , and PLN were associated with biventricular disease, while variants in LMNA and cytoskeleton genes had predominant left ventricle involvement ( P =0.001). The location of the clinical-SMVT substrate was significantly different based on genotype ( P =0.005). DSP and cytoskeleton genes presented SMVTs with right bundle branch block morphology, which origin was identified in the inferolateral segments of the left ventricle. The other desmosomal genes ( PKP2 and DSG2 ), along with TMEM43 , showed SMVTs with left bundle branch block morphology and predominantly right ventricular substrate. In contrast, LMNA substrate was mainly observed in the interventricular septum. During a median of 26 Q1-Q3 (10.6-65) months, 27% of patients experienced recurrences of clinical SMVT with differences between genotypes (log-rank 0.016). Nuclear membrane genes demonstrated the highest recurrence rate compared with desmosomal genes (hazard ratio, 4.56 [95% CI, 1.5-13.8])., Conclusions: The anatomic substrate of SMVTs shows a strong correlation with the underlying genotype, electrocardiographic morphology, and recurrence rate. Particularly, patients with nuclear membrane gene variants have a significantly higher recurrence rate compared with those with desmosomal gene variants., Competing Interests: None.

Idiopathic verapamil-sensitive fascicular ventricular tachycardia (VT) is the most common form of Purkinje-related ventricular tachycardia (PRVT). Left septal fascicle (LSF) involvement and its connections with the other fascicles, have been recently reported as a pathophysiologic mechanism for this form of PRVT. We describe a case of idiopathic PRVT with LSF involvement using omnipolar technology (OT) mapping in relation to a false tendon. Ablation in the area with concealed fusion entrainment did not terminate the tachycardia. However, radiofrequency application in the area of LSF with manifest fusion entrainment, resulted in immediate tachycardia termination. Six months follow-up showed no tachycardia recurrence., (© 2023 Wiley Periodicals LLC.)

Objective: To investigate the association of accelerometer-measured lifestyle physical activity with rapid-rate non-sustained ventricular tachycardias (RR-NSVTs) in patients with arrhythmogenic cardiomyopathy (AC)., Methods: This multicentre, observational study enrolled 72 patients with AC, including right, left and biventricular forms of the disease, with underlying desmosomal and non-desmosomal mutations. Lifestyle physical activity, objectively monitored with accelerometers (ie, movement sensors) and RR-NSVT, identified as >188 bpm and >18 beats from a textile Holter ECG for 30 days., Results: Sixty-three patients with AC (38±17.6 years, 57% men) were included. A total of 17 patients experienced ≥1 RR-NSVTs, and a total of 35 events were recorded. The odds of occurrence of ≥1 RR-NSVT during the recording did not increase as a function of either total physical activity (OR 0.95, 95% CI (CI 95% ) 0.68 to 1.30 for 60 min increase) or moderate-to-vigorous activities (OR 0.89, CI 95% 0.71 to 1.08 for 5 min increase). Participants presenting RR-NSVTs during the recording (n=17) did not present greater odds of RR-NSVT in the days with more time either in total physical activity (OR 1.05, CI 95% 0.84 to 1.29 for additional 60 min) or moderate-to-vigorous activities (OR 1.05, CI 95% 0.97 to 1.12 for additional 5 min). Physical activity levels were neither different between the patients with and without RR-NSVTs during the recording period nor in the days of occurrence of RR-NSVT compared with the rest of the days. Finally, 4 of the 35 RR-NSVTs recorded in the 30 days occurred during physical activity (3 during moderate-to-vigorous intensity and 1 during light-intensity activities)., Conclusions: These findings suggest that lifestyle physical activity is not associated with RR-NSVTs in patients with AC., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)