2023 HRS/APHRS/LAHRS guideline on cardiac physiologic pacing for the avoidance and mitigation of heart failure.

Cardiac physiologic pacing (CPP), encompassing cardiac resynchronization therapy (CRT) and conduction system pacing (CSP), has emerged as a pacing therapy strategy that may mitigate or prevent the development of heart failure (HF) in patients with ventricular dyssynchrony or pacing-induced cardiomyopathy. This clinical practice guideline is intended to provide guidance on indications for CRT for HF therapy and CPP in patients with pacemaker indications or HF, patient selection, pre-procedure evaluation and preparation, implant procedure management, follow-up evaluation and optimization of CPP response, and use in pediatric populations. Gaps in knowledge, pointing to new directions for future research, are also identified.

2023 HRS/APHRS/LAHRS guideline on cardiac physiologic pacing for the avoidance and mitigation of heart failure.

Cardiac physiologic pacing (CPP), encompassing cardiac resynchronization therapy (CRT) and conduction system pacing (CSP), has emerged as a pacing therapy strategy that may mitigate or prevent the development of heart failure (HF) in patients with ventricular dyssynchrony or pacing-induced cardiomyopathy. This clinical practice guideline is intended to provide guidance on indications for CRT for HF therapy and CPP in patients with pacemaker indications or HF, patient selection, pre-procedure evaluation and preparation, implant procedure management, follow-up evaluation and optimization of CPP response, and use in pediatric populations. Gaps in knowledge, pointing to new directions for future research, are also identified.

Predicting arrhythmic event score in Brugada syndrome: Worldwide pooled analysis with internal and external validation.

BACKGROUND: Brugada syndrome is an inherited arrhythmic disease associated with major arrhythmic events (MAE). Risk predictive scores were previously developed with various performances. OBJECTIVE: The purpose of this study was to create a novel score-Predicting Arrhythmic evenT (PAT)-with internal and external validation. METHODS: A systematic review was performed to identify risk factors for MAE. The odds ratios (ORs) of each factor were pooled across studies. The PAT scoring scheme was developed based on pooled ORs. The PAT score was internally validated with published 105 Asian patients (follow-up 8.0 ± 4.1 [SD] years) and externally validated with unpublished 164 multiracial patients (82.3% White, 14.6% Asian, 3.2% Black; mean follow-up 8.0 ± 6.9 years) with Brugada syndrome. Performances were assessed and compared with previous scores using receiver operating characteristic curve (ROC) analysis. RESULTS: Sixty-seven studies published between 2002 and 2022 from 26 countries (7358 patients) were included. Pooled ORs were estimated, indicating that 15 of 23 risk factors were significant. The PAT score was then developed accordingly. The PAT score had significantly better discrimination (ROC 0.9671) than the BRUGADA-RISK score (ROC 0.7210; P = .006), Shanghai Score System (ROC 0.7079; P = .003), and Sieira et al score (ROC 0.8174; P = .026) in an external validation cohort. PAT score ≥ 10 predicted the first MAE with 95.5% sensitivity and 89.1% specificity (ROC 0.9460) and the recurrent MAE (ROC 0.7061) with 15.4% sensitivity and 93.3% specificity. CONCLUSION: The PAT score was shown to be useful in predicting MAE for primary prevention in patients with Brugada syndrome.

Type of syncope and outcome in Brugada syndrome: A systematic review and meta-analysis.

Introduction: Brugada syndrome is an inherited arrhythmic disease associated with major arrhythmic events (MAE). The importance of primary prevention of sudden cardiac death (SCD) in Brugada syndrome is well recognized; however, ventricular arrhythmia risk stratification remains challenging and controversial. We aimed to assess the association of type of syncope with MAE via systematic review and meta-analysis. Methods: We comprehensively searched the databases of MEDLINE and EMBASE from inception to December 2021. Included studies were cohort (prospective or retrospective) studies that reported the types of syncope (cardiac, unexplained, vasovagal, and undifferentiated) and MAE. Data from each study were combined using the random-effects, generic inverse variance method of DerSimonian and Laird to calculate the odds ratio (OR) and 95% confidence intervals (CIs). Results: Seventeen studies from 2005 to 2019 were included in this meta-analysis involving 4355 Brugada syndrome patients. Overall, syncope was significantly associated with an increased risk of MAE in Brugada syndrome (OR = 3.90, 95% CI: 2.22-6.85, p < .001, I 2 = 76.0%). By syncope type, cardiac (OR = 4.48, 95% CI: 2.87-7.01, p < .001, I 2 = 0.0%) and unexplained (OR = 4.71, 95% CI: 1.34-16.57, p = .016, I 2 = 37.3%) syncope was significantly associated with increased risk of MAE in Brugada syndrome. Vasovagal (OR = 2.90, 95% CI: 0.09-98.45, p = .554, I 2 = 70.9%) and undifferentiated syncope (OR = 2.01, 95% CI: 1.00-4.03, p = .050, I 2 = 64.6%, respectively) were not. Conclusion: Our study demonstrated that cardiac and unexplained syncope was associated with MAE risk in Brugada syndrome populations but not in vasovagal syncope and undifferentiated syncope. Unexplained syncope is associated with a similar increased risk of MAE compared to cardiac syncope.

Developing an Echocardiography-Based, Automatic Deep Learning Framework for the Differentiation of Increased Left Ventricular Wall Thickness Etiologies.

AIMS: Increased left ventricular (LV) wall thickness is frequently encountered in transthoracic echocardiography (TTE). While accurate and early diagnosis is clinically important, given the differences in available therapeutic options and prognosis, an extensive workup is often required to establish the diagnosis. We propose the first echo-based, automated deep learning model with a fusion architecture to facilitate the evaluation and diagnosis of increased left ventricular (LV) wall thickness. METHODS AND RESULTS: Patients with an established diagnosis of increased LV wall thickness (hypertrophic cardiomyopathy (HCM), cardiac amyloidosis (CA), and hypertensive heart disease (HTN)/others) between 1/2015 and 11/2019 at Mayo Clinic Arizona were identified. The cohort was divided into 80%/10%/10% for training, validation, and testing sets, respectively. Six baseline TTE views were used to optimize a pre-trained InceptionResnetV2 model. Each model output was used to train a meta-learner under a fusion architecture. Model performance was assessed by multiclass area under the receiver operating characteristic curve (AUROC). A total of 586 patients were used for the final analysis (194 HCM, 201 CA, and 191 HTN/others). The mean age was 55.0 years, and 57.8% were male. Among the individual view-dependent models, the apical 4-chamber model had the best performance (AUROC: HCM: 0.94, CA: 0.73, and HTN/other: 0.87). The final fusion model outperformed all the view-dependent models (AUROC: HCM: 0.93, CA: 0.90, and HTN/other: 0.92). CONCLUSION: The echo-based InceptionResnetV2 fusion model can accurately classify the main etiologies of increased LV wall thickness and can facilitate the process of diagnosis and workup.

Surgical versus catheter ablation in atrial fibrillation: A systematic review and meta-analysis of randomized controlled trials.

INTRODUCTION: Atrial fibrillation (AF) is the most common cardiac arrhythmia with a high stroke and mortality rate. The video-assisted thoracoscopic radiofrequency pulmonary vein ablation is a treatment option for patients who fail catheter ablation. Randomized data comparing surgical versus catheter ablation are limited. We performed a meta-analysis of randomized control trials to explore the outcome efficacy between surgical and catheter radiofrequency pulmonary vein ablation in patients with AF. METHODS: We comprehensively searched the databases of MEDLINE and EMBASE from inception to December 2020. Included studies were published randomized control trials that compared video-assisted thoracoscopic and catheter radiofrequency pulmonary vein ablation. Data from each study were combined using the fixed-effects, generic inverse variance method of DerSimonian, and Laird to calculate odds ratios and 95% confidence intervals. RESULTS: Six studies from November 2013 to 2020 were included in this meta-analysis involving 511 AF patients (79% paroxysmal) with 263 catheter ablation (mean age 56 ± 3 years) and 248 surgical ablations (mean age 52 ± 4 years). Catheter ablation was associated with increased atrial arrhythmias recurrence when compared to surgical ablation (pooled relative risk = 1.85, 95% confidence interval: 1.44-2.39, p < .001, I2 = 0.0%) but associated with less total major adverse events (pooled relative risk = 0.29, 95% confidence interval: 0.16-0.53, p < .001, I2 = 0.0%). In subgroup analysis, catheter ablation was associated with increased AF recurrence in refractory paroxysmal AF when compared to surgical ablation (pooled relative risk = 2.47, 95% confidence interval: 1.31-4.65, p = .005, I2 = 0.0%) but not in persistent AF (relative risk = 1.09, 95% confidence interval: 0.60-2.0, p = .773). CONCLUSION: Catheter ablation was associated with higher atrial arrhythmia recurrence when compared with surgical ablation. However, our study suggests that the benefit of surgical ablation in patients with persistent AF is unclear. More studies and alternative ablation strategies investigation in persistent AF are warranted.

2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders.

This international multidisciplinary document is intended to guide electrophysiologists, cardiologists, other clinicians, and health care professionals in caring for patients with arrhythmic complications of neuromuscular disorders (NMDs). The document presents an overview of arrhythmias in NMDs followed by detailed sections on specific disorders: Duchenne muscular dystrophy, Becker muscular dystrophy, and limb-girdle muscular dystrophy type 2; myotonic dystrophy type 1 and type 2; Emery-Dreifuss muscular dystrophy and limb-girdle muscular dystrophy type 1B; facioscapulohumeral muscular dystrophy; and mitochondrial myopathies, including Friedreich ataxia and Kearns-Sayre syndrome, with an emphasis on managing arrhythmic cardiac manifestations. End-of-life management of arrhythmias in patients with NMDs is also covered. The document sections were drafted by the writing committee members according to their area of expertise. The recommendations represent the consensus opinion of the expert writing group, graded by class of recommendation and level of evidence utilizing defined criteria. The recommendations were made available for public comment; the document underwent review by the Heart Rhythm Society Scientific and Clinical Documents Committee and external review and endorsement by the partner and collaborating societies. Changes were incorporated based on these reviews. By using a breadth of accumulated available evidence, the document is designed to provide practical and actionable clinical information and recommendations for the diagnosis and management of arrhythmias and thus improve the care of patients with NMDs.

A real-world experience of atrioventricular synchronous pacing with leadless ventricular pacemakers.

AIMS: The MicraTM transcatheter pacing system (TPS) (Medtronic) is the only leadless pacemaker that promotes atrioventricular (AV) synchrony via accelerometer-based atrial sensing. Data regarding the real-world experience with this novel system are scarce. We sought to characterize patients undergoing MicraTM -AV implants, describe percentage AV synchrony achieved, and analyze the causes for suboptimal AV synchrony. METHODS: In this retrospective cohort study, electronic medical records from 56 consecutive patients undergoing MicraTM -AV implants at the Mayo Clinic sites in Minnesota, Florida, and Arizona with a minimum follow-up of 3 months were reviewed. Demographic data, comorbidities, echocardiographic data, and clinical outcomes were compared among patients with and without atrial synchronous ventricular pacing (AsVP) ≥ 70%. RESULTS: Sixty-five percent of patients achieved AsVP ≥ 70%. Patients with adequate AsVP had smaller body mass indices, a lower proportion of congestive heart failure, and prior cardiac surgery. Echocardiographic parameters and procedural characteristics were similar across the two groups. Active device troubleshooting was associated with higher AsVP. The likely reasons for low AsVP were small A4-wave amplitude, high ventricular pacing burden, and inadequate device reprogramming. Importantly, in patients with low AsVP, subjective clinical worsening was not noted during follow-up. CONCLUSION: With the increasing popularity of leadless pacemakers, it is paramount for device implanting teams to be familiar with common predictors of AV synchrony and troubleshooting with MicraTM -AV devices.

Step-by-step guide to creating the best syncope units: From combined United States and European experiences.

Syncope can be caused by many physiological and pathophysiological conditions. Causes of syncope encompass a wide range of conditions from benign vasovagal syncope to life-threatening arrhythmias. The lack of a standardized method of evaluation and management of this large patient population leads to a wide practice variation which results in broad-based testing, frequent hospital admission and high healthcare cost. The concept of a syncope observational unit was created for inpatients and outpatients in the United States and Europe. Studies have demonstrated that syncope units, staffed by trained health care providers with sufficient resources could expedite and improve diagnostic yield, reduce hospital admission, and result in decreased healthcare cost with favorable clinical outcomes. The implementation of a standardized syncope unit has been challenging because resources and health care systems are variable regionally, nationally, and internationally. In this review, we provide an overview of the evidences that support a standardized syncope unit practice. We provide step-by-step algorithms for the "best syncope units" in the inpatient and outpatient settings by combining the synergistic experiences from the United States and Europe.

Surviving sudden cardiac arrest-successes, challenges, and opportunities.

Sudden cardiac arrest (SCA) is the most common cause of death in the world. This manuscript highlights the various challenges in prevention and early management of SCA and also discusses the current state of SCA awareness. The manuscript also outlines the various national and international initiatives in improving SCA awareness and their impact on improving outcomes in SCA. Various campaigns have strived for widespread dissemination of cardiopulmonary resuscitation training and advocated for broader public access defibrillator availability. Finally, the manuscript describes future directions including harnessing technology with voice command and artificial intelligence to allow lay person deliver effective CPR, to improve EMS response times, and to allow wider CPR knowledge dissemination in schools and places of employment. Future research should be focused on optimizing SCA outcomes among vulnerable populations and minorities. Advancements in resuscitation science and use of big data for improvement of EMS services will improve outcomes in SCA.

Syncope Time Frames for Adverse Events after Emergency Department Presentation: An Individual Patient Data Meta-Analysis.

Background and Objectives: Knowledge of the incidence and time frames of the adverse events of patients presenting syncope at the ED is essential for developing effective management strategies. The aim of the present study was to perform a meta-analysis of the incidence and time frames of adverse events of syncope patients. Materials and Methods: We combined individual patients' data from prospective observational studies including adult patients who presented syncope at the ED. We assessed the pooled rate of adverse events at 24 h, 72 h, 7-10 days, 1 month and 1 year after ED evaluation. Results: We included nine studies that enrolled 12,269 patients. The mean age varied between 53 and 73 years, with 42% to 57% females. The pooled rate of adverse events was 5.1% (95% CI 3.4% to 7.7%) at 24 h, 7.0% (95% CI 4.9% to 9.9%) at 72 h, 8.4% (95% CI 6.2% to 11.3%) at 7-10 days, 10.3% (95% CI 7.8% to 13.3%) at 1 month and 21.3% (95% CI 15.8% to 28.0%) at 1 year. The pooled death rate was 0.2% (95% CI 0.1% to 0.5%) at 24 h, 0.3% (95% CI 0.1% to 0.7%) at 72 h, 0.5% (95% CI 0.3% to 0.9%) at 7-10 days, 1% (95% CI 0.6% to 1.7%) at 1 month and 5.9% (95% CI 4.5% to 7.7%) at 1 year. The most common adverse event was arrhythmia, for which its rate was 3.1% (95% CI 2.0% to 4.9%) at 24 h, 4.8% (95% CI 3.5% to 6.7%) at 72 h, 5.8% (95% CI 4.2% to 7.9%) at 7-10 days, 6.9% (95% CI 5.3% to 9.1%) at 1 month and 9.9% (95% CI 5.5% to 17) at 1 year. Ventricular arrhythmia was rare. Conclusions: The risk of death or life-threatening adverse event is rare in patients presenting syncope at the ED. The most common adverse events are brady and supraventricular arrhythmias, which occur during the first 3 days. Prolonged ECG monitoring in the ED in a short stay unit with ECG monitoring facilities may, therefore, be beneficial.

Empirical Ablation to Prevent Sequential Purkinje System Recruitment: A Novel Therapy for Idiopathic Ventricular Fibrillation.

We report 3 cases (mean age 48.3 ± 11.6 years) of idiopathic ventricular fibrillation (IVF), in which a triggering premature ventricular complex leading to IVF could not be identified. All patients underwent posterior fascicle transection with empirical linear ablation of the mid-Purkinje potentials identified along the left ventricular interventricular inferior septum, and no ventricular fibrillation recurrence was documented in any of the patients. (Level of Difficulty: Advanced.).

Lower Extremity Arterial Disease as a Predictor of Incident Atrial Fibrillation and Cardiovascular Events.

OBJECTIVE: To evaluate the relationship between peripheral arterial disease (PAD) and incident atrial fibrillation (AF) and its clinical and pathophysiologic implications on ischemic stroke and all-cause mortality. PATIENTS AND METHODS: We identified all adult patients in the Mayo Clinic Health System without a previous diagnosis of AF undergoing ankle-brachial index (ABI) testing for any indication from January 1, 1996, to June 30, 2018. Retrospective extraction of ABI data and baseline echocardiographic data was performed. The primary outcome of interest was incident AF. The secondary outcomes of interest were incident ischemic stroke and all-cause mortality. RESULTS: A total of 33,734 patients were included in the study. After adjusting for demographic and comorbidity variables, compared with patients who had normal ABI (1.0 to 1.39), there was an increased risk of incident AF in patients with low ABI (<1.0) (adjusted hazard ratio, 1.14; 95% CI, 1.06 to 1.22) and elevated ABI (≥1.4) (adjusted hazard ratio, 1.18; 95% CI, 1.06 to 1.31). The risk was greater in patients with increasing severity of PAD. Patients with abnormal ABIs had an increased risk of ischemic stroke and all-cause mortality. We found that patients with PAD and incident AF have certain baseline echocardiographic abnormalities. CONCLUSION: In this large cohort of ambulatory patients undergoing ABI measurement, patients with PAD were at increased risk for incident AF, ischemic stroke, and mortality. In these high-risk patients with abnormal ABI, particularly severe PAD and cardiac structural abnormalities, routine monitoring for AF and management of cardiovascular risk factors may be warranted.

Does the Age of Sudden Cardiac Death in Family Members Matter in Brugada Syndrome?

Background Brugada syndrome is an inherited cardiac channelopathy associated with major arrhythmic events (MAEs). The presence of a positive family history of sudden cardiac death (SCD) as a risk predictor of MAE remains controversial. We aimed to examine the association between family history of SCD and MAEs stratified by age of SCD with a systematic review and meta-analysis. Methods and Results We searched the databases of MEDLINE and EMBASE from January 1992 to January 2020. Data from each study were combined using the random-effects model. Fitted metaregression was performed to evaluate the association between the age of SCD in families and the risk of MAE. Twenty-two studies from 2004 to 2019 were included in this meta-analysis involving 3386 patients with Brugada syndrome. The overall family history of SCD was not associated with increased risk of MAE in Brugada syndrome (pooled odds ratio [OR], 1.11; 95% CI, 0.82-1.51; P=0.489, I2=45.0%). However, a history of SCD in family members of age younger than 40 years of age did increase the risk of MAE by ≈2-fold (pooled OR, 2.03; 95% CI, 1.11-3.73; P=0.022, I2=0.0%). When stratified by the age of cut point at 50, 45, 40, and 35 years old, a history of SCD in younger family member was significantly associated with a higher risk of MAE (pooled OR, 0.49, 1.30, 1.51, and 2.97, respectively; P=0.046). Conclusions A history of SCD among family members of age younger than 40 years was associated with a higher risk of MAE.

Natural history and clinical significance of isolated complete left bundle branch block without associated structural heart disease.

OBJECTIVE: Left bundle branch block (LBBB), which is associated with underlying cardiac disease, is believed to play a role in the pathogenesis of cardiomyopathy through delays in interventricular conduction, leading to dyssynchrony. However, this has not been established in previous studies. It is unclear whether LBBB indicates clinically advanced cardiac disease or is an independent factor responsible for increased mortality and the development of heart failure. We investigated the natural history of isolated LBBB without any associated structural heart disease in order to determine its clinical significance. METHODS: We performed a retrospective chart review on consecutive patients who fulfilled the 12-lead electrocardiographic (ECG) criteria for complete LBBB and had a normal echocardiogram with no evidence of structural heart disease and left or right ventricular systolic dysfunction within three months of the initial ECG between January 1, 2000 and December 31, 2009. We excluded patients with documented coronary artery disease (CAD) at any time, any structural heart disease, or cardiac devices. We evaluated the primary endpoints of mortality and incidence of cardiomyopathy, as well as any heart failure hospitalizations over a 1- and 10-year period. RESULTS: We identified 2522 eligible patients. The mean follow-up duration was 8.4±3.2 years. The one-year mortality rate was 7.8%, with a 10-year mortality rate of 22.0%. The incidence of cardiomyopathy over one year was 3.2% and over 10 years was 9.1%. There was no significant difference in QRS duration between patients who were alive and those that were deceased at 10 years (141+/-18 vs. 141+/-17 ms; p=0.951) and patients with and without cardiomyopathy at 10 years (142±17 vs. 141±17 ms; p=0.532). CONCLUSION: Isolated LBBB occurring without structural heart disease, ventricular dysfunction, or CAD is associated with a low mortality rate and incidence of cardiomyopathy.