Diagnosis of Brugada Syndrome With a Sodium-Channel-Blocker Test: Who Should Be Tested? Who Should Not?

Intravenous infusion of sodium-channel blockers (SCB) with either ajmaline, flecainide, procainamide, or pilsicainide to unmask the ECG of Brugada syndrome is the drug challenge most commonly used for diagnostic purposes when investigating cases possibly related to inherited arrhythmia syndromes. For a patient undergoing an SCB challenge, the impact of a positive result goes well beyond its diagnostic implications. It is, therefore, appropriate to question who should undergo a SCB test to diagnose or exclude Brugada syndrome and, perhaps more importantly, who should not. We present a critical review of the benefits and drawbacks of the SCB challenge when performed in cardiac arrest survivors, patients presenting with syncope, family members of probands with confirmed Brugada syndrome, and asymptomatic patients with suspicious ECG.

Arrhythmogenic Effects of Cardiac Memory.

Cardiac memory is the term used to describe an interesting electrocardiographic phenomenon. Whenever a QRS complex is wide and abnormal, such as during ventricular pacing, the T waves will also be abnormal and will point to the opposite direction of the wide QRS. If the QRS then normalizes, such as after cessation of ventricular pacing, the T waves will normalize as well, but at a later stage. The period of cardiac memory is the phase between the sudden normalization of the QRS and the eventual and gradual return of the T waves to their baseline morphology. Cardiac memory is assumed to be an innocent electrocardiographic curiosity. However, during cardiac memory, reduction of repolarizing potassium currents increases left ventricular repolarization gradients. Therefore, when cardiac memory occurs in patients who already have a prolonged QT interval (for whatever reason), it can lead to a frank long QT syndrome with QT-related ventricular arrhythmias (torsades de pointes). These arrhythmogenic effects of cardiac memory are not generally appreciated and are reviewed here for the first time.

Polymorphic Ventricular Tachycardia: Terminology, Mechanism, Diagnosis, and Emergency Therapy.

Polymorphic ventricular tachyarrhythmias are highly lethal arrhythmias. Several types of polymorphic ventricular tachycardia have similar electrocardiographic characteristics but have different modes of therapy. In fact, medications considered the treatment of choice for one form of polymorphic ventricular tachycardia, are contraindicated for the other. Yet confusion about terminology, and thus diagnosis and therapy, continues. We present an in-depth review of the different forms of polymorphic ventricular tachycardia and propose a practical step-by-step approach for distinguishing these malignant arrhythmias.

Radial strain imaging-guided lead placement for improving response to cardiac resynchronization therapy in patients with ischaemic cardiomyopathy: the Raise CRT trial.

AIMS: To evaluate the benefit of speckle tracking radial strain imaging (STRSI)-guided left ventricular (LV) lead (LVL) positioning in cardiac resynchronization therapy (CRT) in patients (pts) with ischaemic cardiomyopathy with CRT indication. METHODS AND RESULTS: We conducted a prospective randomized controlled trial. Patients were enrolled in nine centres with 2:1 randomization into two groups (guided vs. control). Patients underwent STRSI to identify the optimal LV position from six LV segments at midventricular level. Implantation via STRSI was attempted for recommended segment in the guided group only. Follow-up included echocardiography (6 months) and clinical evaluation (6 and 12 months). The primary endpoint was comparison % reduction in LV end-systolic volume at 6 months with baseline. Secondary endpoints included hospitalizations for heart failure and death, and improvement in additional echocardiographic measurements and quality of life score. A total of 172 patients (115 guided vs. 57 control) were enrolled. In the guided group, 60% of the implanted LV leads were adjudicated to be successfully located at the recommended segment, whereas in the control group 44% reached the best STRSI determined segment. There was no difference between the groups in any of the primary or secondary endpoints at 6 and 12 months. CONCLUSION: Our findings suggest that echo-guided implantation of an LV lead using STRSI does not improve the clinical or echocardiographic response compared with conventional implantation.

Polymorphic ventricular tachycardia, ischaemic ventricular fibrillation, and torsade de pointes: importance of the QT and the coupling interval in the differential diagnosis.

AIMS: Distinctive types of polymorphic ventricular tachycardia (VT) respond differently to different forms of therapy. We therefore performed the present study to define the electrocardiographic characteristics of different forms of polymorphic VT. METHODS AND RESULTS: We studied 190 patients for whom the onset of 305 polymorphic VT events was available. The study group included 87 patients with coronary artery disease who had spontaneous polymorphic VT triggered by short-coupled extrasystoles in the absence of myocardial ischaemia. This group included 32 patients who had a long QT interval but nevertheless had their polymorphic VT triggered by ectopic beats with short coupling interval, a subcategory termed 'pseudo-torsade de pointes] (TdP). For comparison, we included 50 patients who had ventricular fibrillation (VF) during acute myocardial infarction ('ischaemic VF' group) and 53 patients with drug-induced TdP ('true TdP' group). The QT of patients with pseudo-TdP was (by definition) longer than that of patients with polymorphic VT and normal QT (QTc 491.4 ± 25.2 ms vs. 447.3 ± 55.6 ms, P < 0.001). However, their QT was significantly shorter than that of patients with true TdP (QTc 564.6 ± 75.6 ms, P < 0.001). Importantly, the coupling interval of the ectopic beat triggering the arrhythmia was just as short during pseudo-TdP as during polymorphic VT with normal QT (359.1 ± 38.1 ms vs. 356.6 ± 39.4 ms, P = 0.467) but was much shorter than during true TdP (581.2 ± 95.3 ms, P < 0.001). CONCLUSIONS: The coupling interval helps discriminate between polymorphic VT that occurs despite a long QT interval (pseudo-TdP) and polymorphic arrhythmias striking because of a long QT (true TdP).

Comparison of permanent pacemaker implantation rate after first and second generation of transcatheter aortic valve implantation-A retrospective cohort study.

OBJECTIVES: This study aimed to compare permanent pacemaker implantation (PPMI) rates among patients undergoing Trans-catheter Aortic Valve Implantation (TAVI) with first generation (G1) versus second generation (G2) valves and the impact of PPMI on long-term mortality. BACKGROUND: PPMI is a known adverse event after TAVI. Recently, two novel iterations of valve designs of both the balloon expandable valves (BEV) and self-expanding valves (SEV) were introduced as a second generation valves. METHODS: All patients included in the Israeli multicenter TAVI registry were grouped according to valve type (BEV vs. SEV) and generation (G1 vs. G2). A comparison was made for clinical and outcome indices of patients undergoing TAVI with G1 and G2 in each of the valve systems. RESULTS: A total of 1377 patients were included. The incidence of PPMI did not differ between G1-BEV versus G2-BEV (15.3% vs. 17.4%; p = 0.598) nor between G1-SEV versus G2-SEV (23.4% vs. 20.3%; p = 0.302). Depth of implantation and complete right bundle branch block were independently associated with PPMI post-TAVI in both valve systems. PPMI was not associated with an increased risk for 2-year mortality. CONCLUSIONS: The incidence of PPMI remains a relevant adverse event post-TAVI even when the newer generation valves are used. Since the predictors for PPMI are well established, a standardized approach for the management of conduction disorders is much needed.

Arrhythmic storm from ischemic ventricular fibrillation treated with intravenous quinidine.

We present a case who developed an acute right ventricular infarction. The leads demonstrating ST-segment elevation were different than those expected based on previous publications. We explain why this happened with the aid of 3-dimentional imaging. Our case then developed an arrhythmic storm caused by ischemic ventricular fibrillation (VF). Emergency revascularization failed and the VF-storm failed to respond to sedation, lidocaine and amiodarone but responded to intravenous quinidine.

Pulmonary Vein Isolation With Ablation Index via Single Transseptal Crossing: Critical Role of Carina Isolation.

AIM: Reconnection of the pulmonary veins (PVs) is the most common reason for the recurrence of atrial fibrillation (AF). The ablation index is a marker of ablation lesion quality that achieves high percentages of first-pass isolation and improved AF ablation results. Most operators use a double transseptal approach with confirmation of PV isolation with a circular mapping catheter. In the present study we aimed to show that an ablation index-guided procedure using a single transseptal approach and ablation catheter only would achieve adequate PV isolation while demonstrating the critical role of the carina in PV isolation. METHOD: Sixty-six (66) consecutive patients with paroxysmal AF were included. Thirty-four (34) patients underwent wide antral circumferential ablation (WACA-only) and 32 underwent WACA+ (WACA + empiric carina isolation). All procedures were performed via single transseptal approach. Pulmonary vein isolation was confirmed with the use of a circular mapping catheter in both groups. RESULTS: Compared to WACA-only, WACA+ increased the odds of PV isolation from 65% to 94% (p=0.011). In the WACA-only procedure, ablation of the carina was needed to achieve PV isolation. At the 18-month follow-up (interquartile range 15.2-20.8 months), freedom from AF was 84% for the entire cohort. CONCLUSIONS: Our study confirmed the high success rate of PV isolation using the ablation index and showed that this can be achieved via a single transseptal crossing. Our study confirmed the role of the carina in PV isolation.

Quinidine-Responsive Polymorphic Ventricular Tachycardia in Patients With Coronary Heart Disease.

BACKGROUND: Polymorphic ventricular tachycardia (VT) without QT prolongation is well described in patients without structural heart disease (mainly idiopathic ventricular fibrillation and Brugada syndrome) and in patients with acute ST-elevation myocardial infarction. METHODS: Retrospective study of patients with polymorphic VT related to coronary artery disease, but without evidence of acute myocardial ischemia. RESULTS: The authors identified 43 patients in whom polymorphic VT developed within days of an otherwise uncomplicated myocardial infarction or coronary revascularization procedure. The polymorphic VT events were invariably triggered by extrasystoles with short (364±36 ms) coupling interval. Arrhythmic storms (4-16 events of polymorphic VT deteriorating to ventricular fibrillation) occurred in 23 (53%) patients. These arrhythmic storms were always refractory to conventional antiarrhythmic therapy, including intravenous amiodarone, but invariably responded to quinidine therapy. In-hospital mortality was 17% for patients with arrhythmic storm. Patients treated with quinidine invariably survived to hospital discharge. During long-term follow-up (of 5.6±6 years; range, 1 month to 18 years), 3 (16%) of patients discharged without quinidine developed recurrent polymorphic VT. There were no recurrent arrhythmias during quinidine therapy Conclusions: Arrhythmic storm with recurrent polymorphic VT in patients with coronary disease responds to quinidine therapy when other antiarrhythmic drugs (including intravenous amiodarone) fail.

Quinidine-responsive out-of-hospital polymorphic ventricular tachycardia in patients with coronary heart disease.

AIMS: We recently reported that patients with coronary artery disease (CAD) who develop polymorphic ventricular tachycardia (VT) during the healing phase of an acute coronary event, generally fail to respond to revascularization or standard antiarrhythmic therapy but respond immediately to quinidine therapy. Here, we describe that CAD patients presenting with out-of-hospital polymorphic VT without a recent coronary event or an obvious precipitating factor, also respond uniquely to quinidine therapy. METHODS AND RESULTS: Retrospective study of patients with unheralded, mainly out-of-hospital, polymorphic VT related to CAD but without evidence of acute myocardial ischaemia. We identified 20 patients who developed polymorphic VT without precipitating factors. The polymorphic VT events were triggered by extrasystoles with short (376 ± 49 ms) coupling interval. Arrhythmic storms occurred in 70% patients. These arrhythmic storms were generally refractory to conventional antiarrhythmic therapy but invariably responded to quinidine therapy. Revascularization was antiarrhythmic in 3 patients despite the absent clinical or ECG signs of ischaemia. During long-term follow-up (range 2 months to 11 years), 3 (15%) of patients not receiving quinidine developed recurrent polymorphic VT. There were no recurrent arrhythmias during long-term quinidine therapy. CONCLUSIONS: Patients with CAD may develop polymorphic VT in the absence of obvious acute ischaemia or apparent precipitating factors, presenting as out-of-hospital polymorphic VT with high risk of arrhythmic storms that respond uniquely to quinidine therapy.

Continuous heart rhythm monitoring using mobile photoplethysmography in ambulatory patients.

BACKGROUND: Wearable devices using photo-plethysmography (PPG) can accurately detect heart beats and may be useful for heart rate measurement and diagnosis of arrhythmias such as atrial fibrillation (AF). A previous study of a new portable PPG sensor (CardiacSense) showed high accuracy in heart rate measurement and AF detection in resting patients. We report a trial done to test the same device in active ambulatory patients with diverse characteristics. METHODS: A cohort of 24 ambulatory volunteers, underwent simultaneous PPG recording and continuous electrocardiogram (ECG) recording under different environmental conditions and situations. Per study protocol, the subjects were diverse in age, BMI, hair density and skin tone. Four subjects had AF. Heart rate measurement using the PPG device was compared to measurements by ECG. RESULTS: Of 163,527 recorded ECG-detected beats in the trial, 86,929 (53.2%) were also recorded by the PPG device. Most undetected heart beats were due to motion induced noise. Correlation between ECG and PPG was high (R = 0.94, p < 0.0001), yet in subjects with AF correlation was lower (R = 0.80, p < 0.0001). A Bland-Altman analysis showed the mean difference between measurements was -0.7 ms (95% limit of agreement -93.8 to 92.2). A total of 86,217 (99.9%) of all RR measurements were reliably measured (RR difference within 100 ms). Reliability was sustained (>99.8%) in subjects of all groups including subjects with AF. CONCLUSIONS: This study showed that, in the absence of movement-related noise, the CardiacSense PPG device can reliably detect HR in a variety of situations and subjects' characteristics.

Use of Intracardiac Echocardiography in Interventional Cardiology: Working With the Anatomy Rather Than Fighting It.

The indications for catheter-based structural and electrophysiological procedures have recently expanded to more complex scenarios, in which an accurate definition of the variable individual cardiac anatomy is key to obtain optimal results. Intracardiac echocardiography (ICE) is a unique imaging modality able to provide high-resolution real-time visualization of cardiac structures, continuous monitoring of catheter location within the heart, and early recognition of procedural complications, such as pericardial effusion or thrombus formation. Additional benefits are excellent patient tolerance, reduction of fluoroscopy time, and lack of need for general anesthesia or a second operator. For these reasons, ICE has largely replaced transesophageal echocardiography as ideal imaging modality for guiding certain procedures, such as atrial septal defect closure and catheter ablation of cardiac arrhythmias, and has an emerging role in others, including mitral valvuloplasty, transcatheter aortic valve replacement, and left atrial appendage closure. In electrophysiology procedures, ICE allows integration of real-time images with electroanatomic maps; it has a role in assessment of arrhythmogenic substrate, and it is particularly useful for mapping structures that are not visualized by fluoroscopy, such as the interatrial or interventricular septum, papillary muscles, and intracavitary muscular ridges. Most recently, a three-dimensional (3D) volumetric ICE system has also been developed, with potential for greater anatomic information and a promising role in structural interventions. In this state-of-the-art review, we provide guidance on how to conduct a comprehensive ICE survey and summarize the main applications of ICE in a variety of structural and electrophysiology procedures.

Risk of death without appropriate defibrillator shock in patients with advanced renal dysfunction.

AIMS: Heart failure patients with advanced chronic kidney disease (CKD) may experience an increased rate of non-arrhythmic mortality due to associated comorbidities. We aimed to evaluate the risk of mortality without appropriate implantable cardioverter-defibrillator (ICD) shocks in this high-risk population. METHODS AND RESULTS: The study population comprised 3542 patients who received an ICD, were enrolled, and prospectively followed-up in the Israeli ICD registry. Study patients were categorized into two groups: those with advanced CKD [defined by a glomerular filtration rate of <30 mL/min/1.73 m2 or being on dialysis at time of implantation (n = 197)], and those without advanced CKD (n = 3344). The primary endpoint was the risk of death without receiving appropriate ICD shock. Kaplan-Meier survival analysis showed that at 5 years of follow-up the rates of death without prior ICD shock were significantly higher in the advanced kidney disease group (46%) compared with the non-advanced CKD group (19%; log-rank P-value <0.001). Consistently, multivariate analysis showed that the risk of death without receiving appropriate ICD shock therapy at 5 years was 2.5-fold (P < 0.001) higher among advanced CKD patients. In contrast, the rate of appropriate ICD shock therapy at 5 years among advanced CKD patients was only 9%, with a very high mortality rate (63%) within 3.5 years subsequent to shock therapy. CONCLUSION: Nearly one-half of ICD with advanced CKD die within 5 years without receiving an appropriate ICD shock. These findings stress the importance of appropriate patient selection for primary ICD implantation in this high-risk population.

Inadvertent transseptal puncture into the aortic root: the narrow edge between luck and catastrophe in interventional cardiology.

AIMS: Inadvertent puncture of the aortic root (AR) is a well-known complication of transseptal puncture (TSP). Strategies for handling of this potentially lethal complication have not been identified yet. In this study, we present typical anatomical locations and clinical management of aortic root puncture (ARP) due to TSP. METHODS AND RESULTS: All patients with ARP were retrospectively collected from seven hospitals. Aortic root puncture was identified and classified regarding angiographical and intraoperative findings in cardiac surgery: (i) TSP from the right atrium (RA) to the non-coronary sinus (NCS), (ii) TSP from RA to the non-coronary sinutubular junction (STJ), and (iii) TSP from RA to the ascending aorta (AA). A total of 24 patients with inadvertent ARP were identified. In 19 patients, penetration of the aorta was accomplished by the inner dilator, in 5 patients by the complete sheath. Previous cardiac surgery had been performed in six patients. There were 13 RA-to-NCS punctures, 2 RA-to-STJ punctures, and 9 RA-to-AA punctures. No cardiac tamponade (CT) occurred in patients with RA-to-NCS and RA-to-STJ punctures. In 8 of 9 patients with RA-to-AA puncture, CT occurred immediately requiring urgent pericardiocentesis and surgical repair. Two patients died after surgical repair. In the 16 patients without surgical therapy, no shunt from the AR to the RA was observed 3 months after the procedure. CONCLUSION: Aortic root puncture due to mislead TSP via NCS or STJ is usually not associated with a severe clinical course while ARP into the AA via the epicardial space generally leads to CT requiring surgical repair.

Continuous heart rate monitoring for automatic detection of atrial fibrillation with novel bio-sensing technology.

BACKGROUND: Asymptomatic atrial fibrillation [AF] is an important cause of fatal or disabling stroke. A continuous heart-rate monitoring device, comfortable enough to be worn continuously and reliable enough to detect AF, would allow for prompt initiation of anticoagulation therapy to prevent stroke. METHODS: We studied a new custom-made wearable photo-plethysmograph [PPG] wrist-watch sensor, specifically designed for continuous heart rate monitoring and incorporating contact and motion noise-filters. We tested its ability to automatically detect AF in patients undergoing elective cardioversion of AF, using simultaneously recorded electrocardiogram [ECG] as gold standard. RESULTS: A total of 18,608 consecutive R-R-interval measurements were recorded simultaneously with PPG and ECG in 20 patients, including 12,521 [67.3%] R-R-intervals during AF and 6087 [32.7%] R-R intervals during sinus rhythm. Scatter plots and Bland-Altman plots demonstrated that the PPG signals were highly correlated to the simultaneously recorded ECG [R = 0.980, p < 0.001], both during AF and during sinus rhythm. The automatic algorithm distinguished AF from sinus rhythm with a sensitivity of 100% and specificity of 93.1%. CONCLUSIONS: This PPG-based wrist-watch sensor reliably detected AF in non-ambulatory patients.

The Utility of Prophylactic Pacemaker Implantation in Right Bundle Branch Block Patients Pre-Transcatheter Aortic Valve Implantation.

BACKGROUND: Patients with right bundle branch block (RBBB) prior to transcatheter aortic valve implantation (TAVI) are at high risk for immediate post-procedural heart block and long-term mortality when discharged without a pacemaker. OBJECTIVES: To test whether prophylactic permanent pacemaker implantation (PPI) is beneficial. METHODS: Of 795 consecutive patients who underwent TAVI, 90 patients had baseline RBBB. We compared characteristics and outcomes of the prophylactic PPI with post-TAVI PPI. Need for pacing was defined as  greater than 1% ventricular pacing. RESULTS: Forty patients with RBBB received a prophylactic PPI (group 1), and in 50 the decision was based on standard post-procedural indications (group 2). There were no significant differences in clinical baseline characteristics. One patient developed a tamponade after a PPI post-TAVI. A trend toward shorter hospitalization duration in group 1 patients was observed (P = 0.06). On long-term follow-up of 848 ± 56 days, no differences were found in overall survival (P = 0.77), the composite event-free survival of both mortality and hospitalizations (P = 0.66), or mortality and syncope (P = 0.65). On multivariate analysis, independent predictors of the need for pacing included baseline PR interval increase of 10ms (odds ratio [OR] 1.21 per 10 ms increment 95% confidence interval [95%CI] 1.02-1.44, P = 0.028), and the use of new generation valves (OR 3.92, 95%CI 1.23-12.46, P = 0.023). CONCLUSIONS: In patients with baseline pre-TAVI RBBB, no outcome differences were found with prophylactic PPI. On multivariate analysis, predictors of the need for pacing included baseline long PR interval, and the use of newer generation valves.

Should We Perform Catheter Ablation for Asymptomatic Atrial Fibrillation?

Since the original description of atrial fibrillation ablation, numerous studies have demonstrated the superiority of catheter ablation over pharmacological therapy for maintenance of sinus rhythm in patients with both paroxysmal and persistent atrial fibrillation. However, to date, no randomized studies have been powered to demonstrate a mortality or stroke reduction benefit of rhythm control with catheter ablation over a rate control strategy. The results of such ongoing studies are not expected until 2018 or 2019. Thus, the only indication for atrial fibrillation ablation in recent guidelines has been the presence of symptoms. However, up to 40% of an atrial fibrillation population may be asymptomatic. In 2017, in the absence of randomized studies, are there nevertheless data that support atrial fibrillation ablation in asymptomatic patients?

New formula for defining "normal" and "prolonged" QT in patients with bundle branch block.

OBJECTIVES: To predict the QT interval in the presence of normal QRS for patients with left bundle branch block (LBBB). BACKGROUND: There is no acceptable method for simple and reliable QT correction for patients with bundle branch block (BBB). METHODS: We measured the QT interval in patients with new onset LBBB who had a recent electrocardiogram with narrow QRS for comparison. 48 patients who developed in-hospital LBBB were studied. Patients who had similar heart rate before and after LBBB were included. We used linear regression, the Bogossian method, and our new fixed QRS replacement method to evaluate the most reliable correction method. RESULTS: JTc (QTc-QRS) interval was preserved before and after LBBB (328.9 ±â€¯25.4 ms before LBBB vs. 327.3 ms post LBBB (p = 0.550). Mean predicted preLBBB QTc difference was 1.3 ms, -21.3 ms and 1.6 ms for the three methods respectively (p < 0.001 for Bogossian comparison with the other methods). Coefficients of correlation (R) between actual preLBBB QTc with predicted preLBBB QTc were 0.707, 0.683 and 0.665 respectively (p > 0.3 for R comparisons between all methods). The average absolute difference in preLBBB QTc was 15.5 ms and 16.7 ms for the regression and fixed-gender methods (p value between the two = 0.321) and 25.5 ms for the Bogossian method, which was found to be significantly underperforming. CONCLUSIONS: In patients with LBBB, replacing of the QRS duration after deriving the QTc interval with a fixed value of 88 ms for female and 95 ms for male provides a simple and reliable method for predicting the QTc before the development of LBBB.

Arrhythmic Events in Brugada Syndrome: A Nationwide Israeli Survey of the Clinical Characteristics, Treatment; and Long-Term Follow-up (ISRABRU-VF).

BACKGROUND: Limited information exists about detailed clinical characteristics and management of the small subset of Brugada syndrome (BrS) patients who had an arrhythmic event (AE). OBJECTIVES: To conduct the first nationwide survey focused on BrS patients with documented AE. METHODS: Israeli electrophysiology units participated if they had treated BrS patients who had cardiac arrest (CA) (lethal/aborted; group 1) or experienced appropriate therapy for tachyarrhythmias after prophylactic implantable cardioverter defibrillator (ICD) implantation (group 2). RESULTS: The cohort comprised 31 patients: 25 in group 1, 6 in group 2. Group 1: 96% male, mean CA age 38 years (range 13-84). Nine patients (36%) presented with arrhythmic storm and three had a lethal outcome; 17 (68%) had spontaneous type 1 Brugada electrocardiography (ECG). An electrophysiology study (EPS) was performed on 11 patients with inducible ventricular fibrillation (VF) in 10, which was prevented by quinidine in 9/10 patients. During follow-up (143 ± 119 months) eight patients experienced appropriate shocks, none while on quinidine. Group 2: all male, age 30-53 years; 4/6 patients had familial history of sudden death age < 50 years. Five patients had spontaneous type 1 Brugada ECG and four were asymptomatic at ICD implantation. EPS was performed in four patients with inducible VF in three. During long-term follow-up, five patients received ≥ 1 appropriate shocks, one had ATP for sustained VT (none taking quinidine). No AE recurred in patients subsequently treated with quinidine. CONCLUSIONS: CA from BrS is apparently a rare occurrence on a national scale and no AE occurred in any patient treated with quinidine.