Brugada syndrome (BrS) is a cardiac disease caused by an inherited ion channelopathy associated with a propensity to develop ventricular fibrillation. Implantable cardioverter defibrillator implantation is recommended in BrS, based on the clinical presentation in the presence of diagnostic ECG criteria. Implantable cardioverter defibrillator implantation is not always indicated or sufficient in BrS, and is associated with a high device complication rate. Pharmacological therapy aimed at rebalancing the membrane action potential can prevent arrhythmogenesis in BrS. Quinidine, a class 1A antiarrhythmic drug with significant Ito blocking properties, is the most extensively used drug for the prevention of arrhythmias in BrS. The present review provides contemporary data gathered on all drugs effective in the therapy of BrS, and on ineffective or contraindicated antiarrhythmic drugs.
Background: A novel 3D mapping system (KODEX-EPD, EPD Solutions) enables catheter localization and real-time 3D cardiac mapping.Objective: To evaluate left atrium (LA) anatomical mapping accuracy created by the KODEX-EPD system during pulmonary vein isolation (PVI) compared with gold standard computed tomography (CT) images acquired from the same patients before the procedure.Methods: In 15 consecutive patients who underwent PVI, 3D mapping of the LA was created on the KODEX-EPD system using the Achieve catheter. Pulmonary vein (PV), posterior wall, and appendage anatomy and diameters, were compared to the CT 3D reconstruction measured on the CARTO 3 system. Measurements were done independently by two physicians in each method. Linear correlation and agreement between CT and EPD measurements were assessed by Spearman correlation and Bland-Altman plot.Results: Mean LA mapping time was 7.7 ± 3.6 min. Very high interobserver correlation was found for both EPD and CT measurements (Spearman r = .9). High correlation (r = .75) was found between CT and EPD measurements. Bland-Altman plot method revealed that measurements assessed by EPD were slightly higher than those assessed by CT. Mean difference was 3.5 mm, p < .01. In 2 (13.5%) patients each, disagreement regarding the presence of a left common PV and a right middle accessory vein anatomy was seen.
Conclusion:The new KODEX-EPD mapping system allows quick and accurate mapping of the LA with high correlation to CT imaging. Some differences in left common and accessory right middle vein anatomy were seen.
Funding Acknowledgements
Type of funding sources: None.
Background
Short-coupled idiopathic ventricular fibrillation (SC-IVF) is a rare, potentially lethal arrhythmia, initiated by SC-PVCs. Only small series have been published.
Purpose
To assess the clinical characteristics, therapies and long-term outcomes of patients (pts) with SC-IVF.
Methods
Data from all published case reports on SC-IVF/Polymorphic VT were collected. Inclusion criteria were normal cardiac work-up including during follow-up (FU), and documented SC-PVC-VF available for our analysis and ruling out early repolarization syndromes and CPVT at VF onset. Updated information was provided by 82% of authors contacted, with 22 additional ECG tracings provided.
Results
A total of 100 pts’ cases were identified, including 82 who met inclusion criteria. Mean FU increased from 2.75+3.6 years in the original publications to 10.5+7.9 years (p<0.0001). There were 42 (51%) males, aged 39.6+13.5 years at the time of SC-IVF diagnosis. Pts presented with syncope (40%), aborted cardiac arrest (ACA) (16%), ACA + arrhythmic storm (13%), arrhythmic storm (10%), ICD shocks (8%) or palpitations (1%). Prior history included syncope in 32 (39%) pts. Mean coupling interval of the SC-PVC was 293.6+50.7ms. Presumed sites of PVC origin were the RV, LV and RVOT in 54%, 21% and 5% of pts, respectively. A total of 69 (84%) pts had an ICD implanted before or after SC-IVF documentation. During long-term treatment with quinidine (n=11) or verapamil (n=32), SC-IVF did not recur in 82% and 50% of pts, respectively (p=0.08). Ablation was performed in 43 (92%) of the 47 pts in whom it was attempted. Ablation sites were the Purkinje RV (43%) or LV (23%), and the non-Purkinje RV (19%) or LV (2%). Acute and late success rates after a first ablation were 83% and 61.7%, respectively. A second ablation in 7 pts yielded a 74.5% total success rate. No difference was found in the success rate of quinidine compared with ablation procedures (p=0.6). During FU, 4 pts died including 2 from sudden cardiac death; both pts were treated with verapamil and refused ICD implantation or replacement.
Conclusion
SC-IVF mainly presents following syncope. Quinidine and ablation procedures have comparable long-term high efficacy in arrhythmia control. The commonest sites of successful ablation are the RV and LV Purkinje fibers.
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