Defining the blanking period, using continuous ECG monitoring, after cryoballoon pulmonary vein isolation.

BACKGROUND: Pulmonary vein isolation (PVI) is the cornerstone of atrial fibrillation (AF) ablation. A blanking period (BP) of 3 months is used in clinical trials and practice. However, the optimal BP duration after PVI remains undefined. OBJECTIVE: The aim of this study was to objectively define, using continuous monitoring by an implantable loop recorder, the optimal BP duration after cryoballoon PVI. METHODS: We enrolled consecutive patients who had cryoballoon PVI and an implantable loop recorder. We determined the time of the last confirmed episode of AF within the blanking period. This was then correlated with AF recurrence in the first year after ablation. RESULTS: There were 210 patients (66 ± 9 years; 138 [66%] male; 116 [55%] paroxysmal AF; CHA2DS2-VASc score, 2.5 ± 1.6). We defined 4 distinct groups based on the last AF episode within the BP: no AF days 0-90 (n = 96 [46%]) and last AF 0-30 days (n = 46 [22%]), 31-60 days (n = 18 [9%]), and 61-90 days (n = 50 [24%]). After the 3-month BP, 101 (48%) patients had AF recurrence at 160 ± 86 days. Compared with patients with no AF in the BP, those with recurrent AF and AF burden >0% 30 days after ablation had a significantly greater AF recurrence during long-term follow-up (P = .001). CONCLUSION: Our data show that the approximately one-third of patients in whom AF occurs and who have a burden of >0% after the first month that follows PVI are at significantly higher risk of long-term recurrent AF. We therefore suggest that the blanking period be limited to a month after cryoballoon PVI.

Intermediate-term outcome of cryoballoon ablation of persistent atrial fibrillation and improvements in quality of life of patients.

BACKGROUND: Clinical outcome of pulmonary vein isolation (PVI) has been predominantly focused on the reoccurrence of atrial fibrillation (AF) and the maintenance of sinus rhythm. However, there has been a limited intermediate follow-up on health-related quality-of-life (HRQoL) of patients. Given the relatively high recurrence rate of persistent AF after PVI treatment, it is important to follow up with clinical outcomes on symptom improvement as well as health-related quality-of-life. This study was designed to investigate the recurrence rate of atrial tachyarrhythmia (ATa), AF-related symptoms and HRQoL after PVI in patients with persistent AF who were treated with the second generation cryoballoon. METHODS: Total 148 patients participated in the study who were treated by PVI between 2013 and 2017 for persistent AF. All patients visited the out-patient clinic 2-5 years after PVI. During this visit all patients filled-out an AF Effect on Quality-of-life (AFEQT) questionnaire and a seven-day Holter was applied. All rhythm recordings acquired post ablation were collected and analysed, and the modified European Heart Rhythm Association score (mEHRA) scores were calculated before and after the ablation. RESULTS: The average age of patients was 63±9 year old and 44 (27.9%) were female. Mean CHA2DS2 VASc score was 1.9±1.4, and moderate and severe left atrial (LA) dilation was present in 53 (36.1%) and 15 (10.2%) patients, respectively. After a follow-up of 3.7±1.0 years, 81 (54.7%) patients had an ATa recurrence and 35 (23.6%) patients underwent a repeat LA ablation. However, the mEHRA score significantly improved in 80.4% of the patients (p <0.001), with the median overall AFEQT score of 88.9 [70.4-97.2]. CONCLUSIONS: There is a considerable ATa recurrence rate after PVI of persistent AF at intermediate-term follow-up. However, our data indicate that PVI significantly improved AF-related symptoms and resulted in a high HRQoL for 2-5 years in patients with persistent AF.

Comparison of catheter and surgical ablation of atrial fibrillation: A systemic review and meta-analysis of randomized trials.

OBJECTIVE: To compare both the beneficial and adverse effects of catheter ablation (CA) and surgical ablation (SA) on patients with atrial fibrillation (AF). METHODS: We searched MEDLINE and 4 additional databases for randomized controlled trials that compared CA with SA. Following data extraction, we conducted a meta-analysis to estimate the efficacy and safety of CA relative to SA. The primary end point of this study was the absence of AF during a 12-month follow-up period without the use of antiarrhythmic drugs. RESULTS: Seven trials comparing SA with CA met the inclusion criteria for efficacy outcome assessments. Following the meta-analysis, we obtained a summary odds ratio (OR) of achieving success 1 year after CA relative to SA was 0.37:1 (95% confidence interval [CI], 0.20-0.69). The result was robust in the subgroup analysis. CA was associated with a greater incidence of femoral vascular complications (OR, 5.81; 95% CI, 1.03-32.71), but a lower incidence of pneumothorax (OR, 0.09; 95% CI, 0.01-0.74) than SA. Statistically significant differences in the other safety outcomes were not observed between CA and SA. CONCLUSIONS: SA confers a moderate advantage over CA in 1-year efficacy outcomes and may be safely performed by experienced surgeons.

The isthmus characteristics of scar-related macroreentrant atrial tachycardia in patients with and without cardiac surgery.

INTRODUCTION: Identifying the critical isthmus (CI) in scar-related macroreentrant atrial tachycardia (AT) is challenging, especially for patients with cardiac surgery. We aimed to investigate the electrophysiological characteristics of scar-related macroreentrant ATs in patients with and without cardiac surgery. METHODS: A prospective study of 31 patients (mean age 59.4 ± 9.81 years old) with scar-related macroreentrant ATs were enrolled for investigation of substrate properties. Patients were categorized into the nonsurgery (n = 18) and surgery group (n = 13). The CIs were defined by concealed entrainment, conduction velocity less than 0.3 m/s, and the presence of local fractionated electrograms. RESULTS: Among the 31 patients, a total of 65 reentrant circuits and 76 CIs were identified on the coherent map. The scar in the surgical group is larger than the nonsurgical group (18.81 ± 9.22 vs. 10.23 ± 5.34%, p = .016). The CIs in surgical group have longer CI length (15.27 ± 4.89 vs. 11.20 ± 2.96 mm, p = .004), slower conduction velocity (0.46 ± 0.19 vs. 0.69 ± 0.14 m/s, p < .001), and longer total activation time (45.34 ± 9.04 vs. 38.24 ± 8.41%, p = .016) than those in the nonsurgical group. After ablation, 93.54% of patients remained in sinus rhythm during a follow-up of 182 ± 19 days. CONCLUSION: The characteristics of the isthmus in macroreentrant AT are diverse, especially for surgical scar-related AT. The identification of CIs can facilitate the successful ablation of scar-related ATs.

Comparison of electrophysiological characteristics of right- and left-sided Mahaim-type accessory pathways.

AIMS: Mahaim-type accessory pathways (MAPs) are generally right-sided due to the embryological differentiation, but left-sided localization is also possible. This study aims to compare the clinical and electrophysiological characteristics of right- and left-sided MAPs. METHODS: Of 251 patients diagnosed with AP by electrophysiological study between November 2015 and February 2020, 12 patients with MAP were included (right sided n = 8, left sided n = 4). MAP was diagnosed if; (1) no retrograde conduction; (2) anterograde decremental conduction; (3) adenosine sensitivity; and (4) Mahaim potential at successful ablation site were present. RESULTS: Ten of twelve MAPs were clustered on the lateral walls of the mitral (n = 3, 75%) and tricuspid annuli (n = 7, 87.5%). Right-sided MAPs were mostly long pathways extending toward the conduction system whereas left-sided MAPs were short extending toward the neighboring myocardium. For right- and left-sided APs, the median QRS times were 129 and 156 ms (p = .042), the median VAbl -RVApex intervals were -12 and 64 ms (p = .007), the median QRS-V(His) intervals were 16 and 86 ms (p = .120), and the median VAbl -QRS interval was -8 and 12 ms (p = .017), respectively. Coexistence of dual atrioventricular node physiology was observed only in right-sided APs (n = 3, 37.5%). CONCLUSION: MAPs are more typically located on the right but may rarely be seen on the left. Catheter ablation was associated with high success without complications.

Comparative effects of intensive ganglionated plexus ablation in treating paroxysmal atrial fibrillation and vasovagal syncope.

BACKGROUND: Ganglionated plexus (GP) ablation is used to treat atrial fibrillation (AF) and vasovagal syncope (VVS). However, the comparative effects of GP ablation in treating paroxysmal atrial fibrillation (PAF) and VVS have not been well studied. OBJECTIVE: The purpose of this study was to investigate the effects of intensive GP ablation on PAF and VVS. METHODS: PAF and VVS patients were enrolled in this study. Pulmonary vein isolation (PVI) was performed in the PAF group, and additional ablation was performed at GP sites. Anatomic ablation of left atrial GPs was performed in the VVS group. The primary endpoint was freedom from AF or other sustained atrial tachycardia and syncope recurrence. RESULTS: A total of 195 patients were enrolled: 146 patients with PAF, including eight patients with combined VVS (PAF group), and 49 patients with VVS (VVS group). Vasovagal response (VR) was achieved in 78 (53.4%) patients in the PAF group and 48 patients (98.0%) in the VVS group (P < .05). During the 17.8 ± 10.5 (range, 3-42) month follow-up, 126 (86.3%) patients were free of AF in the PAF group, and 45 (91.8%) patients in the VVS group had no syncope recurrence and significantly improved symptoms. CONCLUSIONS: Anatomically guided intensive GP ablation showed efficient clinical outcomes for both groups of patients. Compared with PAF patients, VVS patients had more VR during ablation in the left atrium. Furthermore, VR during ablation indicated a better prognosis in PAF patients.

How valvular calcification can affect the outcomes of transcatheter aortic valve implantation.

Introduction In transcatheter aortic valve implantation (TAVI), assessment of aortic valve calcification is not as standardized as aortic annulus measurement. Aortic valve calcification is important for stable anchoring of the prosthesis to the aortic annulus. However, excessive aortic valve calcification is related to procedural complications. Areas covered This review covers the methods to assess aortic valve calcification and the implications of aortic valve calcium burden for TAVI outcomes. We performed a systematic review of the literature in Pubmed and secondary sources. Furthermore, future perspectives on how to integrate aortic valve calcification assessment in the management of patients with aortic stenosis is discussed. Expert opinion Thorough assessment of the aortic valve and aortic root components including aortic valve calcification is key in the planning of TAVI. Aortic valve calcification load, location and extension are important contributors to paravalvular regurgitation. Asymmetric calcification burden with greater calcification of the left-coronary cusp related to higher need of permanent pacemaker implantation. Patients with moderate and severe left ventricular outflow tract/subannular calcification are more susceptible to aortic annular rupture. Periprocedural dislodgement of calcium form cusps and commissures is one of the main reasons of coronary artery ostial occlusion during transcatheter aortic valve implantation. Abbreviations Ao, aorta; LA, left atrium; LAA, left atrial appendage; LV, left ventricle; LVOT, left ventricular outflow tract; THV, transcatheter heart valve.

Year in Review in Cardiac Electrophysiology.

In the past year, there have been numerous advances in our understanding of arrhythmia mechanisms, diagnosis, and new therapies. We have seen advances in basic cardiac electrophysiology with data suggesting that secretoneurin may be a biomarker for patients at risk of ventricular arrhythmias, and we have learned of the potential role of an NPR-C (natriuretic peptide receptor-C) in atrial fibrosis and the role of an atrial specific 2-pore potassium channel TASK-1 as a therapeutic target for atrial fibrillation. We have seen studies demonstrating the role of sensory neurons in sleep apnea-related atrial fibrillation and the association between bariatric surgery and atrial fibrillation ablation outcomes. Artificial intelligence applied to electrocardiography has yielded estimates of age, sex, and overall health. We have seen new tools for collection of patient-centered outcomes following catheter ablation. There have been significant advances in the ability to identify ventricular tachycardia termination sites through high-density mapping of deceleration zones. We have learned that right ventricular dysfunction may be a predictor of survival benefit after implantable cardioverter-defibrillator implantation in patients with nonischemic cardiomyopathy. We have seen further insights into the role of His bundle pacing on improving outcomes. As our understanding of cardiac laminopathies advances, we may have new tools to predict arrhythmic event rates in gene carriers. Finally, we have seen numerous advances in the treatment of arrhythmias in patients with congenital heart disease.

Transcatheter radiofrequency ablation using near-zero fluoroscopy in children with fascicular ventricular tachycardia: a single-centre experience.

OBJECTIVE: Fascicular tachycardia is a common form of sustained idiopathic left ventricular tachycardia. This study aimed to achieve successful results with catheter ablation procedures performed through three-dimensional electroanatomic mapping using near-zero fluoroscopy in fascicular tachycardia patients. METHODS AND RESULTS: In this study, we included 33 consecutive children with fascicular tachycardia, for whom we performed a transcatheter radiofrequency ablation procedure using the EnSite® system. Activation mapping was performed in all patients during tachycardia, and the earliest presystolic purkinje potentials were the target site for radiofrequency lesions. RESULTS: Twenty-five patients were male, and eight were female. The mean weight of the patients was 39.6 ± 10.4 kg, and the mean age was 13.6 ± 2.5 years. The mean procedure time was 121.3 ± 44.3 minutes. The mean follow-up period was 18.4 ± 6.5 months. No fluoroscopy was needed in 30 patients. The mean fluoroscopy time in the remaining patients was 166.6 ± 80 seconds. All of the patients had left posterior fascicular tachycardia except for one who had left anterior fascicular tachycardia. The acute success rate was perfect (100%). No patients developed left bundle branch block or complete atrioventricular block. Recurrence developed in one patient. CONCLUSION: We suggest that radiofrequency ablations via an electroanatomic mapping system are quite safe and effective, with high success rates in paediatric patients with fascicular tachycardia. This method has the advantage of avoiding ionising radiation exposure for both the patient and operator, thus reducing the lifetime risk of malignancy in the paediatric population.

The Efficacy and Safety of Radiofrequency Catheter Ablation for Cardiac Arrhythmias in Pediatric Patients.

BACKGROUND: We aimed to evaluate the acute and long-term efficacy and safety of radiofrequency catheter ablation (RFCA) in Chinese pediatric patients with arrhythmias. METHODS: We gathered clinical data from pediatric patients who underwent RFCA in several large medical centers in China between 2000 and 2019. RESULTS: A total of 4,622 different substrates in 4,622 patients were ablated, with the majority consisting of supraventricular tachycardia (N = 3,831, 82.9%) and ventricular arrhythmias (VAs) (N = 791, 17.1%). The majority of supraventricular tachycardia (SVT) consists of atrioventricular reentry tachycardia (AVRT) (N = 2,492, 65.0%) and atrioventricular nodal reentry tachycardia (AVNRT) (N = 1,075, 28.1%). The accessory pathway location was left-sided in 1,237(49.7%) and right-sided in 1,251(50.3%) of the 2,488 pathways. The VAs consist of premature ventricular contraction (PVC) (N = 597, 75.5%) and ventricular tachycardia (VT) (N = 194, 24.5%). The duration of follow-up ranged from 1 month to 1 year. The overall acute/long-term success rate of RFCA was 96.4%/92.2%, AVRT 97.8%/93.5%, AVRT 99.3%/95.0, PVC 98.1%/92.2%, and VT 81.3%/75.2%. Serious complications occurred in 29 patients (0.6%). CONCLUSIONS: This present study shows that RFCA is effective and safe in the treatment of arrhythmias in Chinese pediatric patients, with a high success rate exceeding 90%, low recurrence rate, and significantly reduces the risk of complications. The success rate of left-sided pathway ablation is slightly higher than that of the right-sided pathway. There is no difference in the success rate of RFCA among children of different ages. It could be the first-line therapy in the majority of pediatric patients with SVT and/or VAs.

Slow-pathway visualization by using voltage-time relationship: A novel technique for identification and fluoroless ablation of atrioventricular nodal reentrant tachycardia.

BACKGROUND: Atrioventricular nodal reentrant tachycardia (AVNRT) is treatable by catheter ablation. Advances in mapping-system technology permit fluoroless workflow during ablations. As national practice trends toward fluoroless approaches, easily obtained, reproducible methods of slow-pathway identification, and ablation become increasingly important. We present a novel method of slow-pathway identification and initial ablation results from this method. METHODS AND RESULTS: We examined AVNRT ablations performed at our institution over a 12-month period. In these cases, the site of the slow pathway was predicted by latest activation in the inferior triangle of Koch during sinus rhythm. Ablation was performed in this region. Proximity of the predicted site to the successful ablation location, complication rates, and patient outcomes were recorded. Junctional rhythm was seen in 40/41 ablations (98%) at the predicted site (mean, 1.3 lesions and median, 1 lesion per case). One lesion was defined as 5 mm of ablation. The initial ablation was successful in 39/41 cases (95%); in two cases, greater or equal to 2 echo beats were detected after the initial ablation, necessitating further lesion expansion. In 8/41 cases (20%), greater than one lesion was placed during initial ablation before attempted reinduction. Complications included one transient heart block and one transient PR prolongation. During follow-up (median, day 51), one patient had lower-extremity deep-vein thrombosis and pulmonary embolus, and one had a lower-extremity superficial venous thrombosis. There was one tachycardia recurrence, which prompted a redo ablation. CONCLUSIONS: Mapping-system detection of late-activation, low-amplitude voltage during sinus rhythm provides an objective, and fluoroless means of identifying the slow pathway in typical AVNRT.

Contact-Force-Sensing-Based Radiofrequency Catheter Ablation in Paroxysmal Supraventricular Tachycardias (COBRA-PATH): a randomized controlled trial.

BACKGROUND: Multiple studies have demonstrated the importance of adequate catheter-tissue contact in the creation of effective lesions during radiofrequency catheter ablation. The development of contact force (CF)-sensing catheters has contributed significantly to improve clinical outcomes in atrial fibrillation. However, CF-sensing technology is not used in the ablation of paroxysmal supraventricular tachycardia (PSVT). The possible reason for this is that PSVT ablation with the conventional approach (i.e. nonirrigated, non-CF-sensing catheters) is considered a relatively low-risk procedure with fairly high success rates (short and long term). The aim of this study is to determine whether CF sensing can further improve the outcomes of PSVT ablation. METHODS/DESIGN: The COBRA-PATH study is a single-center, two-armed, randomized controlled trial. Patients without structural heart disease being referred for electrophysiology study, because of PSVT and potential treatment with radiofrequency (RF) catheter ablation, will be randomly assigned to either manual ablation with standard nonirrigated ablation catheters or manual ablation with an open-irrigated ablation catheter equipped with CF sensing (used in a virtual nonirrigated modus). The primary study endpoint is the difference in the number of RF applications during the ablation of atrioventricular nodal re-entry tachycardia, and that of Wolff-Parkinson-White syndrome and atrioventricular re-entrant tachycardia. Secondary outcome parameters include acute and long-term procedural success rates, overall duration of RF applications, procedure/fluoroscopy durations and safety parameters. DISCUSSION: We expect to see a reduced number/duration of RF applications required to achieve effective lesion creation, and consequently a decrease in total procedure/fluoroscopy times. Although a significant improvement in procedural success rates (acute/long term) might not be feasible to demonstrate (given the relatively high success rate of the standard ablation method), the possible decrease in procedure duration and the consequential reduction of radiation exposure has important clinical implications for both operators and patients undergoing the procedure. TRIAL REGISTRATION: ClinicalTrials, NCT04078685. Retrospectively registered on 2 September 2019.

Outcomes of convergent atrial fibrillation ablation with continuous rhythm monitoring.

BACKGROUND: Outcomes of catheter ablation for persistent atrial fibrillation (PeAF) are suboptimal. The convergent procedure (CP) may offer improved efficacy by combining endocardial and epicardial ablation. METHODS: We reviewed 113 consecutive patients undergoing the CP at our institution. The cohort was divided into two groups based on the presence (n = 92) or absence (n = 21) of continuous rhythm monitoring (CM) following the CP. Outcomes were reported in two ways. First, using a conventional definition of any atrial fibrillation/atrial tachycardia (AF/AT) recurrence lasting >30 seconds, after a 90 day blanking period. Second, by determining AF/AT burden at relevant time points in the group with CM. RESULTS: Across the entire cohort, 88% had either persistent or long-standing persistent AF, mean duration of AF diagnosis before the CP was 5.1 ± 4.6 years, 45% had undergone at least one prior AF ablation, 31% had impaired left ventricle ejection fraction and 62% met criteria for moderate or severe left atrial enlargement. Mean duration of follow-up after the CP was 501 ± 355 days. In the entire cohort, survival free from any AF/AT episode >30 seconds at 12 months after the blanking period was 53%. However, among those in the CM group who experienced recurrences, mean burden of AF/AT was generally very low (<5%) and remained stable over the duration of follow-up. Ten patients (9%) required elective cardioversion outside the 90 day blanking period, 11 patients (9.7%) underwent repeat ablation at a mean of 229 ± 178 days post-CP and 64% were off AADs at the last follow-up. Procedural complications decreased significantly following the transition from transdiaphragmatic to sub-xiphoid surgical access: 23% versus 3.8% (P = .005) CONCLUSIONS: In a large, consecutive series of patients with predominantly PeAF, the CP was capable of reducing AF burden to very low levels (generally <5%), which appeared durable over time. Complication rates associated with the CP decreased significantly with the transition from transdiaphragmatic to sub-xiphoid surgical access. Future trials will be necessary to determine which patients are most likely to benefit from the convergent approach.

Cardiac anatomy pertinent to the catheter and surgical treatment of atrial fibrillation.

In the 1980s when surgery was first introduced for the treatment of atrial fibrillation (AF), one would often hear comments like "Every little old grandmother has AF. Why on earth would you operate on someone like that?".

Robotics for catheter ablation of cardiac arrhythmias: Current technologies and practical approaches.

Robotic technology has emerged as an important tool to facilitate catheter ablation of arrhythmias. Robotic cardiac electrophysiology technology includes remote magnetic navigation and manual robotic navigation. Robotics can confer advantages with respect to ease of catheter manipulation in anatomically challenging spaces, minimization of fluoroscopic exposure to both patients and operators, and reduction in operator fatigue. This review provides a comprehensive summary of robotic electrophysiology technology, its practical applications and its safety and efficacy for targeting cardiac arrhythmias.

The convergent procedure versus catheter ablation alone in longstanding persistent atrial fibrillation: A single centre, propensity-matched cohort study.

BACKGROUND: Maintenance of sinus rhythm is challenging in patients with longstanding persistent atrial fibrillation (PeAF). Minimally invasive surgical AF ablation may improve outcomes when combined with catheter ablation (the 'convergent' procedure). This study evaluates the safety and efficacy of the convergent procedure versus catheter ablation alone in longstanding PeAF. METHODS: 43 consecutive patients with longstanding PeAF underwent subxiphoid endoscopic ablation of the posterior left atrium followed by catheter ablation from 2013 to 2018. The primary outcome was AF-free survival at 12 months; secondary outcomes included change in EHRA class, echocardiographic data, procedural complications, freedom from anti-arrhythmic drugs (AADs), and long term arrhythmia-free survival. Outcomes were compared with a matched group of 43 patients who underwent catheter ablation alone. Both groups underwent multiple catheter ablations as required. Baseline characteristics were similar between groups. RESULTS: After 12 months, the convergent procedure was associated with increased AF-free survival on AADs (60.5% versus 25.6%, p = .002) and off AADs (37.2% versus 13.9%, p = .025), versus catheter ablation. Allowing for multiple procedures, after 30.5 ±â€¯13.3 months' follow-up the convergent procedure was associated with increased arrhythmia-free survival on AADs (58.1% versus 30.2%, p = .016) and off AADs (32.5% versus 11.6%, p = .036) versus catheter ablation. There were more complications in the convergent procedure group (11.6% versus 2.3%, p = .2). Multivariate analysis identified only the convergent procedure (OR 3.06 (1.23-7.6), p = .017) as predictive of arrhythmia-free survival long term. CONCLUSIONS: In longstanding PeAF, the convergent procedure is associated with improved arrhythmia-free survival versus catheter ablation alone. Complication rates are significant but have been shown to depreciate with experience.

Treatment of atypical pacemaker-mediated tachycardia with ablation of the retrograde atrioventricular nodal pathway.

A 25-year-old runner received a single-lead, VDD pacemaker after ablation of AV nodal reentrant tachycardia complicated by intermittent AV block. The rate-adaptive AV delay algorithm (RAAV), which shortens the sensed AV interval (SAV) at faster atrial rates, was programmed to provide a physiologic SAV with exercise. She developed repetitive, atypical, long-RP pacemaker-mediated tachycardia (PMT) because the RAAV shortened the antegrade SAV and retrograde conduction occurred over the slow AV nodal pathway. PMT was refractory to usual programming solutions. Using high-density electroanatomic mapping, we were able to ablate the retrograde limb of PMT without further damaging AV conduction.

Atrial fibrillation in Brugada syndrome: Current perspectives.

The incidence of atrial fibrillation (AF) in Brugada syndrome (BrS) has been reported at between 9% and 53% by different series, but the true prevalence is unknown. However, AF may be the presenting feature in some patients. The underlying mechanisms for AF may be a combination of multiple factors, genetic or acquired, that may impact upon autonomic function, atrial structure, and conduction velocities or other unknown factors. The presence of AF has been associated with a more malignant course, with a greater incidence of syncope and ventricular arrhythmias, thus acting as marker of more advanced disease. Regarding the management of patients with AF, antiarrhythmic drugs effective in preventing malignant arrhythmias in BrS such as quinidine or invasive treatment with pulmonary vein isolation (PVI) may be useful in AF treatment. In this review, we aim to present the current perspectives regarding the genetics, pathophysiology, management, and prognosis of AF in patients with BrS.