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Although ventricular dysfunction is associated with the occurrence of ventricular arrhythmia (VA), most patients with cardiomyopathy do not experience VA. We therefore investigated other predictors of VA in a large contemporary cohort of patients with cardiomyopathy. All patients at a large academic medical system with left ventricular ejection fraction (LVEF) ≤50% were enrolled at the time of first documented low LVEF. Predictors of hospital admission for VA were examined using multivariable Cox models. The incidence of implantable defibrillator (ICD) placement was also examined. A total of 18,003 patients were enrolled. Over a median follow-up of 3.35 years, 389 patients (2.2%) were admitted for VA (304 of 12,037 [2.5%] among patients with LVEF ≤35% vs 85 of 5,966 [1.4%] among those with LVEF 36% to 50%). Predictors of VA hospitalization included lower LVEF (hazard ratio (HR) = 1.43 per 10% decrease, p0.001), the presence of an ICD at baseline (HR = 1.63, p = 0.010), higher blood glucose (HR = 1.02 per 10 mg/100 ml increase, p = 0.050), the presence of end-stage renal disease (HR = 3.59, p0.001), and the presence of liver cirrhosis (HR = 1.93, p = 0.013). During follow-up, 626 patients were implanted with a new ICD. In addition to being admitted with VA, a lower LVEF and a history of coronary artery disease or heart failure were the main predictors of ICD therapy in this population. In conclusion, in addition to more severe cardiomyopathy and the presence of an implanted ICD, metabolic derangements on initial contact are independent predictors of hospital admissions for VA in patients with cardiomyopathy. Noncardiac co-morbidities play an important role in stratifying patients with cardiomyopathy for their risk of VA or cardiac arrest.

INTRODUCTION Pacing-induced cardiomyopathy (PICM) is a potential complication of chronic right ventricular (RV) pacing, but its characterization in adult patients is often complicated by pre-existing cardiomyopathy. This study investigated the incidence of PICM in patients with congenital heart block (cHB) who have conduction disease from birth without confounding pre-existing cardiac conditions. METHODS AND RESULTS This retrospective cohort analysis included 42 patients with cHB and baseline left ventricular ejection fraction (LVEF) ≥50%. Kaplan-Meier analysis was used to assess freedom from cardiomyopathy (defined as LVEF

Background Obesity is a worldwide epidemic that has been associated with poor outcomes. Previous studies have demonstrated an inverse relationship between body mass index (BMI) and outcomes, the 'obesity paradox', in several diseases. Hypothesis We sought to evaluate whether the obesity paradox is present in patients with left ventricular systolic dysfunction (LVSD) of all etiologies, using all‐cause mortality as the primary endpoint and hospitalization as the secondary endpoint. Methods We conducted a retrospective cohort study of LVSD patients (n = 18 003) seen within the University of Pittsburgh Medical Center network between January 2011 and December 2017. Patients were divided into four BMI categories (underweight, normal weight, overweight, and obese) and stratified by left ventricular ejection fraction (LVEF)

Contemporary guideline-directed medical therapy (GDMT) confers a significant mortality benefit for patients with heart failure with reduced ejection fraction (HFrEF), as compared to GDMT prevalent at the time of landmark primary prevention implantable cardioverter-defibrillator (ICD) trials. The impact of modern era GDMT on survival in this population is unknown.This study sought to investigate the impact of number of GDMT medications prescribed for HFrEF on all-cause mortality in recipients of primary prevention ICD.A cohort of 4,972 recipients with primary prevention ICD (n = 3,210) or cardiac resynchronization therapy-defibrillator (CRT-D) (n = 1,762) was studied. The association of number of GDMT medications prescribed at the time of device implantation and all-cause mortality at 2 years post implantation was examined.In our primary prevention cohort, 5%, 20%, 52%, and 23% of patients were prescribed 0, 1, 2, or 3-4 GDMT medications, respectively. After risk adjustment for age, sex, ejection fraction, body mass index, the Elixhauser comorbidity score, the type of cardiomyopathy, and the year of device implantation, each additional GDMT conferred a reduction in the risk of death of 36% in recipients of ICD (HR: 0.64; P 0.001) and 30% in recipients of CRT-D (HR: 0.70; P 0.001).A higher number of prescribed GDMT medications is associated with an incremental 1-year survival in recipients of primary prevention ICD with or without CRT. Initiation of maximum number of tolerated GDMT medications should therefore be the goal for all patients with HFrEF. In the setting of robust GDMT, the risk versus benefit of a primary prevention ICD warrants re-examination in future studies.

Objectives This study examined the independent predictors of all-cause mortality, all hospitalizations, and cardiac hospitalizations in patients with mild-to-moderate cardiomyopathy (left ventricular ejection fractions [LVEFs] of 36% to 50%). Background Patients with severe cardiomyopathy have high rates of death. Implantable cardioverter-defibrillators (ICDs) improve survival in this setting. It is not known whether the same applies to patients with mild-to-moderate cardiomyopathy. Methods All patients with cardiomyopathy of any etiology seen at our institution between 2011 and 2017 were included. Baseline characteristics and outcomes were compared between patients with mild-to-moderate cardiomyopathy and severe cardiomyopathy (LVEF ≤35%). Results Of the 18,003 patients with cardiomyopathy, 5,966 (33%) had a LVEF between 36% and 50%. Over a median follow-up of 3.35 years, 8,037 patients (45%) died and 11,056 (61%) were hospitalized for cardiac reasons. Independent predictors of all-cause mortality included older age (p Conclusions A history of DM predicts mortality in patients with cardiomyopathy and is associated with worse outcome than the actual severity of cardiomyopathy. Patients with mild-to-moderate cardiomyopathy and DM may therefore benefit from the same life-saving therapies (e.g., ICDs) that are indicated for patients with severe cardiomyopathy. This finding needs to be verified in a prospective, randomized setting.

Atrial Fibrillation (AF) is the most common arrhythmia in adults, and the rapid increase in AF prevalence has been classified by experts as an epidemic. The mechanisms of AF are complex and incompletely understood. While many aspects of management are now based on high quality evidence, other clinical decisions are based on experience and judgment. This article provides an up to date review relating to lifestyle modification and its effect on AF to inform clinical treatment. This comprehensive review used PubMed and Google Scholar to perform keyword searches of articles published between 1998 and the present, with the exception of the 1978 "Holiday Heart" article. Robust data has emerged identifying multiple risk factors for development of AF, including age, sex, hypertension, diabetes mellitus, obesity, alcohol consumption, exercise, and obstructive sleep apnea. Recent evidence indicates that lifestyle modification has a significant role in mitigating the risk and burden of AF. In conclusion, based on the available evidence, an interdisciplinary approach to lifestyle modification will likely reduce risk and/or symptom burden of AF.

Patients with atrial fibrillation (AF) commonly have impaired renal function. The safety and efficacy of direct oral anticoagulants (DOACs) in patients with chronic kidney disease (CKD) and end-stage renal disease has not been fully elucidated. This study evaluated and compared the safety outcomes of DOACs versus warfarin in patients with nonvalvular AF and concomitant CKD. Patients in our health system with AF prescribed oral anticoagulants during 2010 to 2017 were identified. All-cause mortality, bleeding and hemorrhagic, and ischemic stroke were evaluated based on degree of renal impairment and method of anticoagulation. There were 21,733 patients with a CHA2DS2-VASc score of ≥2 included in this analysis. Compared with warfarin, DOAC use in patients with impaired renal function was associated with lower risk of mortality with a hazard ratio (HR): 0.76 (95% confidence interval [CI] 0.70 to 0.84, p value 60, HR 0.74 (95% CI 0.68 to 0.81, p value 30 to 60, and HR 0.76 (95% CI 0.63 to 0.92, p value 60, HR 0.83 (95% CI 0.74 to 0.94, p value 0.003) in patients with eGFR >30 to 60, and HR 0.69 (95% CI 0.50 to 0.93, p value 0.017) in patients with eGFR ≤30 or on dialysis. In conclusion, in comparison to warfarin, DOACs appear to be safe and effective with a lower risk of all-cause mortality and lower bleeding across all levels of CKD.

INTRODUCTION Patients eligible for primary prevention implantable cardioverter-defibrillator (ICD) therapy are faced with a complex decision that needs a clear understanding of the risks and benefits of such an intervention. In this study, our goal was to explore the documentation of primary prevention ICD discussions in the electronic medical records (EMRs) of eligible patients. METHODS In 1523 patients who met criteria for primary prevention ICD therapy between 2013 and 2015, we reviewed patient charts for ICD-related documentation: "mention" by physicians or "discussion" with patient/family. The attitude of the physician and the patient/family toward ICD therapy during discussions was categorized into negative, neutral, or positive preference. Patients were followed to the end-point of ICD implantation. RESULTS Over a median follow-up of 442 days, 486 patients (32%) received an ICD. ICD was mentioned in the charts of 1105 (73%) patients, and a discussion with the patient/family about the risks and benefits of ICD was documented in 706 (46%) charts. On multivariable analyses, positive cardiologist (hazard ratio [HR]: 7.9, 95% confidence of intervals [CI]: 1.0-59.7, P

A re-designed single chamber leadless pacemaker (LP; Aveir) was developed with the goal of improving safety and performance of its predecessor (Nanostim). Herein, we present the first-in-human expe...

Objectives This study sought to investigate the impact of the neurological status of sudden cardiac arrest (SCA) survivors on implantable cardioverter-defibrillator (ICD) insertion and long-term mortality. Background The neurological status of SCA survivors may impact the decision to insert an ICD insertion and influence long-term survival. Methods In 1,433 survivors of SCA between 2002 and 2012, we examined the neurological status immediately after the arrest using the Pittsburgh Cardiac Arrest Category (PCAC) and prior to hospital discharge using the cerebral performance category (CPC) score. Patients were followed up to the endpoints of ICD implantation and all-cause mortality. Results Over a median follow-up period of 3.6 years, 389 (27%) patients received an ICD, and 674 (47%) died. The PCAC (adjusted hazard ratio [HR]: 0.79; 95% confidence interval [CI]: 0.69 to 0.90) and CPC (adjusted HR: 0.73; 95% CI: 0.64 to 0.84) scores were highly predictive of the time to ICD insertion and of all-cause mortality (PCAC score, adjusted HR: 1.39; 95% CI: 1.24 to 1.57; CPC score, adjusted HR: 2.03; 95% CI: 1.77 to 2.34). ICD therapy was associated with better survival even after adjusting for neurological status (HR: 0.56; 95% CI: 0.43 to 0.73). A significant proportion of patients in the worse CPC categories had a >1-year survival after the index SCA. Conclusions In SCA survivors, worse neurological performance was associated with lower likelihood of ICD insertion and higher mortality. ICD insertion was associated with improved survival even after accounting for neurological performance. ICD discussion should therefore not be omitted in these patients.

In patients with hypertrophic cardiomyopathy (HC), atrial fibrillation (AF) is common, often poorly tolerated and difficult to treat. Limited data exists regarding safety or efficacy of drug therapy for AF rhythm control in HC patients. We performed a retrospective analysis of patients with HC followed >6 months, treated with amiodarone, sotalol, dofetilide, or disopyramide for rhythm control of non-postoperative AF. The duration followed on each medication, reasons for discontinuing, and incidences of adverse events were recorded. Confounding factors including maximum ventricular septal thickness, age, left ventricular ejection fraction, and gender were assessed. Ninety-eight patients had 130 drug treatments (defined as a continuous time on 1 drug); 23 patients were treated with >1 medication. The probability of remaining on a single antiarrhythmic drug at 1 year was 62% and at 3 was 42%. Maximum ventricular septal thickness (hazard ratio 1.05, p = 0.03) and presence of resting outflow gradient (hazard ratio 2.50, p = 0.002) were associated with discontinuation of therapy. Patients treated with amiodarone or sotalol had no serious safety events suggesting that these medications may be reasonably safe. Amiodarone was least likely to be discontinued for inefficacy (8.5%), but likely to be discontinued for side effects (19%). The probability of remaining on sotalol was 74% at 1 year and 50.0% at 3 and it was only discontinued for side effects in 2%. A small number of patients were treated with disopyramide and dofetilide. In conclusion, our data suggest that amiodarone and sotalol are likely safe, and that sotalol may be particularly attractive given its low rate of side effects and low rate of discontinuation.

Moderate-intensity exercise improves cardiovascular outcomes. However, mounting clinical evidence demonstrates that long-term, high-intensity endurance training predisposes male and veteran athletes to an increased risk of atrial fibrillation (AF), a risk that is not observed across both genders. Although increased mortality associated with AF in the general population is not shared by athletes, clinically significant morbidities exist (eg, reduced exercise capacity, athletic performance, and quality of life). Additional research is needed to fill current gaps in knowledge pertaining to the natural history, pathophysiologic mechanisms, and management strategies of AF in the athlete.

Racial disparities in health outcomes have been widely documented in medicine, including in cardiovascular care. While some progress has been made, these disparities have continued to plague our healthcare system. Patients with cardiomyopathy are at an increased risk of death and cardiovascular hospitalizations. In the present analysis, we examined the baseline characteristics and outcomes of black and white men and women with cardiomyopathy. All patients with cardiomyopathy (left ventricular ejection fraction (LVEF)50%) cared for at University of Pittsburgh Medical Center (UPMC) between 2011 and 2017 were included in this analysis. Patients were stratified by race, and outcomes were compared between Black and White patients using Cox proportional hazard models. Of a total of 18,003 cardiomyopathy patients, 15,804 were white (88%), 1,824 were black (10%) and 375 identified as other (2%). Over a median follow-up time of 3.4 years, 7,899 patients died. Black patients were on average a decade younger (p0.001) and demonstrated lower unadjusted all-cause mortality (hazard ratio [HR]: 0.83%; 95% CI 0.77 to 0.90; p0.001). However, after adjusting for age and other comorbidities, black patients had higher all-cause mortality compared to white patients (HR: 1.15, 95% CI 1.07 to 1.25; p0.001). These differences were seen in both men (HR:1.19, 95% CI 1.08 to 1.33; p0.001) and women (HR:1.12, 95% CI 0.99 to 1.25; p = 0.065). In conclusion, our data demonstrate higher all-cause mortality in black compared to white men and women with cardiomyopathy. These findings are likely explained, at least in part, by significantly higher rates of comorbidities in black patients. Earlier interventions targeting these comorbidities may mitigate the risk of progression to heart failure and improve outcomes.

The future of the American Board of Internal Medicine Maintenance of Certification (MOC) program is at a crossroads. The current MOC program lacks a clear visible mission, adds to modern health care’s onerous bureaucracy, and thus pulls physicians from the most important humanistic aspects of their profession. The aim of the MOC program should be to promote the best patient care by ensuring certified physicians maintain core skills through continuous education and evaluation. The program should focus on education and be designed with the rigorous obligations of practicing physicians in mind. Moving forward, the American Board of Internal Medicine should cocreate MOC with the physician community and apply innovative adult education techniques. Over time, data-driven methods and member feedback should be used to provide continuous program improvement. This review describes the origins of the current state of MOC, explores its evidence base, provides examples of model programs for the maintenance of complex professional skills, and outlines guiding principles for the future of MOC.

Background: The implantable cardioverter-defibrillator (ICD) is effective for preventing sudden death in patients with hypertrophic cardiomyopathy. However, data on performance and complications of implanted ICDs over particularly long time periods to inform clinical practice is presently incomplete. Methods: The study cohort comprises 217 consecutive hypertrophic cardiomyopathy patients with primary prevention ICDs implanted before 2008 and followed for ≥10 years (mean 12±4; range to 31). Results: Patients were 38±17 years at implant and 45 (21%) experienced appropriate interventions terminating ventricular tachycardia/ventricular fibrillation. The majority of ICD discharges occurred ≥5 years after implant (29 patients; 64%), including ≥10 years in 16 patients (36%). Initial device therapy increased in frequency from 2.3% of patients at P =0.005). Inappropriate ICD shocks in 39 patients occurred most commonly P =0.02). Other major device complications including infection and lead fractures and dislodgement occurred in 27 patients (12%) but did not increase in frequency over follow-up after implant ( P =0.47). There were no arrhythmic sudden death events among the 217 patients with ICD. Conclusions: In hypertrophic cardiomyopathy, after a primary prevention implant, ICD therapy often followed prolonged periods of device dormancy and increased progressively in frequency over time, including one-third of patients with initial therapy after 5 to 9 years, and an additional one-third of patients at ≥10 years. Frequency of inappropriate shocks decreased over follow-up, likely reflecting standard changes in device programming, while occurrence of device complications, such as lead fractures/infection, did not increase during follow-up.

Coronavirus disease 2019 (COVID-19) is a worldwide pandemic, and cardiovascular complications and arrhythmias in these patients are common. Cardiac monitoring is recommended for at risk patients; however, the availability of telemetry capable hospital beds is limited. We sought to evaluate a patch-based mobile telemetry system for inpatient cardiac monitoring during the pandemic.A prospective cohort study was performed of inpatients hospitalized during the pandemic who had mobile telemetry devices placed; patients were studied up until the time of discharge or death. The primary outcome was a composite of management changes based on data obtained from the system and detection of new arrhythmias. Other clinical outcomes and performance characteristics of the mobile telemetry system were studied.Eighty-two patients underwent mobile telemetry device placement, of which 31 (37.8%) met the primary outcome, which consisted of 24 (29.3%) with new arrhythmias detected and 18 (22.2%) with management changes. Twenty-one patients (25.6%) died during the study, but none from primary arrhythmias. In analyses, age and heart failure were associated with the primary outcome. Monitoring occurred for an average of 5.3 ± 3.4 days, with 432 total patient-days of monitoring performed; of these, QT-interval measurements were feasible in 400 (92.6%).A mobile telemetry system was successfully implemented for inpatient use during the COVID-19 pandemic and was shown to be useful to inform patient management, detect occult arrhythmias, and monitor the QT-interval. Patients with advanced age and structural heart disease may be more likely to benefit from this system.

His bundle pacing (HBP) has recently emerged as a technique to avoid the negative effects of long-term right ventricular apical pacing. In addition to providing physiologic ventricular activation, HBP has been shown to correct underlying conduction abnormalities in certain patients. Although large prospective, randomized clinical trials have not yet been completed, the available observational clinical data support the safety and efficacy of this technique. Here, we review the physiology of the his bundle (HB) as it relates to HBP, describe the current clinical experience, and discuss future directions of this emerging therapy.

Background: Atrial fibrillation (AF), the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM), is capable of producing symptoms that impact quality of life and is associated with risk for embolic stroke. However, the influence of AF on clinical course and outcome in HCM remains incompletely resolved. Methods: Records of 1558 consecutive patients followed at the Tufts Medical Center Hypertrophic Cardiomyopathy Institute for 4.8±3.4 years (from 2004 to 2014) were accessed. Results: Of the 1558 patients with HCM, 304 (20%) had episodes of AF, of which 226 (74%) were confined to symptomatic paroxysmal AF (average, 5±5; range, 1 to >20), whereas 78 (26%) developed permanent AF, preceded by 7±6 paroxysmal AF episodes. At last evaluation, 277 patients (91%) are alive at 62±13 years of age, including 89% in New York Heart Association class I or II. No difference was found in outcome measures for patients with AF and age- and sex-matched patients with HCM without AF. Four percent of patients with AF died of HCM-related causes (n=11), with annual mortality 0.7%; mortality directly attributable to AF (thromboembolism without prophylactic anticoagulation) was 0.1% per year (n=2 patients). Patients were treated with antiarrhythmic drugs (most commonly amiodarone [n=103] or sotalol [n=78]) and AF catheter ablation (n=49) or the Maze procedure at surgical myectomy (n=72). Freedom from AF recurrence at 1 year was 44% for ablation patients and 75% with the Maze procedure ( P P Conclusions: Transient symptomatic episodes of AF, often responsible for impaired quality of life, are unpredictable in frequency and timing, but amenable to effective contemporary treatments, and infrequently progress to permanent AF. AF is not a major contributor to heart failure morbidity or a cause of arrhythmic sudden death; when treated, it is associated with low disease-related mortality, no different than for patients without AF. AF is an uncommon primary cause of death in HCM virtually limited to embolic stroke, supporting a low threshold for initiating anticoagulation therapy.

Although the cardiovascular benefits of moderate exercise are well established, there is growing epidemiological support for the notion that high-intensity endurance athletics increases the risk of atrial fibrillation (AF). There are many gaps in evidence related to epidemiology and mechanisms of AF in endurance athletes. The proposed pathophysiological mechanisms include alterations of autonomic tone, electrical remodeling, anatomical remodeling, fibrosis, and inflammation. Clinical management of the athlete with AF often includes a period of decreased frequency, intensity, and duration of exercise with assessment for improvement in AF recurrence. Based on symptoms, a strategy of rate or rhythm control should be selected; however, due to side effects and intolerance of medications, ablation may be a preferred approach. The risks and benefits of anticoagulation for stroke prevention must be carefully assessed in the athlete with AF. All patients should be encouraged to be physically active with moderation; however, men should be advised of the higher risk of AF with long-term, high-intensity endurance training.

Overt symptomatic atrial fibrillation (AF) occurs in over 20% of patients with hypertrophic cardiomyopathy (HC) leading to impaired quality of life, loss of productivity, and the risk for embolic stroke. However, the overall burden presented by AF in the HC population is unresolved due to the unknown frequency of silent asymptomatic episodes that do not necessarily achieve clinical recognition but nevertheless may have important disease-related implications. Therefore, stored electrograms were analyzed retrospectively for AF in 75 consecutive patients with HC (without AF history) implanted with dual-chamber cardioverter-defibrillators. Patients were followed for 5.0 ± 4.1 years at the Tufts Medical Center HCM Institute; ages were 50 ± 15 years, and 55% were male. Implantable cardioverter-defibrillator interrogation in the 75 patients showed AF to be absent in 54 (72%), 18 (24%) had clinically silent AF episodes, and the remaining 3 (4%) without previous asymptomatic episodes developed symptomatic and clinically overt paroxysmal AF. Of the 18 patients with clinically silent AF, 8 developed symptomatic AF, 4.1 ± 1.5 years later. Nonfatal embolic stroke occurred in 1 patient associated with asymptomatic AF and without other risk factors. In conclusion, clinically silent AF appears to be common in HC, occurring in almost 25% of patients. Such asymptomatic episodes of AF have important future implications, including potential thromboembolic risk, and development of symptomatic and clinically overt AF requiring prophylactic anticoagulation.

Ventricular Arrhythmias in HCMIntroduction Triggers and ICD interventions of ventricular arrhythmias in patients with hypertrophic cardiomyopathy (HCM) offer insight into mechanisms and treatment. Methods and Results Intracardiac ICD electrograms from 71 HCM patients in the HCM I and II studies were analyzed by three individuals. Rhythms were defined as VF (polymorphic ventricular arrhythmia), VT (monomorphic ventricular tachycardia), and ventricular flutter (VFL; VT ≥ 240 bpm). Physical activity and rhythm preceding the arrhythmia were ascertained. Of 149 arrhythmias, VF was present in 74, VT in 57, and VFL in 18. In those whose activity was known, moderate or intense physical activity was associated with over 50% of the tachycardias (57 of 111). Rhythms preceding ventricular arrhythmias were often sinus tachycardia (49 of 149; 33%) or rapid atrial fibrillation (7 of 149; 5%). VF and VFL were more likely preceded by supraventricular rhythms >100 bpm (30 of 68 with VF; 44%; 12 of 16 with VFL 75%, vs. 14 of 50 with VT 28%; P = 0.001). Antitachycardia pacing (ATP) was successful in 39 of 53 (74%). Multiple shocks were more often required to terminate VFL (10 of 18; 56%) compared to VF (10 of 72; 14%) and VT (2 of 25; 8%; P < 0.0001). Of arrhythmias requiring more than one shock to terminate, 16 of 22 were preceded by sinus tachycardia and/or moderate or extreme physical activity. Conclusions Rapid supraventricular rhythms, and at least moderate activity, frequently precede VT and VF, and when they occur in these situations often require multiple ICD shocks to restore sinus rhythm. ATP is successful in terminating VT and VFL, and should be a programmed in all HCM patients with ICDs.

In reply to Dr. White and colleagues, we wish to clarify that our intention was to advance the hypothesis that patients with diabetes mellitus (DM) and mild to moderate cardiomyopathy may benefit from interventions such as implantable cardioverter-defibrillator (ICD) placement rather than draw any

As the most common sustained arrhythmia in adults, atrial fibrillation (AF) is an established and growing epidemic. To provide optimal patient care, it is important for clinicians to be aware of AF's epidemiological trends, methods of risk reduction, and the various available treatment modalities. Our understanding of AF's pathophysiology has advanced, and with this new understanding has come advancements in prevention strategies as well as pharmacological and nonpharmacological treatment options. Following PubMed and MEDLINE searches for AF risk factors, epidemiology, and therapies, we reviewed relevant articles (and bibliographies of those articles) published from 2000 to 2016. This “state-of-the-art” review provides a comprehensive update on the understanding of AF in the world today, contemporary therapeutic options, and directions of ongoing and future study.

International audience; Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.

Sudden cardiac death (SCD) secondary to sudden cardiac arrest (SCA) is a leading cause of death in the United States, claiming over a quarter million lives annually, and is directly responsible for 50% of all cardiovascular mortality. Brugada Syndrome (BrS) is an arrhythmogenic cardiovascular channelopathy that predisposes asymptomatic patients who have no identified disease to a high-risk of SCD/SCA as their first cardiac event/disease manifestation. Limited progress has been made in risk prediction of SCA and SCD, with the greatest challenge being the ability to identify the small high-risk subgroups concealed within the larger general population. In conclusion, accurate identification of high-risk asymptomatic BrS patients (through multiparametric risk scores composed of reliable and validated unambiguous clinical variables and biomarkers) may hold utility in improving current SCD prediction algorithms, and the appropriate primary prevention therapy may prove valuable in reducing risk of sudden death for this patient population. This systematic review aims to comprehensively summarize qualitative evidence that explore proposed clinical, electrocardiographic, electrophysiological, and genetic markers for risk stratification of patients with BrS phenotype, and to discuss the best available contemporary evidence regarding therapeutic approach.

International audience; Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.