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Abstract Cardiac tumors are a rare cause of arrhythmias in clinical practice. They can cause a broad spectrum of arrhythmias, from low‐grade ectopics to incessant ventricular tachycardias, including sudden cardiac arrest. Both primary and secondary cardiac tumors can produce arrhythmias, but not all tumors cause arrhythmias. Although cardiac tumors can cause arrhythmias in fetuses and older adults alike, only specific cardiac tumors are the underlying cause of arrhythmia in different age groups. This article reviews various cardiac tumors that are associated with arrhythmias, their clinical presentations, diagnostic features, and management.

Curative radiofrequency catheter modification of the slow pathway is the recommended therapy for patients suffering from recurrent symptomatic atrioventricular nodal reentry tachycardia. This is usually performed via femoral vein and the inferior vena cava (IVC). Presence of venous occlusion or complex venous anomaly involving the IVC may preclude this approach. Here, we report a case with a complex venous anomaly involving the inferior vena cava, who underwent electrophysiological study and successful radiofrequency ablation by an alternative approach.

Cardiac channelopathies are a group of heritable disorders that affect the heart's electrical activity due to genetic variations present in genes coding for ion channels. With the advent of new sequencing technologies, molecular diagnosis of these disorders in patients has paved the way for early identification, therapeutic management and family screening. The objective of this retrospective study was to understand the efficacy of whole-genome sequencing in diagnosing patients with suspected cardiac channelopathies who were reported negative after whole exome sequencing and analysis. We employed a 3-tier analysis approach to identify nonsynonymous variations and loss-of-function variations missed by exome sequencing, and structural variations that are better resolved only by sequencing whole genomes. By performing whole genome sequencing and analyzing 25 exome-negative cardiac channelopathy patients, we identified 3 pathogenic variations. These include a heterozygous likely pathogenic nonsynonymous variation, CACNA1C:NM_000719:exon19:c.C2570G:p. P857R, which causes autosomal dominant long QT syndrome in the absence of Timothy syndrome, a heterozygous loss-of-function variation CASQ2:NM_001232.4:c.420+2T>C classified as pathogenic, and a 9.2 kb structural variation that spans exon 2 of the KCNQ1 gene, which is likely to cause Jervell-Lange-Nielssen syndrome. In addition, we also identified a loss-of-function variation and 16 structural variations of unknown significance (VUS). Further studies are required to elucidate the role of these identified VUS in gene regulation and decipher the underlying genetic and molecular mechanisms of these disorders. Our present study serves as a pilot for understanding the utility of WGS over clinical exomes in diagnosing cardiac channelopathy disorders., (© 2024. The Author(s), under exclusive licence to The Japan Society of Human Genetics.)

Background: Bilateral cardiac sympathetic denervation (BCSD) for refractory life-threatening ventricular arrhythmias is a neuromodulatory intervention targeting sympathetically driven focal or re-entrant ventricular arrhythmias., Objectives: This study sought to provide a more complete and successful option for intervention in patients in whom premature ventricular contraction (PVC) ablation is not feasible or has been unsuccessful., Methods: A total of 43 patients with >5% PVC burden and concomitant nonischemic cardiomyopathy (NICM) who previously failed medical and ablation therapies were referred for BCSD. All patients underwent bilateral video-assisted thoracoscopic surgical approach with T1-T4 sympathectomy. Primary effectiveness endpoints were postprocedural PVC burden resolution, improvement in left ventricular ejection fraction (LVEF), and cessation of antiarrhythmic drugs (AADs). Safety endpoints included peri- and postprocedural complications. Outcomes were assessed over a 1-year follow-up period., Results: Among the 43 patients who underwent BCSD, the mean age was 52.3 ± 14.7 years, 69.8% of whom were male patients. Presenting mean LVEF was 38.7% ± 7.8%, and PVC burden was 23.7% ± 9.9%. There were significant reductions in PVC burden postprocedurally (1.3% ± 1.1% post-BCSD, compared with 23.7% ± 9.9% pre-BCSD, P < 0.001) and improvements in LVEF (46.3% ± 9.5% post-BCSD, compared with 38.7% ± 7.8% pre-BCSD, P < 0.001). The rate of ICD therapies decreased from 81.4% (n = 35) to 11.6% (n = 5) (P < 0.001), leading to a significant reduction in use of AADs (100.0% to 11.6%, P < 0.001) and improvement in mean NYHA functional class (2.5 ± 0.5 to 1.4 ± 0.2, P < 0.001). Major intraoperative complications were seen in 4.7% of patients (hemothorax and chylothorax). Of the patients, 81.4% (n = 35) experienced no mortality or major complications over a 1-year follow-up period, with the remaining still within their first year postprocedure., Conclusions: BCSD is effective for the management of refractory PVCs and ventricular tachycardia who have failed previous ablation therapy., Competing Interests: Funding Support and Author Disclosures Dr Lakkireddy has served as consultant for Abbott, Acutus, AltaThera, Boston Scientific, Biosense Webster, and Medtronic. Dr Pothineni has received speaking honoraria from Boston Scientific. Dr Gopinathannair has served as consultant and has received honoraria from Abbott Medical, and Sanofi; and has served on an advisory board for Pacemate. Dr Natale has served as consultants for Medtronic, Abbott, Biosense Webster, Boston Scientific, and Acutus. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024. Published by Elsevier Inc.)

Surgical aortic valve replacement (SAVR) and transcatheter aortic valve implantation (TAVI) in aortic stenosis are associated with arrhythmic complications that can require cardiac implantable electronic device (CIED) implantation, but impact on healthcare-associated cost (HAC) and length of stay (LOS) are unknown. This study aimed to assess differences among SAVR/TAVI patients with CIED implantation on HAC and LOS. Patients hospitalized for SAVR or TAVI between 2011 and 2017 on the National Inpatient Sample database were identified and stratified according to presence/type of CIED implantation. During this period, 95,262 patients were identified; 6,435 (6.8%) patients received CIED (median [interquartile range] age: 74.0 [66.0 to 82.0] years). The median adjusted HAC was $44,271 and LOS was 6 days. CIED implantation was associated with longer LOS and higher adjusted HAC in patients with SAVR and TAVI (p <0.0001). Patients with in-hospital death and complications because of SAVR or TAVI had longer preceding in-hospital days of admission. Male patients admitted to small hospitals and the West region had the highest HAC. In conclusion, CIED implantation for arrhythmias results in higher HAC and longer LOS in patients with aortic stenosis for both SAVR and TAVI., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: We compared the efficacy and safety of cardioneuroablation (CNA) vs. permanent pacing (PM) for recurrent cardioinhibitory vasovagal syncope (CI-VVS)., Methods: One hundred sixty-two patients (CNA = 61, PM = 101), age 36 + 11 years) with syncope frequency of 6.7 ± 3.9/year were included in this multicenter study. All patients with CNA were provided by a single center, while patients with PM were provided by 4 other centers. In the CNA arm, an electroanatomic mapping guided approach was used to detect and ablate ganglionated plexus sites. Dual chamber rate drop response (RDR) or close loop stimulation (CLS) transvenous and leadless pacemakers were implanted using standard technique. The primary endpoint was freedom from syncope., Results: Of 101 patients in the PM group, 39 received dual-chamber pacemaker implants with the CLS algorithm, 38 received dual-chamber pacemakers with the RDR algorithm, and 24 received a leadless pacemaker. At 1-year follow-up, 97% and 89% in the CNA and PM group met the primary endpoint (adjusted HR = 0.27, 95% CI 0.06-1.24, p = 0.09). No significant differences in adverse events were noted between groups. There was no significant association between age (HR:1.01, 95% CI 0.96-1.06, p = 0.655), sex (HR:1.15, 95% CI 0.38-3.51, p = 0.809), and syncope frequency in the past year (HR:1.10, 95% CI 0.97-1.25, p = 0.122) and the primary outcome in univariable analyses., Conclusions: After adjustment for patient characteristics, the medium-term syncope recurrence risk of CI-VVS patients who underwent CNA was similar to that of a population of patients undergoing pacemaker implantation with a similar safety profile., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Rheumatic heart disease (RHD) is the underlying cause of a significant proportion of atrial fibrillation (AF) in the low- and middle-income countries, while nonvalvular AF is the most common cause of AF in high-income countries. RHD is also common among African Americans, migrants, and the indigenous population of high-income countries. The onset of AF in RHD patients is a clinical marker of worse outcomes and is associated with significant morbidity and mortality. Despite RHD being a major cause of morbidity and mortality in the young in many parts of the world, it is often neglected by policymakers, the media, and even the medical fraternity. Stroke risk assessment using various risk scores has not been systematically evaluated in rheumatic AF patients. Rate control may not be ideal for symptom control in rheumatic AF patients considering the young age and an active lifestyle. There is limited information regarding the nonpharmacological management of rheumatic AF. The current management guidelines based on nonvalvular AF do not apply to rheumatic AF patients who are often younger, are women, and have fewer comorbidities. This review critically looks at specific areas such as stroke prevention with reference to direct oral anticoagulants, cardioversion, rate and rhythm control strategies, and the role of nonpharmacological methods in rheumatic AF management. Future recommendations must be cognizant of local health care systems and resourcing considering the geographic distribution of the disease., (© 2022 Heart Rhythm Society. Published by Elsevier Inc.)

Background: Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease with preserved or increased ejection fraction in the absence of secondary causes. Mutations in the sarcomeric protein-encoding genes predominantly cause HCM. However, relatively little is known about the genetic impact of signalling proteins on HCM., Methods and Results: Here, using exome and targeted sequencing methods, we analysed two independent cohorts comprising 401 Indian patients with HCM and 3521 Indian controls. We identified novel variants in ribosomal protein S6 kinase beta-1 ( RPS6KB1 or S6K1 ) gene in two unrelated Indian families as a potential candidate gene for HCM. The two unrelated HCM families had the same heterozygous missense S6K1 variant (p.G47W). In a replication association study, we identified two S6K1 heterozygotes variants (p.Q49K and p.Y62H) in the UK Biobank cardiomyopathy cohort (n=190) compared with matched controls (n=16 479). These variants are neither detected in region-specific controls nor in the human population genome data. Additionally, we observed an S6K1 variant (p.P445S) in an Arab patient with HCM. Functional consequences were evaluated using representative S6K1 mutated proteins compared with wild type in cellular models. The mutated proteins activated the S6K1 and hyperphosphorylated the rpS6 and ERK1/2 signalling cascades, suggesting a gain-of-function effect., Conclusions: Our study demonstrates for the first time that the variants in the S6K1 gene are associated with HCM, and early detection of the S6K1 variant carriers can help to identify family members at risk and subsequent preventive measures. Further screening in patients with HCM with different ethnic populations will establish the specificity and frequency of S6K1 gene variants., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Cardiomyopathy (CMP) is a heritable disorder. Over 50% of cases are gene-elusive on clinical gene panel testing. The contribution of variants in non-coding DNA elements that result in cryptic splicing and regulate gene expression has not been explored. We analyzed whole-genome sequencing (WGS) data in a discovery cohort of 209 pediatric CMP patients and 1953 independent replication genomes and exomes. We searched for protein-coding variants, and non-coding variants predicted to affect the function or expression of genes. Thirty-nine percent of cases harbored pathogenic coding variants in known CMP genes, and 5% harbored high-risk loss-of-function (LoF) variants in additional candidate CMP genes. Fifteen percent harbored high-risk regulatory variants in promoters and enhancers of CMP genes (odds ratio 2.25, p = 6.70 × 10 -7 versus controls). Genes involved in α-dystroglycan glycosylation (FKTN, DTNA) and desmosomal signaling (DSC2, DSG2) were most highly enriched for regulatory variants (odds ratio 6.7-58.1). Functional effects were confirmed in patient myocardium and reporter assays in human cardiomyocytes, and in zebrafish CRISPR knockouts. We provide strong evidence for the genomic contribution of functionally active variants in new genes and in regulatory elements of known CMP genes to early onset CMP., (© 2022. The Author(s).)

Background: The long-term outcomes of patients with congenital and childhood complete atrioventricular block (CCAVB/CAVB) after pacemaker implantation are unclear., Methods: We performed a meta-analysis of all the studies of CCAVB. A systematic search of PubMed and CENTRAL databases from January 1, 1967 to January 31, 2020 was performed. The quality of studies included was critically appraised using the Newcastle-Ottawa scale, and outcome data were analyzed using the restricted maximum likelihood function., Results: Twenty-nine studies were eligible for analysis, with a total of 1553 patients. The all-cause-mortality was 5.7% (95% confidence interval [CI]: 2.5%-9.9%), while pacing-induced cardiomyopathy (PICM) was seen in 3.8% (95% CI: 1.2-7.2). Diagnosis at birth (effect size [ES] [95%CI]: -2.23 [-0.36 to -0.10]; p < .001), presence of congenital heart disease (ES [95%CI]: -0.67 [0.41-0.93]; p < .001), younger age at pacemaker implantation (ES [95%CI]: -0.01 [-0.02 to -0.001]; p = .02), and duration of pacing (ES [95%CI]: -0.03 [-0.05 to -0.003]; p = .03), were associated with an higher mortality on binominal logistic regression. None of the parameters were significant on multivariate analysis., Conclusion: Pooled proportional mortality in patients with CCAVB and CAVB is 5.7% with an infrequent incidence of PICM (3.8%) in the paced patients with AVB suggesting that pacing in these patients is an effective management strategy with a low incidence of long-term side effects. Registry and randomized data can throw additional light regarding the natural history and appropriate management strategy in these patients., (© 2022 Wiley Periodicals LLC.)

Background: Adequate and effective therapy for resistant vasovagal syncope patients is lacking and the benefit of cardioneuroablation (CNA) in this cohort is still debated. The aim of this study is to assess the long-term effect of CNA versus conservative therapy (CT) in a retrospectively followed cohort., Methods: A total of 2874 patients underwent head-up tilt test (HUT) and 554 (19.2 %) were reported as positive, with VASIS type 2B response or > 3 s asystole in 130 patients. After exclusion of 29 patients under 18 years and over 65 years of age, 101 patients were included final analysis. Fifty-one patients (50.4%) underwent CNA and 50 (49.6%) patients received CT. After propensity score matching, 19 pairs of patients were successfully matched. The recurrence rate of syncope was compared between groups., Results: During a median follow-up of 22 months (IQR, 13-35), syncope was seen in 12 (11.8%) cases. In the 19 propensity-matched patients, recurrent syncope was observed in 8 patients in the CT group and in 2 patients in the CNA group, respectively. In mixed effect Cox regression analysis, CNA was associated with less syncope recurrence risk at follow-up (HR 0.23, 95% CI 0.03-0.99, p = 0.049). The 4-year Kaplan-Meier syncope free rate was 0.86 (95% CI, 0.63-1.00) for CNA group and 0.50 (95% CI, 0.30-0.82) for CT group in the matched cohort., Conclusions: In highly selected patients with HUT-induced cardioinhibitory response, CNA is associated with a significant reduction in syncope recurrence during follow-up when compared to CT., (© 2021. Springer Science+Business Media, LLC, part of Springer Nature.)

Background: There is a high incidence of atrial fibrillation (AF) in patients with isolated rheumatic mitral regurgitation (MR). The histopathologic changes in the atria of patients with isolated rheumatic MR with and without AF are unknown., Objectives: We aimed to determine the histological findings in patients with isolated severe rheumatic MR with and without AF., Methods: Patients with severe isolated rheumatic MR undergoing valve replacement surgeries underwent endocardial biopsies from right atrial appendage, left atrial appendage, right free wall, left free wall, left posterior wall, and mitral valve. Group I consisted of patients in sinus rhythm (SR), and Group II included patients with AF. We analyzed and compared these 10 histological features in the biopsies of patients in Groups I and II., Results: Of the 25 patients, 12 were in Group I and 13 in Group II. In Group I, patients had severe myocyte hypertrophy (60% vs. 18%, p = .04) that was significantly more in the right atrium (22.7% vs. 11.4%, p = .059). Interstitial adipose tissue deposition was more common in Group I (30% vs. 25%, p = .06). Interstitial fibrosis was evenly distributed at all sites without significant difference between the two groups. Group II patients had a higher prevalence and severity of vacuolar degeneration (91% vs. 60%, p = .09)., Conclusions: Patients with isolated severe rheumatic MR and AF have more vacuolar degeneration in the atrial tissue. Patients with SR have myocyte hypertrophy and interstitial adipose tissue deposition. Interstitial fibrosis is uniformly distributed in patients in SR and AF., (© 2021 Wiley Periodicals LLC.)

Objectives: The STROKE-VT (Safety and Efficacy of Direct Oral Anticoagulant Versus Aspirin for Reduction of Risk of Cerebrovascular Events in Patients Undergoing Ventricular Tachycardia Ablation) study is a multicenter, randomized controlled trial that examined the differences in cerebrovascular events between direct oral anticoagulant (DOAC) and aspirin (ASA) use postprocedurally in patients who underwent left ventricular arrhythmia (LVA) ablation (ventricular tachycardia [VT] or premature ventricular contraction [PVC]) using radiofrequency ablation (RFA)., Background: There exists limited data regarding antiplatelet or anticoagulation strategy following LVA ablation., Methods: A total of 246 patients scheduled for LVA-RFA were randomized 1:1 postprocedurally to receive DOACs or ASA. The study's primary endpoint was the incidence of stroke or transient ischemic attack (TIA) or asymptomatic cerebrovascular events (ACEs) detected by magnetic resonance imaging at 24 hours and 30 days of follow-up. The secondary endpoints included procedure-related complications (composite of any vascular complication, pericardial complication, heart block, and thromboembolic event, excluding stroke or TIA) and in-hospital mortality., Results: There were no differences between groups regarding baseline and ablation characteristics (except the percentage of patients who underwent VT ablation, rate of amiodarone use, and total RFA time). Postprocedure cerebrovascular events (stroke and TIA) were lower in the DOAC arm versus the ASA arm (0% vs 6.5%; P < 0.001 and 4.9% vs. 18%; P < 0.001, respectively). Patients in the ASA group had more MRI-detected ACEs compared with the DOAC group both at 24-hour (23% vs 12%; P = 0.03) and 30-day (18% vs 6.5%; P = 0.006) follow-up. Acute procedure-related complications and in-hospital mortality were similar between the 2 groups., Conclusions: DOAC use following endocardial and/or epicardial ablation for LVA-RFA was associated with reduced risk of TIA or stroke and asymptomatic MRI-detected cerebrovascular events., Competing Interests: Funding Support and Author Disclosures Dr Lakkireddy has been a consultant for Abbott, Biosense Webster, Boston Scientific, Phillips, Stereotaxis, and Atricure Inc. Dr Gopinathannair has been a consultant for and has received honoraria from Abbott Medical, Biotronik, Boston Scientific, Pfizer, and Zoll Medical; and has been a physician advisor (no compensation) for HealthTrust PG, Altathera, and PaceMate. Dr Natale has been a consultant for Abbott, Biosense Webster, Boston Scientific, and Medtronic. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Conduction system pacing prevents pacing-induced cardiomyopathy, but it can be challenging to perform in patients with congenital heart disease (CHD), and mid/high septal lead implantation is an alternative. This study aimed to assess intraprocedural angiography's utility as a guide for mid/high-septal lead implantation in CHD patients., Methods: The study subjects were CHD patients with Class I/IIa indications for permanent pacemaker implantation. To guide septal lead implantation, we performed an intraprocedural right ventricular angiogram in anteroposterior, 40° left anterior oblique, and 30° right anterior oblique. The primary endpoint was the lead tip in the mid/high septum on computed tomography (CT). The secondary endpoints were complications and systemic ventricular function on follow-up., Results: From January 2008 to December 2018, we enrolled 27 patients (mean age: 30 ± 20 years; M:F 17:10) with CHD (unoperated: 20, operated: 7). The mean paced QRS duration was 131.7 ± 5.8 ms, and CT done in 22/27 patients confirmed the lead tip in the mid-septum in 16, high septum in 5, and apical septum in 1 patient. There were no procedural complications, and during a mean follow-up of 58 ± 35.2 months, there was no significant change in the systemic ventricular ejection fraction (56.4 ± 8.3% vs 53.9 + 5.9%, P  = .08). Two patients with Eisenmenger syndrome died because of refractory heart failure., Conclusions: Intraprocedural angiography is safe and useful to guide mid/high-septal lead implantation in CHD patients. Mid/high septal lead position preserves systemic ventricular function in patients with CHD during medium-term follow-up., Competing Interests: Authors declare no conflict of interests for this article., (© 2021 The Authors. Journal of Arrhythmia published by John Wiley & Sons Australia, Ltd on behalf of Japanese Heart Rhythm Society.)

Aims: Vasovagal syncope (VVS) is a common cardiovascular dysautonomic disorder that significantly impacts health and quality of life (QoL). Yoga has been shown to have a positive influence on cardiovascular autonomics. This study assessed the effectiveness of yoga therapy on the recurrence of VVS and QoL., Methods and Results: We randomized subjects with recurrent reflex VVS (>3 episodes in the past 1 year) and positive head-up tilt test to guideline-directed therapy (Group 1) or yoga therapy (Group 2). Patients in Group 1 were advised guideline-directed treatment and Group 2 was taught yoga by a certified instructor. The primary endpoint was VVS recurrences and QoL. Between June 2015 and February 2017, 97 highly symptomatic VVS patients were randomized (Group 1: 47 and Group 2: 50). The mean age was 33.1 ± 16.6 years, male:female of 40:57, symptom duration of 17.1 ± 20.7 months, with a mean of 6.4 ± 6.1 syncope episodes. Over a follow-up of 14.3 ± 2.1 months Group 2 had significantly lower syncope burden compared with Group 1 at 3 (0.8 ± 0.9 vs. 1.8 ± 1.4, P < 0.001), 6 (1.0 ± 1.2 vs. 3.4 ± 3.0, P < 0.001), and at 12 months (1.1 ± 0.8 vs. 3.8 ± 3.2, P < 0.001). The Syncope functional score questionnaire was significantly lower in Group 2 compared with Group 1 at 3 (31.4 ± 7.2 vs. 64.1 ± 11.5, P < 0.001), 6 (26.4 ± 6.3 vs. 61.4 ± 10.7, P < 0.001), and 12 months (22.2 ± 4.7 vs. 68.3 ± 11.4, P < 0.001)., Conclusion: For patients with recurrent VVS, guided yoga therapy is superior to conventional therapy in reducing symptom burden and improving QoL., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: journals.permissions@oup.com.)

Background: Intracardiac echocardiography (ICE) use during catheter ablation of atrial fibrillation (AF) provides real-time information to guide transseptal access, for monitoring the ablation and recognition of pericardial bleed. We describe trends of ICE use, impact on complications, and its in-hospital outcomes., Methods: The national in-patient sample database was queried from 2001 to 2014 for diagnosis of AF based on ICD-9-CM 427.31 with a catheter ablation procedure code (37.34) in the same hospitalization and its associated complications. ICE was identified using ICD-9-CM procedure code (37.28). Statistical Analysis System (SAS) was used for analysis., Results: There was an estimated total 299,152 patients who underwent AF ablation from 2001 to 2014 of which ICE was used in 46,688 (15.6%) patients. The use of ICE significantly increased from 0.08% in 2001 to 15.7% in 2014. In-hospital mortality was significantly lower in patients in whom ICE was used (0.11% vs 0.54%, p < 0.0001). Complications were 52% lower in procedures using ICE vs without ICE (HR [95%CI]; 0.48 [0.44-0.51]). The rate of cardiac complications was also lower in ICE users (3.67% vs 4.51%; p = 0.025). The use of ICE during AF ablation resulted in significantly higher cost of hospitalization ($98,436 ± 597 vs $81,300 ± 310; p < 0.0001), but this was offset by a decreased length of hospital stay (2.1 ± 0.02 vs 4 ± 0.02 days; p < 0.0001)., Conclusions: The use of ICE during AF ablation has increased over the years and is associated with lower in-hospital mortality and procedural complications, shorter LOS but an increased cost of hospitalization., (© 2020. Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Right ventricular (RV) mid-septal pacing has been proposed as an alternative to RV apical pacing. Fluoroscopic and electrocardiogram criteria are unreliable for predicting the RV mid-septal lead position. This study aimed to define the optimal RV mid-septal pacing site using RV angiography., Methods: We randomized patients undergoing pacemaker implantation (PPM) to the RV angiography-guided group (Group A) or conventional fluoroscopy-guided group (Group F). In Group A, we performed an angiogram in right anterior oblique (RAO 30°), left anterior oblique (LAO 40°), and left lateral (LL) views. We made a 5-segment grid in RAO 30° and LL views and a 3-segment grid in LAO 40° on the angiographic silhouette to define the lead position. Computed tomography (CT) was used to validate the lead tip position in both groups., Results: We enrolled 53 patients (Group A: 26, Group F: 27) with a mean age of 55.9 ± 12.2 years. CT images validated the lead position in the mid-septum (Group A, 23 [88.5%]; Group F, 11 [40.7%], P  = .0003) and anteroseptal (Group A, 3 [11.5%]; Group F, 5 [18.5%], P  = .24). In Group F, the lead was in the anterior wall in 9 patients (33.3%) and the right ventricular outflow tract in 2 (7.4%) patients and none in these two positions in Group A. The lead tip in segment one on the angiographic 5-segment grid in RAO 30° and LL views indicated a mid-septal lead position on CT., Conclusions: RV angiography is safe and may be used to confirm the mid-septal lead position during PPM., Competing Interests: The authors have no conflicts of interest to declare., (© 2021 The Authors. Journal of Arrhythmia published by John Wiley & Sons Australia, Ltd on behalf of Japanese Heart Rhythm Society.)

Introduction: The impact of cardioneuroablation (CNA) on ventricular repolarization by using corrected QT interval (QTc) measurements has been recently demonstrated. The effects of cardiac pacing (CP) on ventricular repolarization have not been studied in patients with vasovagal syncope (VVS). We sought to compare ventricular repolarization effects of CNA (group 1) with CP (group 2) in patients with VVS., Methods: We enrolled 69 patients with age 38 ± 13 years (53.6% male), n = 47 in group 1 and n = 22 in group 2. Clinical diagnosis of cardioinhibitory type was supported by cardiac monitoring or tilt testing. QTc was calculated at baseline (time-1), at 24 h after ablation (time-2), and at 9-12 months (time-3) in the follow-up., Results: In the group 1, from time-1 to time-2, a significant shortening in QTcFredericia (from 403 ± 27 to 382 ± 27 ms, p < 0.0001), QTcFramingham (from 402 ± 27 to 384 ± 27 ms, p < 0.0001), and QTcHodges (from 405 ± 26 to 388 ± 24 ms, p < 0.0001) was observed which remained lower than baseline in time-3 (373 ± 29, 376 ± 27, and 378 ± 27 ms, respectively). Although the difference between measurements in time-1 and time-2 was not statistically significant for QTcBazett, a significant shortening was detected between time-1 and time-3 (from 408 ± 30 to 394 ± 33, p = 0.005). In the group 2, there was no time-based changes on QTc measurements. In the linear mixed model analysis, the longitudinal reduction tendency in the QTcFredericia and QTcFramingham was more pronounced in group 1., Conclusions: Our results demonstrate that CNA reduces QTc levels through neuromodulation effect whereas CP has no effect on ventricular repolarization in patients with VVS., Competing Interests: Declaration of Competing Interest None declared., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Hypertrophic cardiomyopathy (HCM) is a heterogeneous genetic heart muscle disease characterized by hypertrophy with preserved or increased ejection fraction in the absence of secondary causes. However, recent studies have demonstrated that a substantial proportion of individuals with HCM also have comorbid diabetes mellitus (~10%). Whether genetic variants may contribute a combined phenotype of HCM and diabetes mellitus is not known. Here, using next-generation sequencing methods, we identified novel and ultrarare variants in adiponectin receptor 1 ( ADIPOR1 ) as risk factors for HCM. Biochemical studies showed that ADIPOR1 variants dysregulate glucose and lipid metabolism and cause cardiac hypertrophy through the p38/mammalian target of rapamycin and/or extracellular signal-regulated kinase pathways. A transgenic mouse model expressing an ADIPOR1 variant displayed cardiomyopathy that recapitulated the cellular findings, and these features were rescued by rapamycin. Our results provide the first evidence that ADIPOR1 variants can cause HCM and provide new insights into ADIPOR1 regulation., (Copyright © 2021 The Authors, some rights reserved; exclusive licensee American Association for the Advancement of Science. No claim to original U.S. Government Works. Distributed under a Creative Commons Attribution NonCommercial License 4.0 (CC BY-NC).)

Background: Fluoroscopic imaging involves exposure of the patients and the laboratory staff to ionizing radiation. One of the strategies that reduce such exposure in an electrophysiology laboratory is using a three-dimensional electroanatomic mapping (3D EAM) system for performing these procedures. In this analysis, we have analyzed the effect of fluoroscopy frame rate on the radiation exposure and in-hospital outcomes in ablation procedures performed under 3D EAM guidance., Methods: We retrospectively analyzed all the ablation procedures performed under 3D EAM guidance at our institute from September 2015 to December 2018. The procedures were divided into two groups based on whether the procedures were performed before (pre) or after (post) January 26, 2018. After January 2018, fluoroscopy was used at a frame rate of 3.75 frames per second (fps). Radiation exposure indices and in-hospital outcomes were compared between the two groups., Results: Ablation procedures included in the analysis were ventricular arrhythmias (n = 192), atrial flutter (115), atrial tachycardia (AT) (43), and atrial fibrillation (AF) (30). Over the study period, there was a significant reduction in procedure time, fluoroscopy time, dose area product, and effective dose (ED) ( P  < .001). Except for AT and AF ablation procedures, there was a significant reduction in the radiation exposure indices when the "post" group was compared with the "pre" group ( P  ≤ .02). The decrease in the frame rate had no significant effect on in-hospital outcomes., Conclusion: The use of 3D EAM combined with decreasing the fluoroscopy frame rate significantly reduced the total radiation exposure without adversely affecting in-hospital outcomes., Competing Interests: None., (© 2021 The Authors. Journal of Arrhythmia published by John Wiley & Sons Australia, Ltd on behalf of the Japanese Heart Rhythm Society.)

Introduction: Ibutilide is indicated for acute cardioversion of nonvalvular atrial fibrillation (AF). However, its efficacy and safety in the pharmacological cardioversion of rheumatic AF are unknown., Methods: Patients with mild-to-moderate rheumatic mitral valve (MV) disease with symptomatic, paroxysmal, or persistent AF were included in the analysis. Intravenous ibutilide was administered at doses tailored to body weight (0.5-2.0 mg) for over 10 min. The primary end point was efficacy, assessed as the rate of conversion of AF to sinus rhythm. The secondary end point was safety, including arrhythmic events and death within 24 h of drug initiation., Results: From June 2016 to October 2018, 165 patients (94 with mitral stenosis, 23 with mitral regurgitation, 11 with mixed MV disease, and 37 with MV replacement) received ibutilide (mean dose 0.90 ± 0.54 mg). Ibutilide successfully converted AF to sinus rhythm in 127/165 (76.9%) patients, with a conversion time of 7.9 ± 4.1 min. The QTc increased from 419.9 ± 15.8 to 487.5 ± 34 ms after ibutilide administration (p < 0.001). The mean change in QTc after ibutilide administration (∆QTc) was 72.01 ± 36.03. There were no deaths, but 3 patients (1.8%) developed torsades de pointes (TdP) requiring defibrillation 55 ± 37 min after infusion., Conclusion: Ibutilide cardioverted 77% of rheumatic AF to sinus rhythm, indicating its potential as a clinically useful option for pharmacological cardioversion of rheumatic AF. TdP is a potentially serious adverse event that requires careful monitoring., (© 2021 S. Karger AG, Basel.)

Background: Computed tomography (CT) is used for the diagnosis of cardiac perforation (CP) although it has significant limitations. We report our experience with angiography to assist in the diagnosis and management of cardiac perforation during electrophysiology procedures., Methods: Patients with suspected CP after pacemaker lead insertion (temporary = 2, permanent = 2) or during epicardial mapping/ablation (n = 2) were included. All patients underwent initial angiography with repeat study performed post-lead repositioning/withdrawal for the pacemaker cases. Patients with CP due to permanent pacing leads underwent CT comparison., Results: In 4 pacemaker patients, temporary leads caused two acute perforations, permanent active fixation leads caused one subacute right ventricular perforation and one delayed right atrial perforation. CT overdiagnosed CP in one temporary pacemaker patient, and was non-diagnostic in an atrial lead perforation, whereas angiography was accurate in both. Angiography identified an active leak in atrial lead CP, guided percutaneous closure in one case and demonstrated sealing of perforation in all cases. In the 2 epicardial ablation cases, 1 patient underwent surgical repair after a persistent right ventricular perforation, but the other avoided surgery with novel use of an Amplazter® patent ductus arteriosus (PDA) closure device (Abbott, St Paul, MN, USA)., Conclusions: Angiography may be more accurate than CT in the diagnosis of CP. Angiography is easy to perform, can be done acutely, reveals active leaks and can demonstrate sealing of perforations after percutaneous lead repositioning. Utilisation of a PDA closure device may avoid the need for surgery for RV perforation.

With the expanding integration of complementary and alternative medicine (CAM) practices in conjunction with modern medicine, yoga has quickly risen to being one of the most common CAM practices across the world. Despite widespread use of yoga, limited studies are available, particularly in the setting of dysrhythmia. Preliminary studies demonstrate promising results from integration of yoga as an adjunct to medical therapy for management of dysrhythmias. In this review, we discuss the role of autonomic nervous system in cardiac arrhythmia,interaction of yoga with autonomic tone and its subsequent impact on these disease states. The role of yoga in specific disease states, and potential future direction for studies assessing the role of yoga in dysrhythmia.

Introduction: There is a surge of interest in alternate site pacing to prevent pacing-induced left ventricular dysfunction. However, little is known regarding the appropriate atrioventricular (AV) delay between right ventricular (RV) septal and RV apical pacing for optimal hemodynamic benefit., Objectives: To determine the programmed values of atrial sensed and atrial paced AV delays in basal RV septal and apical RV pacing that results in the maximum delivered stroke volume (SV)., Methods: We calculated the Doppler-derived SV at various sensed and paced AV delays in 50 patients with complete AV block implanted with a dual-chamber pacemaker (group A: 25 RV apical pacing; group B: 25 RV septal pacing). The hemodynamic difference in terms of the SV between sensed and paced AV delay, corresponding to the site of RV pacing was then compared for statistical significance., Results: In group A, maximal SV was derived at a sensed AV delay of 123.2 ± 11 ms and paced AV delay of 129.2 ± 10 ms, and in group B, at a sensed AV delay of 123.6 ± 8 ms and paced AV delay of and 132.8 ± 7 ms. At these intervals, there was no difference in the SV between septal and apical RV pacing (P = .28 and .22, respectively)., Conclusion: The atrial sensed and atrial paced AV delays for septal and apical RV pacing for optimal hemodynamics are similar. For optimal hemodynamics, the atrial paced AV delay is longer than the atrial sensed AV delay., (© 2019 Wiley Periodicals, Inc.)

Background: Incessant focal atrial tachycardia (FAT), if untreated, can lead to ventricular dysfunction and heart failure (tachycardia-induced cardiomyopathy). Drug therapy of FAT is often difficult and ineffective. The efficacy of ivabradine has not been systematically evaluated in the treatment of FAT., Methods: The study group consisted of patients with incessant FAT (lasting >24 hours) and structurally normal hearts. Patients with ventricular dysfunction as a consequence of FAT were not excluded. All antiarrhythmic drugs were discontinued at least 5 half-lives before the initiation of ivabradine. Oral ivabradine (adults, 10 mg twice 12 hours apart; pediatric patients: 0.28 mg/kg in 2 divided doses) was initiated in the intensive care unit under continuous electrocardiographic monitoring. A positive response was defined as the termination of tachycardia with the restoration of sinus rhythm or suppression of the tachycardia to <100 beats per minute without termination within 12 hours of initiating ivabradine., Results: Twenty-eight patients (mean age, 34.6±21.5 years; women, 60.7%) were included in the study. The most common symptom was palpitation (85.7%) followed by shortness of breath (25%). The mean atrial rate during tachycardia was 170±21 beats per minute, and the mean left ventricular ejection fraction was 54.7±14.3%. Overall, 18 (64.3%) patients responded within 6 hours of the first dose of ivabradine. Thirteen of 18 ivabradine responders subsequently underwent successful catheter ablation. FAT originating in the atrial appendages was a predictor of ivabradine response compared with those arising from other atrial sites (P=0.046)., Conclusions: Ivabradine-sensitive atrial tachycardia constitutes 64% of incessant FAT in patients without structural heart disease. Incessant FAT originating in the atrial appendages is more likely to respond to ivabradine than that arising from other atrial sites. Our findings implicate the funny current in the pathogenesis of FAT.

Objectives: This study sought to determine the cause of recurrent syncope and clinical outcomes by using the head-up tilt test (HUTT) and an insertable loop recorder (ILR) in patients with structural heart disease (SHD) and negative electrophysiology study (EPS) results., Background: Patients with syncope and SHD with negative EPS findings have a low risk of sudden cardiac arrest. Nevertheless, the cause of recurrent syncope and the outcomes in these patients are not well characterized., Methods: This prospective study evaluated syncope patients with SHD and negative EPS results by using HUTT (with sublingual nitroglycerine [NTG] provocation as needed) and ILR. A total of 41 SHD patients (27 patients [66%] had coronary arterial disease, and 14 patients [34.15%] had dilated cardiomyopathy with mean EF of 42 ± 4.8% [range 30% to 49%]) were included., Results: HUTT findings were positive in 25 patients (61%) in group A and negative in 16 patients (39%) in group B. An ILR was implanted in 21 of 25 group A patients (84%) and in 12 of 16 group B patients (75%), and they were followed for 15 ± 8 months. During follow-up, 17 of 21 patients (81%) in group A and 5 of 12 patients (41.7%) in group B had ILR evidence consistent with reflex syncope. One group B patient had documented atrioventricular block and underwent pacemaker implantation. There were no malignant ventricular arrhythmias or deaths on follow-up., Conclusions: Reflex syncope is the most common cause of syncope and accounts for approximately 60% of cases in patients with SHD, negative EPS results, left ventricular systolic dysfunction with left ventricular EF >30%, and not in heart failure., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Transvenous pacing in patients with postoperative complex congenital heart disease (CHD) can be challenging and pose technical challenges to lead placement because of the complex anatomy, distortions produced by the surgical procedures, and the altered relationship of cardiac chambers. We describe the utility of angiography for transvenous dual chamber pacemaker implantation in a post-operative complex congenital heart disease., (Copyright © 2018 Indian Heart Rhythm Society. Production and hosting by Elsevier B.V. All rights reserved.)

The left ventricular summit is the most common site of idiopathic epicardial ventricular tachycardia (VT). We report a case of a 48-yr-old lady who presented with VT of RBBB configuration, inferior axis and delayed initial activation. During electrophysiological study, local activation in the distal great cardiac vein (GCV) preceded surface QRS by 56 ms whereas mapping in the aortic sinuses and left ventricular outflow tract endocardially revealed late activation. Ablation in the distal GCV with an irrigated catheter successfully terminated the tachycardia. The 12‑lead electrocardiogram is an invaluable tool for predicting the VT focus and planning the mapping strategy., (Copyright © 2018 Elsevier Inc. All rights reserved.)