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Background and Aims: Patients with repaired tetralogy of Fallot remain at risk of life-threatening ventricular tachycardia related to slow-conducting anatomical isthmuses (SCAIs). Preventive ablation of SCAI identified by invasive electroanatomical mapping is increasingly performed. This study aimed to non-invasively identify SCAI using 3D late gadolinium enhancement cardiac magnetic resonance (3D-LGE-CMR)., Methods: Consecutive tetralogy of Fallot patients who underwent right ventricular electroanatomical mapping (RV-EAM) and 3D-LGE-CMR were included. High signal intensity threshold for abnormal myocardium was determined based on direct comparison of bipolar voltages and signal intensity by co-registration of RV-EAM with 3D-LGE-CMR. The diagnostic performance of 3D-LGE-CMR to non-invasively identify SCAI was determined, validated in a second cohort, and compared with the discriminative ability of proposed risk scores., Results: The derivation cohort consisted of 48 (34 ± 16 years) and the validation cohort of 53 patients (36 ± 18 years). In the derivation cohort, 78 of 107 anatomical isthmuses (AIs) identified by EAM were normal-conducting AI, 22 were SCAI, and 7 blocked AI. High signal intensity threshold was 42% of the maximal signal intensity. The sensitivity and specificity of 3D-LGE-CMR for identifying SCAI or blocked AI were 100% and 90%, respectively. In the validation cohort, 85 of 124 AIs were normal-conducting AI, 36 were SCAI, and 3 blocked AI. The sensitivity and specificity of 3D-LGE-CMR were 95% and 91%, respectively. All risk scores showed an at best modest performance to identify SCAI (area under the curve ≤ .68)., Conclusions: 3D late gadolinium enhancement cardiac magnetic resonance can identify SCAI with excellent accuracy and may refine non-invasive risk stratification and patient selection for invasive EAM in tetralogy of Fallot., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background and Aims: Available data on continuous rhythm monitoring by implantable loop recorders (ILRs) in patients with Brugada syndrome (BrS) are scarce. The aim of this multi-centre study was to evaluate the diagnostic yield and clinical implication of a continuous rhythm monitoring strategy by ILRs in a large cohort of BrS patients and to assess the precise arrhythmic cause of syncopal episodes., Methods: A total of 370 patients with BrS and ILRs (mean age 43.5 ± 15.9, 33.8% female, 74.1% symptomatic) from 18 international centers were included. Patients were followed with continuous rhythm monitoring for a median follow-up of 3 years., Results: During follow-up, an arrhythmic event was recorded in 30.7% of symptomatic patients [18.6% atrial arrhythmias (AAs), 10.2% bradyarrhythmias (BAs), and 7.3% ventricular arrhythmias (VAs)]. In patients with recurrent syncope, the aetiology was arrhythmic in 22.4% (59.3% BAs, 25.0% VAs, and 15.6% AAs). The ILR led to drug therapy initiation in 11.4%, ablation procedure in 10.9%, implantation of a pacemaker in 2.5%, and a cardioverter-defibrillator in 8%. At multivariate analysis, the presence of symptoms [hazard ratio (HR) 2.5, P = .001] and age >50 years (HR 1.7, P = .016) were independent predictors of arrhythmic events, while inducibility of ventricular fibrillation at the electrophysiological study (HR 9.0, P < .001) was a predictor of VAs., Conclusions: ILR detects arrhythmic events in nearly 30% of symptomatic BrS patients, leading to appropriate therapy in 70% of them. The most commonly detected arrhythmias are AAs and BAs, while VAs are detected only in 7% of cases. Symptom status can be used to guide ILR implantation., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Aims: Mapping data of human ventricular fibrillation (VF) are limited. We performed detailed mapping of the activities underlying the onset of VF and targeted ablation in patients with structural cardiac abnormalities., Methods and Results: We evaluated 54 patients (50 ± 16 years) with VF in the setting of ischaemic (n = 15), hypertrophic (n = 8) or dilated cardiomyopathy (n = 12), or Brugada syndrome (n = 19). Ventricular fibrillation was mapped using body-surface mapping to identify driver (reentrant and focal) areas and invasive Purkinje mapping. Purkinje drivers were defined as Purkinje activities faster than the local ventricular rate. Structural substrate was delineated by electrogram criteria and by imaging. Catheter ablation was performed in 41 patients with recurrent VF. Sixty-one episodes of spontaneous (n = 10) or induced (n = 51) VF were mapped. Ventricular fibrillation was organized for the initial 5.0 ± 3.4 s, exhibiting large wavefronts with similar cycle lengths (CLs) across both ventricles (197 ± 23 vs. 196 ± 22 ms, P = 0.9). Most drivers (81%) originated from areas associated with the structural substrate. The Purkinje system was implicated as a trigger or driver in 43% of patients with cardiomyopathy. The transition to disorganized VF was associated with the acceleration of initial reentrant activities (CL shortening from 187 ± 17 to 175 ± 20 ms, P < 0.001), then spatial dissemination of drivers. Purkinje and substrate ablation resulted in the reduction of VF recurrences from a pre-procedural median of seven episodes [interquartile range (IQR) 4-16] to 0 episode (IQR 0-2) (P < 0.001) at 56 ± 30 months., Conclusions: The onset of human VF is sustained by activities originating from Purkinje and structural substrate, before spreading throughout the ventricles to establish disorganized VF. Targeted ablation results in effective reduction of VF burden., Key Question: The initial phase of human ventricular fibrillation (VF) is critical as it involves the primary activities leading to sustained VF and arrhythmic sudden death. The origin of such activities is unknown., Key Finding: Body-surface mapping shows that most drivers (≈80%) during the initial VF phase originate from electrophysiologically defined structural substrates. Repetitive Purkinje activities can be elicited by programmed stimulation and are implicated as drivers in 37% of cardiomyopathy patients., Take-Home Message: The onset of human VF is mostly associated with activities from the Purkinje network and structural substrate, before spreading throughout the ventricles to establish sustained VF. Targeted ablation reduces or eliminates VF recurrence., (© The Author(s) 2022. Published by Oxford University Press on behalf of European Society of Cardiology.)

Aims: Risk stratification of sudden cardiac arrest (SCA) in Brugada syndrome (Brs) remains the main challenge for physicians. Several scores have been suggested to improve risk stratification but never replicated. We aim to investigate the accuracy of the Brs risk scores., Methods and Results: A total of 1613 patients [mean age 45 ± 15 years, 69% male, 323 (20%) symptomatic] were prospectively enrolled from 1993 to 2016 in a multicentric database. All data described in the risk score were double reviewed for the study. Among them, all patients were evaluated with Shanghai score and 461 (29%) with Sieira score. After a mean follow-up of 6.5 ± 4.7 years, an arrhythmic event occurred in 75 (5%) patients including 16 SCA, 11 symptomatic ventricular arrhythmia, and 48 appropriate therapies. Predictive capacity of the Shanghai score (n = 1613) and the Sieira (n = 461) score was, respectively, estimated by an area under the curve of 0.73 (0.67-0.79) and 0.71 (0.61-0.81). Considering Sieira score, the event rate at 10 years was significantly higher with a score of 5 (26.4%) than with a score of 0 (0.9%) or 1 (1.1%) (P < 0.01). No statistical difference was found in intermediate-risk patients (score 2-4). The Shanghai score does not allow to better stratify the risk of SCA., Conclusions: In the largest cohort of Brs patient ever described, risk scores do not allow stratifying the risk of arrhythmic event in intermediate-risk patient., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2020. For permissions, please email: journals.permissions@oup.com.)

Aims: In patients with catecholaminergic polymorphic ventricular tachycardia (CPVT), implantable cardioverter-defibrillator (ICD) shocks are sometimes ineffective and may even trigger fatal electrical storms. We assessed the efficacy and complications of ICDs placed in patients with CPVT who presented with a sentinel event of sudden cardiac arrest (SCA) while undiagnosed and therefore untreated., Methods and Results: We analysed 136 patients who presented with SCA and in whom CPVT was diagnosed subsequently, leading to the initiation of guideline-directed therapy, including β-blockers, flecainide, and/or left cardiac sympathetic denervation. An ICD was implanted in 79 patients (58.1%). The primary outcome of the study was sudden cardiac death (SCD). The secondary outcomes were composite outcomes of SCD, SCA, appropriate ICD shocks, and syncope. After a median follow-up of 4.8 years, SCD had occurred in three patients (3.8%) with an ICD and none of the patients without an ICD (P = 0.1). SCD, SCA, or appropriate ICD shocks occurred in 37 patients (46.8%) with an ICD and 9 patients (15.8%) without an ICD (P < 0.0001). Inappropriate ICD shocks occurred in 19 patients (24.7%) and other device-related complications in 22 patients (28.9%)., Conclusion: In previously undiagnosed patients with CPVT who presented with SCA, an ICD was not associated with improved survival. Instead, the ICD was associated with both a high rate of appropriate ICD shocks and inappropriate ICD shocks along with other device-related complications. Strict adherence to guideline-directed therapy without an ICD may provide adequate protection in these patients without all the potential disadvantages of an ICD., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2019. For permissions, please email: journals.permissions@oup.com.)

Aims: Assess the impact of persistent/permanent atrial fibrillation (AF) ablation on endocrine and mechanical cardiac functions., Methods and Results: In all, 43 patients (40 males, 53 +/- 12 years) undergoing persistent/permanent AF ablation had atrial (ANP) and brain natriuretic peptide (BNP) measurements before day 1, 3, and 3 months after ablation. In the same period of time transthoracic echocardiography was performed. With a mean radiofrequency delivery of 98 +/- 29 min, sinus rhythm (SR) was restored in 30 patients (70%) without DC shock. ANP decreased significantly (P < 0.001) with restoration of SR and then increased until day 3 post ablation without reaching the level observed during AF. At 3 months, ANP was significantly lower than day 3 reaching normal value in 28 (65%) patients and being <7 pg/mL in 4 (9%). The BNP followed the same trend with normal BNP level in 23 (53%) patients at 3 months. Identifiable atrial filling waves on the pulsed Doppler transmitral recordings performed between day 2 and day 4 after the procedure were seen in 18 patients (42%). At 3 months, 39 (95%) of the patients with SR during echocardiography had a significant A wave., Conclusion: SR following persistent/permanent AF ablation is associated with a dramatic decrease in natriuretic peptides. At 3 months, despite relatively extensive atrial ablation, endocrine and mechanical cardiac functions are significantly improved.

Aims: To evaluate the contribution of the posterior left atrium (LA) to chronic atrial fibrillation (AF)., Methods and Results: Twenty-seven patients with chronic-AF were studied. After pulmonary vein (PV) isolation, the posterior-LA was isolated by ablation joining the right- and left-PVs using an irrigated-tip catheter. Isolation was demonstrated by absent/dissociated posterior-LA activity and the inability to pace the region. Ablation impact was determined by the effect on cycle length (CL) and AF termination. Posterior-LA isolation was achieved using 35 +/- 12 min of radiofrequency with total fluoroscopic and procedural durations of 64 +/- 16 and 199 +/- 46 min, resulting in abolition of electrograms (n = 21) or autonomous activity (n = 6; CL 820 +/- 343 ms). AFCL increased from 156 +/- 28 ms to 162 +/- 27 ms with PV-isolation and to 175 +/- 32 ms by posterior-LA exclusion (P < 0.0001). AF persisted in all after PV-isolation and terminated in 5 (19%) during posterior-LA-isolation. After 10 +/- 6 months, 12 patients developed atrial tachycardia (four) or AF (eight); four underwent repeat posterior-LA-isolation, while the others required additional ablation/antiarrhythmics. After 21 +/- 5 months, 17 (63%) were in sinus rhythm following posterior-LA-isolation., Conclusion: This study demonstrates the feasibility of complete posterior-LA exclusion by catheter ablation. This strategy results in maintenance of sinus rhythm in 63% at 2 years follow-up.

Aims: Catheter ablation of atrial fibrillation (AF) is centred on pulmonary vein (PV) ablation with or without additional atrial substrate modification. These procedures may be prolonged with significant fluoroscopy exposure. This study evaluates a new non-fluoroscopic navigation system during ablation of AF., Methods and Results: Seventy-two patients undergoing catheter ablation of symptomatic drug refractory AF were prospectively randomized to ablation with (n=35; study group) or without (n=37; control group) non-fluoroscopic navigation. PV isolation was performed in all patients. In patients with persistent or inducible sustained AF after PV isolation linear ablation was performed by joining the superior PVs. PV isolation was achieved in all patients; fluoroscopy (15.4+/-3.4 vs. 21.3+/-6.4 min; P<0.001) and procedural (52+/-12 vs. 61+/-17 min; P=0.02) durations were significantly reduced in the study group. Linear block was achieved in 37 of the 39 patients; with a significant reduction in fluoroscopy (5.6+/-2.2 vs. 9.9+/-4.8 min; P=0.003) and procedural (14.7+/-5.5 vs. 26.6+/-16.9 min; P=0.007) durations in the study group. After a follow-up of 6.9+/-2.9 months (range 3-10), 26 (74%) patients in the non-fluoroscopic navigation group and 29 (78%) patients in the control group were arrhythmia-free after the first procedure., Conclusion: This prospectively randomized study demonstrates significant reduction of fluoroscopy exposure and procedural duration using supplementary non-fluoroscopic imaging system for AF ablation.