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Cardiac magnetic resonance (CMR) findings suggesting a suspected left-dominant arrhythmogenic cardiomyopathy (LDAC) may be difficult to distinguish from those related to previous myocarditis; however, especially in patients with ventricular arrhythmias (VA) with ECG morphology consistent with a left ventricle (LV) origin differential diagnosis is fundamental. Aim of the study was to identify potential imaging features at CMR specific for LDAC diagnosis. Between January 2011 and December 2019, we enrolled 15 consecutive stable patients with a recent diagnosis of significant VA and ECG morphology consistent with a LV origin, detection of potential LV arrhythmic substrate at CMR and undergoing a clinically-indicated LV endomyocardial biopsy showing tissue abnormalities consistent with the diagnosis of LDAC. From the same CMR-endomyocardial biopsy registry, a second group of 30 consecutive patients who underwent CMR and biopsy with a histological diagnosis of previous myocarditis were identified. (1) Subepicardial LGE at the level of the posterolateral wall of the LV was detected in 13 cases of LDAC vs. 21 cases of myocarditis; (2) fat infiltration, and particularly subepicardial posterolateral fat infiltration, was found in almost all LDAC patients vs. one myocarditis only (p

Objectives This study aimed to assess the long-term outcomes of minimally fluoroscopic approach (MFA) compared with conventional fluoroscopic ablation (ConvA) in terms of recurrences of arrhythmia and long-term complications. Background Catheter ablation (CA) of supraventricular tachycardia (SVT) with an MFA, under the guidance of electroanatomic mapping (EAM) systems, results in a significant reduction in exposure to ionizing radiations without impairing acute procedural success and complication rate. However, data regarding long-term outcomes of MFA compared with ConvA are lacking. Methods This is a retrospective observational study. All patients undergoing MFA CA of SVT (atrioventricular nodal re-entrant tachycardia and atrioventricular re-entrant tachycardia) between 2010 and 2015 were enrolled and were compared with matched subjects (1 MFA: 2 ConvA) undergoing ConvA during the same period. The 2 co-primary outcomes were recurrence of arrhythmias and long-term complications. Results A total of 618 patients (mean age 38 ± 15 years, 60% female) were enrolled. MFA included 206 patients, whereas 412 were treated with ConvA. Acute success (99% vs. 97%; p = 0.10) and acute complications (2.4% vs. 5.3%; p = 0.14) were similar in the 2 groups. During a median follow-up of 4.4 years, 5.9% of patients experienced recurrence of arrhythmias. At multivariate analysis, ConvA (hazard ratio [HR]: 3.03) and procedural success (HR: 0.10) were independently associated with recurrence of arrhythmias. Late complications (i.e., advance atrioventricular block and need for pacemaker implantation) occurred more frequently in ConvA (3.4% vs. 0.5%; p = 0.03) compared with MFA. Conclusions CA guided by EAM systems with MFA provided better long-term results and reduced risk of complications compared with ConvA.

Background Outcomes of catheter ablation (CA) among patients with non-paroxysmal atrial fibrillation (AF) are largely disappointing. Purpose We sought to evaluate the feasibility, effectiveness, and safety of a single-stage stepwise endo-/epicardial approach in patients with persistent/longstanding-persistent AF. Methods We enrolled 25 consecutive patients with symptomatic persistent (n=4) or longstanding-persistent (n=21) AF and at least one prior endocardial procedure, who underwent CA using an endo-/epicardial approach. Our anatomical stepwise protocol included multiple endocardial as well as epicardial (Bachmann's bundle [BB] and ligament of Marshall ablations) components, and entailed ablation of atrial tachycardias emerging during the procedure. The primary outcome was freedom from any AF/atrial tachycardia episode after a 3-month blanking period. The secondary outcome was patients' symptom status during follow-up. Results The stepwise endo-/epicardial approach allowed sinus rhythm restoration in 72% of patients, either directly (n=6, 24%) or after AF organization into atrial tachycardia (n=12, 48%). BB's ablation was commonly implicated in arrhythmia termination. After a median follow-up of 266 days (interquartile range, 96 days), survival free from AF/atrial tachycardia was 88%. Antiarrhythmic drugs could be discontinued in 22 patients (88%). As compared to baseline, more patients were asymptomatic at 9-month follow-up (0% vs- 56%, p=0.02). Five patients (20%) developed mild medical complications, whereas one subject (4%) had severe kidney injury requiring dialysis. Conclusion A single-stage endo-/epicardial CA resulted in favorable rhythm and symptom outcomes in a cohort of patients with symptomatic persistent/longstanding-persistent AF and one or more prior endocardial procedures. Epicardial ablation of BB was commonly implicated in procedural success. Funding Acknowledgement Type of funding sources: None.

Interest in endomyocardial biopsy (EMB) has progressively grown during the past decade. Still, its use remains limited to highly specialized centers, mostly because it is considered an invasive procedure with poor diagnostic yield and inherent complications. Indeed, the diagnostic performance of EMB is strictly linked to the sample of myocardium we can obtain. If we can precisely localize areas of diseased myocardium, sampling error or inadequate withdrawals are minimized. In this state-of-the-art review, we provide guidance on how to technically and practically perform EMB guided by electroanatomic voltage mapping and intracardiac echocardiography, and review the evidence supporting this combined approach.

A patient with history of dilated cardiomyopathy, a cardiac resynchronization therapy defibrillator, and endocardial ablation presented for refractory ventricular tachycardia 3 years after implantation of a Jarvik 2000 left ventricular assist device (Jarvik Heart, Inc., New York, New York). Open-chest epicardial ablation safely and effectively terminated the arrhythmia, without ventricular tachycardia recurrence at 9-month follow-up and in the absence of complications during the hospital stay. (Level of Difficulty: Advanced.), Central Illustration

BACKGROUND: Cardiac implantable electronic device (CIED) implantation rates as well as the clinical and procedural characteristics and outcomes in patients with known active coronavirus disease 2019 (COVID-19) are unknown. OBJECTIVE: The purpose of this study was to gather information regarding CIED procedures during active COVID-19, performed with personal protective equipment, based on an international survey. METHODS: Fifty-three centers from 13 countries across 4 continents provided information on 166 patients with known active COVID-19 who underwent a CIED procedure. RESULTS: The CIED procedure rate in 133,655 hospitalized COVID-19 patients ranged from 0 to 16.2 per 1000 patients (P

Background Ventricular arrhythmias (VAs) represent a critical issue with regard to sports eligibility assessment in athletes. The ideal diagnostic evaluation of competitive and leisure-time athletes with complex VAs has not been clearly defined. Objective The purpose of this study was to assess the clinical implications of invasive electrophysiological assessments and endomyocardial biopsy (EMB) among athletes with VAs. Methods We evaluated 227 consecutive athletes who presented to our institutions after being disqualified from participating in sports because of VAs. After noninvasive tests, electrophysiological study (EPS), electroanatomic mapping (EAM), and EAM- or cardiac magnetic resonance imaging–guided EMB was performed, following a prespecified protocol. Sports eligibility status was redefined at 6-month follow-up. Results From our sample, 188 athletes (82.8%) underwent EAM and EPS, and 42 (15.2%) underwent EMB. A diagnosis of heart disease could be formulated in 30% of the study population (67/227; 95% confidence interval [CI] 0.24–0.36) after noninvasive tests; in 37% (83/227; 95% CI 31%–43%) after EPS and EAM; and in 45% (102/227; 95% CI 39%–51%) after EMB. In the subset of athletes undergoing EMB, invasive diagnostic workup allowed diagnostic reclassification of half of the athletes (n = 21 [50%]). Reclassification was particularly common among subjects without definitive findings after noninvasive evaluation (n = 23; 87% reclassified). History of syncope, abnormal echocardiogram, presence of late gadolinium enhancement, and abnormal EAM were linked to sports ineligibility at 6-month follow-up. Conclusion A comprehensive invasive workup provided additional diagnostic elements and could improve the sports eligibility assessment of athletes presenting with VAs. The extensive invasive evaluation presented could be especially helpful when noninvasive tests show unclear findings.

Background Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by small vessel vasculopathy, autoantibodies production and exaggerated extracellular matrix deposition, leading to extensive tissue fibrosis. Cardiac involvement in SSc, albeit often asymptomatic, is frequent and represents a negative prognostic factor. Speckle tracking global longitudinal strain (GLS) has proved itself to be an effective tool to identify the presence and the progression of subclinical SSc-related cardiomyopathy. The aim of our study was to assess whether SSc-related cardiomyopathy affects not only the ventricles but also the right (RA) and left atria (LA) in patients with SSc and no overt cardiac disease nor pulmonary hypertension. Materials and methods Observational prospective study enrolling all consecutive patients with SSc age- and gender-matched 1:1 to healthy controls. Patients with structural heart disease, heart failure, atrial fibrillation and pulmonary hypertension were excluded. For every patient, standard echocardiographic parameters and speckle-tracking derived variables were registered. The reservoir function (from the end of ventricular contraction to mitral valve opening), conduit function (from mitral valve opening through the onset of atrium contraction) and contraction function (from the onset of atrium contraction to the end of ventricular diastole) were assessed via GLS. Zero strain reference was set at left ventricular end diastole. Results Fifty-two SSc patients and 52 matched controls were consecutively enrolled. Left ventricular ejection fraction (66.5%±7.4% vs. 66.1%±5.9%; p=ns) right fractional area change (49.4%±9.6% vs. 49.2%±9.2%; p=ns) and mean sPAP (29.0%±5.3% vs. 24.4%±4.1%; p=ns) were well within the normal range and similar between SSc patients and controls. Right atrial reservoir function (35.0%±7.3% vs. 42.3%±8.5%; p=.024) and contraction function (14.8%±4.3% vs. 18.5%±4.1%; p=.034) were significantly lower in SSc patients when compared to matched controls. No difference was seen in right atrial conduit function or left atrial strain. In patients with SSc, RA reservoir (r=.194; p=.033) and conduit function (r=.174; p=.036) were directly associated to right ventricular GLS. LA reservoir (r=.260; p=.008) and conduit function (r=.271; p=.006) were directly associated with left ventricular GLS. No association was observed between contraction function and GLS in both left and right chambers. Moreover, RA and LA reservoir (r=.358; p=.02), conduit (r=.525; p=.004) and contraction functions (r=.30; p=.0.18) were directly correlated. Conclusions While no significant difference was seen between cases and controls in terms of common echocardiographic parameters, RA reservoir and contraction function assessed through GLS were significantly impaired in patients with SSc. The correlation between impaired atrial and ventricular GLS in SSc may represent another indirect evidence of SSc-related heart global involvement. Funding Acknowledgement Type of funding sources: None.

Background Tachycardiomyopathy (TCM) is a reversible cause of left ventricular dysfunction, secondary to rapid and/or asynchronous, irregular myocardial contraction. Two categories of the disease have been described: arrhythmia-induced TCM (pure TCM), where the arrhythmia is the sole reason for the dysfunction, and arrhythmia-mediated TCM (impure TCM), where the arrhythmia can exacerbate or worsen heart failure (HF) or an underlying heart disease. Pure TCM has already been described as affecting almost 1 out of 10 patients admitted for acute HF-like symptoms. On the other side, acute HF leads to a chronic irreversible condition which tends to worsen over time and is burdened by a high rate of complications. Purpose The aim of our study was to compare pure TCM and de novo acute HF patients (including all forms of structural heart disease) in terms of mortality and cardiovascular (CV)-related hospitalizations. Methods Prospective, observational study enrolling all consecutive patients with a confirmed diagnosis of TCM and all patients admitted for de novo acute HF. The TCM diagnosis was suspected in all patients admitted for HF-related symptoms, an ejection fraction Results One-hundred-and-ten patients with TCM (61.8% males, 68±13 years old) were propensity matched with a control population of patients with HF and structural heart disease (76.6% males, 71±15 years old, 75% ischemic heart disease). After a median follow-up of 5.1 years (1st-3rd quartile 2.6–7.0 years) TCM patients showed an overall higher estimate of survival when compared to HF patients (78% vs. 58%; p=0.031; figure 1A) but a lower estimate of time free from CV-related hospitalization (31% vs. 57%, p=0.014; figure 1B). TCM patients got most often readmitted for AF-related elective procedures (60.8% of all hospitalizations), TCM recurrence (13.7%) and elective coronary angiography (5.9%). On the other hand, HF patients got readmitted for HF worsening (40.9%), cardiac or vascular surgery (22.7%) and elective coronary angiography (9.1%). Propensity-score matched analysis confirmed the results for all-cause death (81% vs. 49%; p=0.006) and CV-related hospitalizations (29% vs. 54%; p=0.007). Conclusions TCM is associated with a better survival when compared to de novo acute HF, even after propensity score adjustment. On the other hand, patients with TCM got readmitted more frequently, requiring more often elective procedures in order to control the triggering arrhythmia. Funding Acknowledgement Type of funding sources: None.

Background Both primary (SSc related cardiomyopathy) and secondary cardiac involvement in systemic sclerosis (SSc) is frequent albeit mostly asymptomatic. It represents a negative prognostic factor as almost 25% of SSc patients die from either heart failure or arrhythmia complications. Speckle tracking global longitudinal strain has been proven to be an effective tool both to identify and detect the progress of subclinical heart disease in SSc. The aim of our study was to assess the association between SSc diagnosis and the development of heart failure, pulmonary hypertension, death and need for hospitalization. Materials and methods We conducted an observational prospective study enrolling all patients with a diagnosis of SSc and no overt cardiac disease. We excluded all patients with a known diagnosis of pulmonary hypertension and atrial fibrillation. For each patient standard echocardiogram and GLS variables were collected. Results We enrolled 70 patients (61 females, age 56.2±15.4 years) who were followed for a median of 3 years. 68% of the patients had a limited variant of the disease. All-cause mortality was 10% in a 5-year follow-up. During the same period, PH was reached in 13% of all patients, HF in 7% and 18% required at least one hospital admission for cardiovascular causes. A diagnosis of PH was associated with an increased risk of death (ARR 34%; p Conclusions Pulmonary hypertension and heart failure often occur in patients with cardiac involvement. Both proved themselves to be associated with an increased risk of death and hospitalization for CV causes. Moreover, GLS worsening of both the left and right ventricles may allow us to predict the diagnosis of PH and therefore preemptively start appropriate management. Funding Acknowledgement Type of funding sources: None.

Background: Atrial arrhythmias are present in up to 20% of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Catheter ablation (CA) is an effective treatment for atrial arrhythmias in the general population. Data regarding CA for atrial arrhythmias in ARVC are scarce. Objective: To assess the safety and efficacy of CA for atrial arrhythmias in patients with ARVC. Methods: In this international collaborative effort, all patients with a definite diagnosis of ARVC undergoing CA for atrial fibrillation (AF), focal atrial tachycardia (AT), or cavotricuspid isthmus (CTI)-dependent atrial flutter (AFl) were extracted from twelve ARVC registries. Demographic, periprocedural, and long-term arrhythmic outcome data were collected. Results: Thirty-seven patients were enrolled in the study (age 50.2 ± 16.6 years, male 84%, CHA2DS2VASc 1 (1,2), HAS-BLED 0 (0–2)). The arrhythmia leading to CA was AF in 23 (62%), focal left AT in 5 (14%), and CTI-dependent AFl in 9 (24%). Acute procedural success was achieved in all procedures but one (n = 1 focal left AT, 97% acute success). The median follow-up period was 27 (13–67) months, and 96%, 74%, and 61% of patients undergoing AF ablation were free from any atrial arrhythmia recurrence after a single procedure at 6 months, 12 months, and last follow-up, respectively. After focal AT ablation, freedom from atrial arrhythmia recurrence was 80%, 80%, and 60% at 6 months, 12 months, and last follow-up, respectively. All patients undergoing CTI ablation were free from atrial arrhythmia recurrences at 6 months, with 89% single-procedural arrhythmic freedom at last follow-up. One major complication (2.7%, PV stenosis requiring PV stenting) occurred. Conclusions: CA is safe and effective in managing atrial arrhythmias in patients with ARVC, with success rates comparable to the general population.

Arrhythmogenic cardiomyopathy (ACM) is hallmarked by ventricular fibro‐adipogenic alterations, contributing to cardiac dysfunctions and arrhythmias. Although genetically determined (e.g., PKP2 mutations), ACM phenotypes are highly variable. More data on phenotype modulators, clinical prognosticators, and etiological therapies are awaited. We hypothesized that oxidized low‐density lipoprotein (oxLDL)‐dependent activation of PPARγ, a recognized effector of ACM adipogenesis, contributes to disease pathogenesis. ACM patients showing high plasma concentration of oxLDL display severe clinical phenotypes in terms of fat infiltration, ventricular dysfunction, and major arrhythmic event risk. In ACM patient‐derived cardiac cells, we demonstrated that oxLDLs are major cofactors of adipogenesis. Mechanistically, the increased lipid accumulation is mediated by oxLDL cell internalization through CD36, ultimately resulting in PPARγ upregulation. By boosting oxLDL in a Pkp2 heterozygous knock‐out mice through high‐fat diet feeding, we confirmed in vivo the oxidized lipid dependency of cardiac adipogenesis and right ventricle systolic impairment, which are counteracted by atorvastatin treatment. The modulatory role of oxidized lipids on ACM adipogenesis, demonstrated at cellular, mouse, and patient levels, represents a novel risk stratification tool and a target for ACM pharmacological strategies., ACM mutations are necessary but not sufficient for disease penetrance. The contribution of oxidised lipids as novel pharmacologically targetable cofactors was demonstrated by a multi‐layer approach (patients – in vitro – in vivo), leading to an advancement in the knowledge of ACM pathogenesis.

INTRODUCTION The PRAETORIAN score (PS) was developed to assess the implant position and predict defibrillation success of the subcutaneous implantable cardioverter defibrillators (S-ICD). The main critique moved to the routine use of PS has been its postprocedural timing, that limits its usefulness on procedure guidance. The aim of this proof-of-concept study was to assess the feasibility of an intraprocedural use of PS. METHODS Forty consecutive patients undergoing S-ICD implantation were enrolled. Intraprocedural PS (IP-PS) obtained with fluoroscopy before closure of the pocket and postprocedural PS (PP-PS) obtained with two-views chest X-ray were compared. Intraprocedural data and PS were compared with the historic cohorts of the involved institutions. RESULTS When assessing IP-PS and PP-PS, a complete overall agreement was observed (100%, 1.00-κ; p

Complete atrioventricular block (CAVB) is a total dissociation between the atrial and ventricular activity, in the absence of atrioventricular conduction. Several diseases may result in CAVB in the pediatric and young-adult population. Permanent right ventricular (RV) pacing is required in permanent CAVB, when the cause is neither transient nor reversible. Continuous RV apical pacing has been associated with unfavorable outcomes in several studies due to the associated ventricular dyssynchrony. This study aims to summarize the current literature regarding CAVB in the pediatric and young adult population and to explore future treatment perspectives.

Background Premature ventricular complexes (PVCs) are the most common form of ventricular arrhythmia in the general population. While in most cases PVCs represent a primitive phenomenon with benign behavior, in a non-negligible proportion of subjects frequent PVCs may be epiphenomenon of underlying occult heart diseases, requiring special medical attention since they have been resulted linked to increased total and cardiac mortality. Nevertheless, PVCs themselves, when incessantly frequent, may be responsible for left ventricular dysfunction in otherwise normal heart. Aim of this narrative review is to update current knowledge on the general approach to patients with frequent PVCs on the basis of available data, with a special focus on the value of imaging. Hypothesis Routine diagnostic work-up not infrequently miss subtle concealed arrhythmic substrate, leading to erroneously refer to such arrhythmias as to "idiopathic". Methods Literature search of PVCs articles was conducted in PubMed and Scopus electronic database. Results Conflicting data arise from literature about the true clinical significance of idiopathic PVCs. There is growing body of data providing evidence that more advanced non-invasive imaging modalities, such as cardiac magnetic resonance, have an incremental diagnostic and prognostic value. On the other hand, in some cases the prognostic significance of isolated subtle myocardial structural abnormalities in patients with PVCs, still remains area of uncertainty. Conclusion In selected subjects with PVCs and high-risk features for concealed arrhythmic substrate, traditional assessment to rule out the presence of heart disease, including surface ECG and transthoracic echocardiography, should be implemented with more advanced cardiovascular imaging modalities.

Background The combined left atrial appendage closure (LAAC) and cryoenergy pulmonary vein isolation (PVI) procedure has been proven safe and effective in managing stroke in patients with nonvalvular atrial fibrillation (AF), although most data refer to procedures performed using radiofrequency as the main energy source. Objective The purpose of this study was to evaluate long-term follow-up of patients with AF undergoing concomitant LAAC and cryoenergy PVI. Methods Patients undergoing LAAC and cryoballoon PVI at our institution were enrolled. At 3, 6, and 24 months from the index procedure, we determined the atrial arrhythmia recurrence rate, the extent of LAAC, and the rate of cerebrovascular/bleeding events. Results Forty-nine patients (mean age 69 ± 8 years; 32/49 (67%) men; CHA2DS2-VASc score 2.8 ± 1.2; HAS-BLED score 3 ± 1) with a guideline-recommended LAAC indication were included. Acute PVI and complete LAAC were achieved in 100% of patients. All patients completed at least 24 months of follow-up. At 8 weeks and 6 months, complete or satisfactory ( Conclusion Concomitant cryoballoon ablation and LAAC procedures appear safe and effective at long-term follow-up, with high antithrombotic drug withdrawal rates at 24 months.

Transcather aortic valve implantation (TAVI) has become a safe and indispensable treatment option for patients with severe symptomatic aortic stenosis who are at high surgical risk. Recently, outcomes after TAVI have improved significantly and TAVI has emerged as a qualified alternative to surgical aortic valve replacement in the treatment of intermediate risk patients and greater adoption of this procedure is to be expected in a wider patients population, including younger patients and low surgical risk patients. However since the aortic valve has close spatial proximity to the conduction system, conduction anomalies are frequently observed in TAVI. In this article, we aim to review the key aspects of pathophysiology, current incidence, predictors and clinical association of conduction anomalies following TAVI.

BACKGROUND The novel fourth-generation cryoballoon (CB4) potentially allows for enhanced catheter maneuverability and more frequent capture of pulmonary vein (PV) potentials which can be used to monitor real-time PV isolation (PVI). The aim of our study is to compare the acute procedural endpoints between the CB4 and second-generation cryoballoon (CB2). METHODS A single-center retrospective chart review was used to examine 50 consecutive patients with drug-refractory atrial fibrillation undergoing CB4-based PVI. Procedural data and acute success of these patients were compared to 50 propensity-matched controls who underwent cryoballoon ablation procedure using CB2. RESULTS Procedures performed with the CB4 showed significant shorter fluoroscopy time (14.8 ± 5.5 vs 18.0 ± 6.5 minutes, P = .04), shorter procedure time (58.3 ± 15.7 vs 65.3 ± 21 minutes, P = .13), and shorter total ablation time (10.8 ± 1.5 vs 13.8 ± 1.9 minutes, P = .42). The real-time PVI visualization rate was 33.3% in the CB2 group and 74.7% in the CB4 group (P

Background Nowadays, transvenous lead extraction (TLE) is considered an essential technique in lead management strategy. Since 2011, a multidisciplinary approach was undertaken in our centre involving electrophysiologists, cardiac surgeons and anaesthesiologists to improve cross- unit cooperation and minimize complications and mortality. The present paper reports procedural outcomes and complications of our lead extraction experience. Methods We retrospectively collected and analysed data from all consecutive patients undergoing cardiac implantable electronic device leads TLE at the IRCCS Centro Cardiologico Monzino between January 2011 and November 2017. Results One-hundred fifty patients (111 males, 68 ± 13 years) underwent extraction procedures. The most common extraction indication were infections (86.7%) and TLE was carried out by laser-based approach in 88 (58.6%) patients, by mechanical dilating sheaths in 58 (38.7%) patients and by a combined approach (TLE + open surgical intervention) in 4 (2.7%) patients. Procedural success was obtained in 146 (97.3%) cases with only 3 (2.0%) major complications with 2 cases of structural injury with tamponade requiring emergent median sternotomy. Open surgery extraction was required in 4 patients, after an attempt to TLE, due to leads strict adhesion to cardiac or vascular structures, whereas in 5 (3.3%) cases, the treatment of choice was a combined approach consisting in transvenous leads extraction followed by planned surgery. Conclusions TLE is a complex procedure that sometimes leads to fatal complications. In our single center experience, a multidisciplinary approach involving electrophysiologist, cardiac surgeon, anaesthesiologist in an operating room allows a safer approach and major complications treatment.

Aims Radiation use in medicine has significantly increased over the last decade, and cardiologists are among the specialists most responsible for X-ray exposure. The present study investigates a broad range of aspects, from specific European Union directives to general practical principles, related to radiation management among a national cohort of cardiologists. Methods and results A voluntary 31-question survey was run on the Italian Arrhythmology and Pacing Society (AIAC) website. From June 2019 to January 2020, 125 cardiologists, routinely performing interventional electrophysiology, participated in the survey. Eighty-seven (70.2%) participants are aware of the recent European Directive (Euratom 2013/59), although only 35 (28.2%) declare to have read the document in detail. Ninety-six (77.4%) participants register the dose delivered to the patient in each procedure, in 66.1% of the cases both as fluoroscopy time and dose area product. Years of exposition (P = 0.009) and working in centers performing pediatric procedures (P = 0.021) related to greater degree of X-ray equipment optimization. The majority of participants (72, 58.1%) did not recently attend radioprotection courses. The latter is related to increased awareness of techniques to reduce radiation exposure (96% vs. 81%, P = 0.022), registration of the delivered dose in each procedure (92% vs. 67%, P = 0.009), and X-ray equipment optimization (50% vs. 36%, P = 0.006). Conclusion Italian interventional cardiologists show an acceptable level of radiation awareness and knowledge of updated European directives. However, there is clear space for improvement. Comparison to other health professionals, both at national and international levels, is needed to pursue proper X-ray management and protect public health.

The “Extreme Exercise Hypothesis” states that when individuals perform training beyond the ideal exercise dose, a decline in the beneficial effects of physical activity occurs. This is due to significant changes in myocardial structure and function, such as hemodynamic alterations, cardiac chamber enlargement and hypertrophy, myocardial inflammation, oxidative stress, fibrosis, and conduction changes. In addition, an increased amount of circulating biomarkers of exercise-induced damage has been reported. Although these changes are often reversible, long-lasting cardiac damage may develop after years of intense physical exercise. Since several features of the athlete’s heart overlap with arrhythmogenic cardiomyopathy (ACM), the syndrome of “exercise-induced ACM” has been postulated. Thus, the distinction between ACM and the athlete’s heart may be challenging. Recently, an autoimmune mechanism has been discovered in ACM patients linked to their characteristic junctional impairment. Since cardiac junctions are similarly impaired by intense physical activity due to the strong myocardial stretching, we propose in the present work the novel hypothesis of an autoimmune response in endurance athletes. This investigation may deepen the knowledge about the pathological remodeling and relative activated mechanisms induced by intense endurance exercise, potentially improving the early recognition of whom is actually at risk.

Background To date, only a few comparisons between subcutaneous implantable cardioverter-defibrillator (S-ICD) patients undergoing and those not undergoing defibrillation testing (DT) at implantation (DT+ vs DT–) have been reported. Objective The purpose of this study was to compare long-term clinical outcomes of 2 propensity-matched cohorts of DT+ and DT– patients. Methods Among consecutive S-ICD patients implanted across 17 centers from January 2015 to October 2020, DT– patients were 1:1 propensity-matched for baseline characteristics with DT+ patients. The primary outcome was a composite of ineffective shocks and cardiovascular mortality. Appropriate and inappropriate shock rates were deemed secondary outcomes. Results Among 1290 patients, a total of 566 propensity-matched patients (283 DT+; 283 DT–) served as study population. Over median follow-up of 25.3 months, no significant differences in primary outcome event rates were found (10 DT+ vs 14 DT–; P = .404) as well as for ineffective shocks (5 DT– vs 3 DT+; P = .725). At multivariable Cox regression analysis, DT performance was associated with a reduction of neither the primary combined outcome nor ineffective shocks at follow-up. A high PRAETORIAN score was positively associated with both the primary outcome (hazard ratio 3.976; confidence interval 1.339–11.802; P = .013) and ineffective shocks alone at follow-up (hazard ratio 19.030; confidence interval 4.752–76.203; P = .003). Conclusion In 2 cohorts of strictly propensity-matched patients, DT performance was not associated with significant differences in cardiovascular mortality and ineffective shocks. The PRAETORIAN score is capable of correctly identifying a large percentage of patients at risk for ineffective shock conversion in both cohorts.

Recently, the Food and Drug Administration issued a recall for the subcutaneous implantable cardioverter-defibrillator (S-ICD) because of the possibility of lead ruptures and accelerated battery depletion.The aim of this study was to evaluate device-related complications over time in a large real-world multicenter S-ICD cohort.Patients implanted with an S-ICD from January 2015 to June 2020 were enrolled from a 19-institution European registry (Experience from the Long-term Italian S-ICD registry [ELISIR]; ClinicalTrials.gov identifier NCT0473876). Device-related complication rates over follow-up were collected. Last follow-up of patients was performed after the Boston Scientific recall issue.A total of 1254 patients (median age 52.0 [interquartile range 41.0-62.2] years; 973 (77.6%) men; 387 (30.9%) ischemic) was enrolled. Over a follow-up of 23.2 (12.8-37.8) months, complications were observed in 117 patients (9.3%) for a total of 127 device-related complications (23.6% managed conservatively and 76.4% required reintervention). Twenty-seven patients (2.2%) had unanticipated generator replacement after 3.6 (3.3-3.9) years, while 4 (0.3%) had lead rupture. Body mass index (hazard ratio [HR] 1.063 [95% confidence interval 1.028-1.100]; P.001), chronic kidney disease (HR 1.960 [1.191-3.225]; P = .008), and oral anticoagulation (HR 1.437 [1.010-2.045]; P = .043) were associated with an increase in overall complications, whereas older age (HR 0.980 [0.967-0.994]; P = .007) and procedure performed in high-volume centers (HR 0.463 [0.300-0.715]; P = .001) were protective factors.The overall complication rate over 23.2 months of follow-up in a multicenter S-ICD cohort was 9.3%. Early unanticipated device battery depletions occurred in 2.2% of patients, while lead fracture was observed in 0.3%, which is in line with the expected rates reported by Boston Scientific.

Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): The Zurich ACM Program is supported by generous grants from the Georg and Bertha Schwyzer-Winniker Foundation, the Baugarten Foundation, Swiss National Science Foundation, Swiss Heart Foundation and Wild Foundation. This work is also supported by a Canadian Institutes of Health Research grant (FRN: 162402) and the Labatt Heart Centre and Waugh Family Innovation Funds, Caitlin Elizabeth Morris Memorial Fund, Alex Corrance Memorial Foundation and Meredith Cartwright. BACKGROUND Arrhythmogenic right ventricular cardiomyopathy (ARVC) has several phenocopies such as cardiac sarcoidosis (CS), idiopathic outflow tract ventricular tachycardia (OT-VT) and myocarditis. Differentiation between these entities can be challenging. Recently, we have identified diagnostic anti-desmoglein-2 autoantibodies (anti-DSG2 Abs) in patients with ARVC. PURPOSE We sought to examine whether anti-DSG2 Abs are also present in clinical phenocopies of ARVC. METHODS Anti-DSG2 Abs in sera of 25, 19 and 22 patients with sarcoidosis, OT-VT and myocarditis, respectively, were assessed by western blots and ELISA. Clinical and imaging parameters, as well as conventional biomarkers were correlated to detected anti-DSG2 Ab intensity levels. RESULTS Anti-DSG2 Abs, at various intensities, were identified in 6/25 (24%) patients with sarcoidosis, all presenting with CS, but were absent in patients with OT-VT and myocarditis. Cardiac 18F- fluorodeoxyglucose positron emission tomography (18F-FDG PET) was positive in all sarcoidosis patients with positive anti-DSG2 Abs, corresponding to a median PET maximum standardized uptake value (SUVmax) of 5.65 [IQR: 5.15 – 10.9]. In sarcoidosis patients without anti-DSG2 Abs, the SUVmax values were significantly lower with a median of 0 [IQR: 0 – 4] (p = 0.011). The Pearson correlation coefficient (R) was 0.188 (p = 0.039) indicating a positive correlation between cardiac 18F-FDG uptake and anti-DSG2 Abs. No significant correlation was detected for any of the other clinical parameters and biomarkers. CONCLUSIONS In addition to being present in ARVC, anti-DSG2 Abs are also found in CS, a common phenocopy of ARVC; conversely, anti-DSG2 Abs are absent in idiopathic OT-VT and myocarditis. Anti-DSG2 Ab levels positively correlate with myocardial disease activity in CS as indicated by cardiac 18F-FDG PET scanning. Abstract Figure. Central illustration

Funding Acknowledgements Type of funding sources: None. BACKGROUND Postoperative atrial fibrillation (POAF) is defined as de novo onset of atrial fibrillation in the post operative period, in patients with no previous history of atrial fibrillation. It affects almost 3% of all over 45 year old patients undergoing non cardiovascular surgery and is associated with a higher risk of stroke and mortality. PURPOSE To assess independent predictors of POAF in elective non-cardiac surgery. METHODS Retrospective observational database including all patients attending a cardiological preoperative assessment from the 1st of January 2016 to the 31st of December 2019. The primary endpoint was the occurrence of POAF. Individual predictors of the primary endpoint were tested through a series of multivariable logistic regression models, including only those variables with a p RESULTS A total of 2048 patients were enrolled (1350 men, age 71 ± 12 years). Fourty-four patients experienced POAF (2.1%) – median 3 days (1st-3rd quartile 2-3 days). Age (OR 1.03 for each year; 95% CI 1.01-1.07), hypertension (OR 3.43; 95% CI 1.22-9.63), thyroid dysfunction (OR 2.70; 95% CI 1.35-5.42) and intermediate or high risk surgery (OR 18.28; 95% CI 2.51-33.09) resulted as independent predictors of POAF (all p 0.001). A cut-off score ≥ 5 has a 70% sensitivity and a 72% specificity in detecting POAF in our population (AUC 0.74). Bootstrapping for internal validation confirmed the overall results. CONCLUSIONS A four items point-based risk score such as the HART score, could be effective in implementing effective POAF screening and improve management. The HART score Variable Points H Hypertension 1 point A Age (65-74 years) 1 point Age (75+ years) 2 points R Intermediate Risk surgery 3 points High Risk surgery 3 points T Thyroid dysfunction 1 point

Background We evaluated long‐term outcome of isolation of pulmonary veins, left atrial posterior wall, and superior vena cava, including time to recurrence and prevalent triggering foci at repeat ablation in patients with paroxysmal atrial fibrillation with or without cardiovascular comorbidities. Methods and Results A total of 1633 consecutive patients with paroxysmal atrial fibrillation that were arrhythmia‐free for 2 years following the index ablation were classified into: group 1 (without comorbidities); n=692 and group 2 (with comorbidities); n=941. We excluded patients with documented ablation of areas other than pulmonary veins, the left atrial posterior wall, and the superior vena cava at the index procedure. At 10 years after an average of 1.2 procedures, 215 (31%) and 480 (51%) patients had recurrence with median time to recurrence being 7.4 (interquartile interval [IQI] 4.3–8.5) and 5.6 (IQI 3.8–8.3) years in group 1 and 2, respectively. A total of 201 (93.5%) and 456 (95%) patients from group 1 and 2 underwent redo ablation; 147/201 and 414/456 received left atrial appendage and coronary sinus isolation and 54/201 and 42/456 had left atrial lines and flutter ablation. At 2 years after the redo, 134 (91.1%) and 391 (94.4%) patients from group 1 and 2 receiving left atrial appendage/coronary sinus isolation remained arrhythmia‐free whereas sinus rhythm was maintained in 4 (7.4%) and 3 (7.1%) patients in respective groups undergoing empirical lines and flutter ablation ( P Conclusions Very late recurrence of atrial fibrillation after successful isolation of pulmonary veins, regardless of the comorbidity profile, was majorly driven by non‐pulmonary vein triggers and ablation of these foci resulted in high success rate. However, presence of comorbidities was associated with significantly earlier recurrence.

Athlete’s heart (AH) is the result of morphological and functional cardiac modifications due to long-lasting athletic training. Athletes can develop very marked structural myocardial changes, which may simulate or cover unknown cardiomyopathies. The differential diagnosis between AH and cardiomyopathy is necessary to prevent the risk of catastrophic events, such as sudden cardiac death, but it can be a challenging task. The improvement of the imaging modalities and the introduction of the new technologies in cardiac magnetic resonance (CMR) and cardiac computed tomography (CCT) can allow overcoming this challenge. Therefore, the radiologist, specialized in cardiac imaging, could have a pivotal role in the differential diagnosis between structural adaptative changes observed in the AH and pathological anomalies of cardiomyopathies. In this review, we summarize the main CMR and CCT techniques to evaluate the cardiac morphology, function, and tissue characterization, and we analyze the imaging features of the AH and the key differences with the main cardiomyopathies.

Supplemental Digital Content is available in the text., Background: Coronavirus disease 2019 (COVID-19) has led to over 1 million deaths worldwide and has been associated with cardiac complications including cardiac arrhythmias. The incidence and pathophysiology of these manifestations remain elusive. In this worldwide survey of patients hospitalized with COVID-19 who developed cardiac arrhythmias, we describe clinical characteristics associated with various arrhythmias, as well as global differences in modulations of routine electrophysiology practice during the pandemic. Methods: We conducted a retrospective analysis of patients hospitalized with COVID-19 infection worldwide with and without incident cardiac arrhythmias. Patients with documented atrial fibrillation, atrial flutter, supraventricular tachycardia, nonsustained or sustained ventricular tachycardia, ventricular fibrillation, atrioventricular block, or marked sinus bradycardia (heart rate

The prediction and prevention of sudden cardiac death is the philosopher’s stone of clinical cardiac electrophysiology. Sports can act as triggers of fatal arrhythmias and therefore it is essential to promptly frame the athlete at risk and to carefully evaluate the suitability for both competitive and recreational sports activity. A history of syncope or palpitations, the presence of premature ventricular complexes or more complex arrhythmias, a reduced left ventricular systolic function, or the presence of known or familiar heart disease should prompt a thorough evaluation with second level examinations. In this regard, cardiac magnetic resonance and electrophysiological study play important roles in the diagnostic work-up. The role of genetics is increasing both in cardiomyopathies and in channelopathies, and a careful evaluation must be focused on genotype positive/phenotype negative subjects. In addition to being a trigger for fatal arrhythmias in certain cardiomyopathies, sports also play a role in the progression of the disease itself, especially in the case arrhythmogenic right ventricular cardiomyopathy. In this paper, we review the latest European guidelines on sport cardiology in patients with cardiovascular diseases, focusing on arrhythmic risk stratification and the management of cardiomyopathies and channelopathies.

Arrhythmogenic Cardiomyopathy (ACM) is characterized by the replacement of the myocardium with fibrotic or fibro-fatty tissue and inflammatory infiltrates in the heart. To date, while ACM adipogenesis is a well-investigated differentiation program, ACM-related fibrosis remains a scientific gap of knowledge. In this study, we analyze the fibrotic process occurring during ACM pathogenesis focusing on the role of cardiac mesenchymal stromal cells (C-MSC) as a source of myofibroblasts. We performed the ex vivo studies on plasma and right ventricular endomyocardial bioptic samples collected from ACM patients and healthy control donors (HC). In vitro studies were performed on C-MSC isolated from endomyocardial biopsies of both groups. Our results revealed that circulating TGF-β1 levels are significantly higher in the ACM cohort than in HC. Accordingly, fibrotic markers are increased in ACM patient-derived cardiac biopsies compared to HC ones. This difference is not evident in isolated C-MSC. Nevertheless, ACM C-MSC are more responsive than HC ones to TGF-β1 treatment, in terms of pro-fibrotic differentiation and higher activation of the SMAD2/3 signaling pathway. These results provide the novel evidence that C-MSC are a source of myofibroblasts and participate in ACM fibrotic remodeling, being highly responsive to ACM-characteristic excess TGF-β1.

Arrhythmogenic cardiomyopathy (ACM) is a genetic-based cardiac disease accompanied by severe ventricular arrhythmias and a progressive substitution of the myocardium with fibro-fatty tissue. ACM is often associated with sudden cardiac death. Due to the reduced penetrance and variable expressivity, the presence of a genetic defect is not conclusive, thus complicating the diagnosis of ACM. Recent studies on human induced pluripotent stem cells-derived cardiomyocytes (hiPSC-CMs) obtained from ACM individuals showed a dysregulated metabolic status, leading to the hypothesis that ACM pathology is characterized by an impairment in the energy metabolism. However, despite efforts having been made for the identification of ACM specific biomarkers, there is still a substantial lack of information regarding the whole metabolomic profile of ACM patients. The aim of the present study was to investigate the metabolic profiles of ACM patients compared to healthy controls (CTRLs). The targeted Biocrates AbsoluteIDQ® p180 assay was used on plasma samples. Our analysis showed that ACM patients have a different metabolome compared to CTRLs, and that the pathways mainly affected include tryptophan metabolism, arginine and proline metabolism and beta oxidation of fatty acids. Altogether, our data indicated that the plasma metabolomes of arrhythmogenic cardiomyopathy patients show signs of endothelium damage and impaired nitric oxide (NO), fat, and energy metabolism.

The number of patients affected by electrical storm has been continuously increasing in emergency departments. Patients are often affected by multiple comorbidities requiring multidisciplinary interventions to achieve a clinical stability. Careful reprogramming of cardiac devices, correction of electrolyte imbalance, knowledge of underlying heart disease and antiarrhythmic drugs in the acute phase play a crucial role. The aim of this review is to provide a comprehensive overview of pharmacological treatment, latest transcatheter ablation techniques and advanced management of patients with electrical storm.

Background: We aim to evaluate the value of Cardiac magnetic resonance (CMR) feature tracking (CMR-FT) in addition to Task Force Criteria(TFC) in patients with (arrhythmogenic cardiomyopathy) AC biopsy-proved. Methods: Thirty-five patients with AC histologically proven who performed CMR with late gadolinium enhancement (LGE) acquisition were enrolled. The study population was divided in Group1 (negative CMR TFC and LV ejection fraction≥55%) and Group2 (positive CMR TFC and/or LVEF

Background Life-threatening ventricular tachyarrhythmias (VAs) represent a significant cause of death in myocarditis. Objective The purpose of this study was to identify predictors of sustained VAs in patients with myocarditis and ventricular phenotype diagnosed by workflow including endomyocardial biopsy (EMB) guided by 3D electroanatomic mapping (3D-EAM). Methods We prospectively enrolled patients with suspected myocarditis and VAs, undergoing cardiac magnetic resonance imaging, coronary angiography, 3D-EAM, and EMB guided by 3D-EAM. At follow-up, sustained VAs were detected by device interrogation and 24-hour electrocardiographic Holter monitoring. Results We enrolled 54 consecutive patients (mean age 41 ± 14 years; 32(59%) men) with normal ventricular function; left ventricular and right ventricular (RV) late gadolinium enhancement was present, respectively, in 21 (46%) and 6 (13%) of the 46 patients who underwent cardiac magnetic resonance. In 31 patients, the histological diagnosis was myocarditis, while in 14 patients, focal replacement myocardial fibrosis (FRMF); in 9 patients, specimens were inadequate (diagnostic yield of EMB 83%). 3D-EAM showed a larger endocardial scar area for both ventricles in myocarditis than in FRMF (RV bipolar mean scar area 22 ± 16 cm2 vs 3 ± 2 cm2; P = .02; left ventricular bipolar mean scar area 13 ± 5 cm2 vs 4 ± 2 cm2; P = .02, respectively). At a follow-up of 21 months, freedom from sustained VAs was 58% in myocarditis and 92% in FRMF (log-rank, P = .008). Histological diagnosis of myocarditis and RV endocardial scar were independent predictors of sustained VAs (P = .02 for both). Conclusion Our data highlight the need for 3D-EAM–guided EMB in apparently healthy young patients with suspected myocarditis and VAs.

Background Endomyocardial biopsy (EMB) is part of 2010 Task Force Criteria (TFC) for arrhythmogenic right ventricular cardiomyopathy (ARVC). However, its usage has been curtailed because of its low presumed diagnostic yield, and it is now a poorly used tool. This study aims to analyze the contribution of EMB to the final diagnosis of ARVC. Methods and Results We included 104 consecutive patients evaluated for a suspicion of ARVC, who were referred for EMB. Patients with suspected left dominant pattern were excluded from the primary analysis. Subjects were initially stratified according to TFC without considering EMB. After EMB, patients were reclassified accordingly, and the reclassification rate was calculated. EMB yielded a diagnostic finding in 92 patients (85.5%). After including EMB evaluation, 20 (43%) more patients “at risk” received a definite diagnosis of ARVC. Overall, 59 patients received a definite diagnosis of ARVC, 34% only after EMB. EMB appeared to be the better‐performing exam with respect to the final diagnosis (β, 2.2; area uder the curve, 0.73; P P Conclusions Electroanatomic voltage mapping–guided EMB was safe and yielded an optimal diagnostic yield. It allowed upgrading of the diagnosis of nearly one‐third of the patients considered “at risk.” Classical TFC without EMB performed poorly in patients with the left dominant form of ARVC.

Background Arrhythmogenic Cardiomyopathy (AC) is a familial cardiac disease, mainly caused by mutations in desmosomal genes. AC hearts show fibro-fatty myocardial replacement, which favors stress-related life-threatening arrhythmias, predominantly in the young and athletes. AC lacks effective therapies, as its pathogenesis is poorly understood. Recently, we showed that cardiac Mesenchymal Stromal Cells (cMSCs) contribute to adipose tissue in human AC hearts, although the underlying mechanisms are still unclear. Purpose We hypothesize that the sympathetic neurotransmitter, Neuropeptide Y (NPY), participates to cMSC adipogenesis in human AC. Methods For translation of our findings, we combined in vitro cytochemical, molecular and pharmacologic assays on human cMSCs, from myocardial biopsies of healthy controls and AC patients, with the use of existing drugs to interfere with the predicted AC mechanisms. Sympathetic innervation was inspected in human autoptic heart samples, and NPY plasma levels measured in healthy and AC subjects. Results AC cMSCs expressed higher levels of pro-adipogenic isotypes of NPY-receptors (i.e. Y1-R, Y5-R). Consistently, NPY enhanced adipogenesis in AC cMSCs, which was blocked by FDA-approved Y1-R and Y5-R antagonists. AC-associated PKP2 reduction directly caused NPY-dependent adipogenesis in cMSCs. In support of the involvement of sympathetic neurons (SNs) and NPY in AC myocardial remodeling, patients had elevated NPY plasma levels and, in human AC hearts, SNs accumulated in fatty areas and were close to cMSCs. Conclusions Independently from the disease origin, AC causes in cMSCs a targetable gain of responsiveness to NPY, which leads to increased adipogenesis, thus playing a role in AC myocardial remodeling.

Ventricular tachycardia is a major health issue in patients with structural heart disease (SHD). Implantable cardioverter defibrillator (ICD) therapy has significantly reduced the risk of sudden cardiac death (SCD) in such patients, but on the other hand, it has led to frequent ICD shocks as an emerging problem, being associated with poor quality of life, frequent hospitalizations and increased mortality. Myocardial scar plays a central role in the genesis and maintenance of re-entrant arrhythmias, as the coexistence of surviving myocardial fibres within fibrotic tissue leads to the formation of slow conduction pathways and to a dispersion of activation and refractoriness that constitutes the milieu for ventricular tachycardia circuits. Catheter ablation has repeatedly proven to be well tolerated and highly effective in treating VT and in the last two decades has benefited from continuous efforts to determine ventricular tachycardia mechanisms by integration with a wide range of invasive and noninvasive imaging techniques such as intracardiac echocardiography, cardiac magnetic resonance, multidetector computed tomography and nuclear imaging. Cardiovascular imaging has become a fundamental aid in planning and guiding catheter ablation procedures by integrating structural and electrophysiological information, enabling the ventricular tachycardia arrhythmogenic substrate to be characterized and effective ablation targets to be identified with increasing precision, and allowing the development of new ablation strategies with improved outcomes. In this review, we provide an overview of the role of cardiac imaging in patients undergoing catheter ablation of ventricular tachycardia.

Multiple studies have addressed the importance of anteroseptal scar in patients with nonischemic cardiomyopathy. However, this pattern has never been fully evaluated in patients with prior myocarditis.The purpose of this study was to evaluate whether anteroseptal scar is associated with worse outcome in patients with prior myocarditis and how it affects the efficacy of catheter ablation (CA).This was a retrospective study of consecutive patients with prior myocarditis and arrhythmic presentation. Cardiac magnetic resonance and electroanatomic voltage mapping were used to identify the scar pattern. Patients were referred for either CA or escalated antiarrhythmic drug (AAD) therapy. The main outcome was ventricular arrhythmia (VA)-free survival according to the presence of anteroseptal scar.A total of 144 consecutive patients with prior myocarditis were included. Mean age was 42.1 ± 14.9 years, and 58% were men. Ejection fraction was normal in 73% of patients. Anteroseptal scar was present in 44% of cases. Sixty-one patients (42%) underwent CA. Overall, at 2-year follow-up, VA-free survival was 77% in the CA group. After CA, the mean number of AADs taken by each patient decreased from 1.8 to 0.9 per day (p0.001). The presence of anteroseptal scar was found to be an independent predictor of VA relapse both in patients treated with CA (hazard ratio [HR] 3.6; 95% confidence interval [CI] 1.1-11.4; P = .03) and in the overall population (HR 2.0; 95% CI 1.2-3.5; P = .02) .In patients with prior myocarditis and VA, the presence of anteroseptal scar negatively predicts outcomes irrespective of treatment strategy.

Background:Arrhythmogenic left ventricular cardiomyopathy (ALVC) is an under-characterized phenotype of arrhythmogenic cardiomyopathy involving the LV ab initio. ALVC was not included in the 2010 International Task Force Criteria for arrhythmogenic right ventricular cardiomyopathy diagnosis and data regarding this phenotype are scarce.Methods:Clinical characteristics were reported from all consecutive patients diagnosed with ALVC, defined as a LV isolated late gadolinium enhancement and fibro-fatty replacement at cardiac magnetic resonance plus genetic variants associated with arrhythmogenic right ventricular cardiomyopathy and of an endomyocardial biopsy showing fibro-fatty replacement complying with the 2010 International Task Force Criteria in the LV.Results:Twenty-five patients ALVC (53 [48–59] years, 60% male) were enrolled. T wave inversion in infero-lateral and left precordial leads were the most common ECG abnormalities. Overall arrhythmic burden at study inclusion was 56%. Cardiac magnetic resonance showed LV late gadolinium enhancement in the LV lateral and posterior basal segments in all patients. In 72% of the patients an invasive evaluation was performed, in which electroanatomical voltage mapping and electroanatomical voltage mapping-guided endomyocardial biopsy showed low endocardial voltages and fibro-fatty replacement in areas of late gadolinium enhancement presence. Genetic variants in desmosomal genes (desmoplakin and desmoglein-2) were identified in 12/25 of the cohort presenting pathogenic/likely pathogenic variants. A definite/borderline 2010 International Task Force Criteria arrhythmogenic right ventricular cardiomyopathy diagnosis was reached only in 11/25 patients.Conclusions:ALVC presents with a preferential involvement of the lateral and postero-lateral basal LV and is associated mostly with variants in desmoplakin and desmoglein-2 genes. An amendment to the current International Task Force Criteria is reasonable to better diagnose patients with ALVC.

Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a leading cause of sudden cardiac death, but its diagnosis is challenging and the role of endomyocardial biopsy (EMB) is controversial and has been recently questioned. Purpose We aimed to 1) analyse the role of EMB in improving the diagnostic performance of 2010 Task Force Criteria (TFC) in the diagnosis of ARVC; 2) assess EMB safety in our population. Methods We retrospectively analysed data from 54 consecutive patients admitted to our Hospital with a clinical suspicion of ARVC undergoing endomyocardial biopsy. During hospitalization a complete assessment was performed for every patient (including electrocardiogram, echocardiogram, cardiac MRI, genetic analysis, and electroanatomic-mapping-guided endomyocardial biopsy). ARVC diagnosis was assessed for every patient using both traditional 2010 TFC and a non-invasive modified TFC (2010 TFC criteria excluding biopsy). Results Overall, 9/54 (17%) patients showed a left-dominant variant of ARVC and were therefore excluded from the analysis. Non-invasive modified TFC allowed 16/45 (36%) patients to receive a definite diagnosis; when biopsy results were added the number of definite diagnosis increased to 22/45 (49%), increasing the number of patients with a definite diagnosis by 13%. More specifically: 8/11 patients not reaching a possible diagnosis were reclassified as either possible (4/8) or borderline (4/8); 3/9 patients with a possible diagnosis were reclassified as borderline; 6/9 borderline patients received a definite diagnosis of ARVC. Globally, in 6 out of 29 patients with a non-definite diagnosis, EMB confirmed ARVC diagnosis and 17/45 (38%) patients received an upgrade in their diagnostic status with EMB. Notably, EMB also revealed the coexistence of myocarditis and fibro-fatty replacement in 5/45 (9%) patients. No patient experienced complications related to EMB. Conclusions Endomyocardial biopsy is a safe, reliable, and useful tool for ARVC diagnosis, allowing to upgrade the diagnostic status of 38% of our patients with a suspect of ARVC diagnosis. It should be performed in experienced centers and it should be guided by electro-anatomic mapping, to maximize its diagnostic power. Funding Acknowledgement Type of funding source: None

Background Current arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnostic criteria are mostly based on ventricle function and dimension. Previous studies have reported a significant overlap between ARVC and chronic myocarditis, at non-invasive assessment. Purpose Tto compare biopsy-proven ARVC and myocarditis patients, in order to identify clinical, imaging and invasive electroanatomic voltage mapping (EVM) differences between the two groups. Methods Patients with borderline diagnosis of ARVC or suspected myocarditis underwent compete assessment with cardiac magnetic resonance (CMR). All patients underwent endomyocardial biopsy (EMB) with targeted tissue sampling guided by EVM. All patients with an histological diagnosis of myocarditis or ARVC were included. Results 83 patients were included, divided into 35 (42.2%) ARVC and 48 (57.8%) myocarditis. Among ARVC patients, 25 (71.4%) had right dominant ARVC, 5 (14.3%) left dominant patter and 5 (14.3%) bi-ventricular involvement. Nine patients (23.1%) with suspected clinic diagnosis of ARVC before EMB, received and histological diagnosis of myocarditis. Two (5.7%) patients with suspected myocarditis were proven to have ARVC. When comparing patients with ARVC and patients with myocarditis, univariate analysis showed that age, sex, family history, arrhythmic disorders at presentation and ECG abnormalities were similar between the two groups (P>0.05 for all the variables). There was also no significant difference with regards to bi-ventricular function and dimension at CMR evaluation. More patients with myocarditis resulted positive at late gadolinium enhancement (LGE) evaluation, although non-significantly (P=0.082). Oedema was more frequently present in patients with myocarditis (P=0.01), while adipose tissue infiltration and segmental wall motion abnormalities were more often observed in patients with ARVC (P=0.002 and P Conclusion(s) A significant number of patients who received a clinical diagnosis of Myocarditis or ARVC according to current guidelines, were subsequently reclassified after histological analysis. Patients with ARVC and myocarditis were not distinguishable on the basis of clinical features and ventricular function and dimensions. Conversely, tissue analysis with CMR demonstrated how patient with ARVC had less oedema, more adipose tissue infiltration and had more extensive scar at EVM evaluation. Funding Acknowledgement Type of funding source: None

Introduction Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically inherited disease characterized by fibro-fatty infiltrations (FFI). FFI in ARVC patients usually originates in the ventricles, but recent imaging studies showed FFI at the atrial level as well. Effectiveness of catheter ablation (CA) for atrial arrhythmias (AA) in this subset of patients is currently unknown. Purpose Aim of our study is to describe acute and long-term effectiveness of CA for AA in ARVC patients. Methods Nine ARVC registries from Europe, US, and China were retrospectively searched for ARVC patients undergoing CA for AA (namely: atrial fibrillation (AF), atrial tachycardia (AT), and cavo-tricuspid dependent atrial flutter (CTI-FL)). Baseline, procedural, and long-term outcome data were collected. Results Thirty-five pts (86% male, median CHA2DS2-VASc 1 [1–2], HAS-BLED 1 [0–2], and EHRA scores 2 [2–3]) were enrolled, in which a total of 45 CA procedures for AA were performed (left atrial CA: n=19 AF, n=10 AT; right atrial CA: n=16 CTI). Mean age at AA CA was 48.2±14.8 y.o. At baseline, 63% of pts were on oral anticoagulants (OAC) (n=9 warfarin; n=13 NOAC). Catheter ablation was successful and sinus rhythm obtained at the end of the procedure in all patients, with 2 (6%) AF patients requiring electrical cardioversion. Over a median follow-up of 36 [14–74] months, 12 (27%) pts experienced arrhythmia recurrence (left atrial group: n=6 AF recurrences, n=3 AT recurrences; CTI-FL group: n=1 CTI-FL recurrence; n=1 new AF with previous CTI-dependent flutter ablation), with a 1-year follow-up resulting comparable to what has been reported in the literature for the general population. [Figure 1 and 2]. 61% pts were on OAC at last follow-up. Conclusion Age at the time of CA for AA is about 10 years younger in patients with ARVC as compared to the general population. CA for AA in ARVC pts is safe and effective; surprisingly, long-term CA outcomes for AF and left AT result comparable to those reported in the general population, whereas recurrence rates of CTI-dependent flutter seem to be higher. Funding Acknowledgement Type of funding source: None

Background A myocardial substrate assessment through percutaneous endomyocardial biopsy (EMB) represents an important additional diagnostic test for cardiomyopathies when uncertainties remain after non-invasive evaluation. Yet, extensive application of EMB has been limited by the low sensitivity of biopsies. Electroanatomic voltage mapping (EVM) is a promising modality for guiding Endomyocardial biopsies (EMB). Aim The aim of our study is to evaluate the diagnostic yield of EVM-guided EMB and the role of histological analysis in the diagnosis of patients with suspected cardiomyopathies and arrhythmic presentation. Methods One-hundred and sixty-two consecutive patients undergoing EMB at our Institution from 2010 to 2019 were included. Demographics, clinical data, CMR data and peri-procedural complications were retrospectively retrieved. All procedures were guided by endo-cavitary EVM. According to non-invasive data collected before proceeding with EMB a suspected clinical diagnosis was expressed and compared to histological diagnosis Results One-hundred and sixty-two patients were included in the study. Mean age of the cohort resulted 40.9±14.7 years, with 26.5% of the included patients being females. ECG alterations were present in 51.3% of the population, with the most common abnormality being T wave inversion. Sustained or non-sustained ventricular tachycardia was registered in 51 (31.5%) of the patients, while 44 (27.2%) patients were referred for frequent isolated premature ventricular complex (PVC), and 19 (11.7%) after an episode of an arrhythmogenic syncope or resuscitated cardiac arrest. Suspected ARVC (41.6%) together with acute/chronic myocarditis (28.0%) were the main clinical diagnosis leading to an invasive approach. The sampling site was the right ventricle in 116 (72.5%), the left ventricle in 31 (19.4%), and both ventricles in 13 (8.1%) patients. Biopsy samplings were judged appropriate for histological analysis in 141 (87.0%) patients. Among the analyzed samples, a diagnosis was reached in 120 patients (74.1%). In the remaining 21 cases (25.9%), the analysis yielded nonspecific histologic findings, inconclusive results, or sampling error. The biopsy allowed to confirm the clinical diagnosis in 72 (60.0%) patients, while a different diagnosis was reached in 48 (39.0%) cases (Reclassification are showed Figure 1).In particular of 67 (41.6%) patients suspected for ARVC, only 32 (22.7) reached a confirmation. Conversely, the number of patients with acute/chronic myocarditis augmented from 45 (28.0%) to 47 (33.3%). Conclusion EMB guided by EVM reached a diagnostic yield as high as 74.1%. EMB proved to be a useful tool in the clinical management of patients, as it allowed to correctly reclassify a significant number of patients who would have been misdiagnosed based only on non-invasive assessment. Sankey Diagram Funding Acknowledgement Type of funding source: None

Background Electroanatomic voltage mapping (EVM) is a promising modality for guiding Endomyocardial biopsies (EMB). Previous experiences on this techniques have reported safety and feasibility of this approach. These reports however, resulted limited by sample size or imperfect designs, preventing reliable comparisons of the effectiveness of this new methods with a conventional or a cardiac magnetic resonance (CMR) imaging guided approach. Aim We now report the largest cohort of patients undergoing EVM-guided EMB in order to show its diagnostic yield and comparing it with a cardiac magnetic resonance (CMR) guided approach. Methods One-hundred and sixty-two consecutive patients undergoing EMB at our Institution from 2010 to 2019 were included. Pathological areas identified at EVM and CMR underwent EMB. According to EMB results, CMR and EVM sensitivity and specificity regarding the identification of pathological substrates of myocardium were evaluated. Results A gadolinium-enhanced CMR had been performed in 143 (88.9%) of the population and yielded pathological findings in 121 (85.8%) of such cases. Late gadolinium enhancement (LGE) was present in 94 (70%) of the patients, while EVM identified areas of low voltages in 61%. Right (73%), left (19%) or both ventricles (8%) underwent sampling. EVM proved to have similar sensitivity to CMR (74% vs. 77%; P=0.479), with non-significantly higher specificity (70% vs. 47% P=0.738). In 12 patients with EMB-proven cardiomyopathy, EVM identified pathological areas, which had been undetected at CMR evaluation (concordance rate 53.8%; k = 0.26). Sensitivity of pooled EVM and CMR was as high as 95%. Five cases (3,8%) of cardiomyopathies were undetected by both CMR and EVM. Complications rate was low (4,9%), mostly vascular access related, with no patients requiring urgent management. Conclusion EVM proved to be a promising tool for targeted-EMB due to its sensitivity and specificity in identifying myocardial pathological substrates. EVM demonstrated to have an accuracy similar to CMR. EVM and CMR together conferred EMB a positive predictive value of 89%. Funding Acknowledgement Type of funding source: None

Introduction Myocarditis is a complex inflammatory disease, usually secondary to viral infections or immune system dysregulation, with extremely heterogeneous clinical manifestations. Among them, potentially life-threatening ventricular arrhythmias (VA) may present at any stage of the disease as an expression of myocardial electrical instability. Purpose Our aim was to evaluate the efficacy of radiofrequency catheter ablation (RFCA) of VA in our large cohort of myocarditis, trying to understand the predictors of RFCA success. Methods and results 144 patients (61 men; age 43 [29–54] years) with history of myocarditis with arrhythmic presentation (118 biopsy-proven, 82%) composed our population. At presentation, 26% of patients suffered of ventricular tachycardia (VT) while in 17% cardiac arrest occurred: overall 49 patients (35%) were implanted with an ICD. The median left ventricular ejection fraction (LVEF) was 58% (48–61%). An intensive non-invasive and invasive work-up was performed: 104 patients underwent cardiac magnetic resonance (CMR) that showed late gadolinium enhancement (LGE) in 67 of them (63%). In 37 patients LGE was found in the anteroseptal portion of the left ventricle: this pattern showed association with major arrhythmic relapse (VT and ventricular fibrillation) during follow up (Fig. 1; OR 4.0, CI 95% 1.14–14.1, p=0.03). 95 patients underwent endocardial RFCA, using contact electroanatomic mapping. Interestingly, in patients with anteroseptal LGE RCFA didn't affect significantly the arrhythmic relapse (OR 5, CI 95% 0.9–33, p=0.06). Otherwise RFCA prevent arrhythmic relapse in patients that showed LGE in ventricular portion other than the anteroseptal one (OR 0.027, IC 95% 0.002–0.40, p Conclusions In myocarditis patients with VA, LGE pattern predicts arrhythmic relapse during follow-up. RFCA success rate is strictly linked to scar location, being significantly higher in patients with non-anteroseptal LGE. Figure 1 Funding Acknowledgement Type of funding source: None

The clinical presentation of cardiac sarcoidosis (CS) may resemble that of arrhythmogenic right ventricular cardiomyopathy (ARVC).The purpose of this study was to identify clinical variables to better discriminate between patients with genetically determined ARVC and those with CS fulfilling definite 2010 ARVC Task Force Criteria (TFC).In this multicenter study, 10 patients with CS fulfilling definite 2010 ARVC TFC were age and gender matched with 10 genetically proven ARVC patients. A cardiacThe 2010 ARVC TFC did not reliably differentiate between the 2 diseases. CS patients presented with longer PR intervals, advanced atrioventricular block (AVB), and longer QRS duration (P .001 and P = .009, respectively), whereas T-wave inversions (TWIs) in the peripheral leads were more common in ARVC patients (P = .009). CS patients presented with more extensive left ventricular involvement and lower left ventricular ejection fraction (LVEF), whereas ARVC patients had a larger right ventricular outflow tract (RVOT) (P = .044). PET scan positivity was only present in CS patients (90% vs 0%).The 2010 ARVC TFC do not reliably differentiate between CS patients fulfilling 2010 ARVC TFC and those with hereditary ARVC. Prolonged PR interval, advanced AVB, longer QRS duration, right ventricular apical involvement, reduced LVEF, and positive

Background A cardiologist-only approach to procedural sedation with midazolam in the setting of elective cardioversion (DCC) for AF has already been proven as safe as sedation with propofol and anaesthesiologist assistance. No data exist regarding the safety of such a strategy during emergency procedures. The aim of this study is to compare the feasibility of sedation with midazolam, administered by a cardiologist, to an anaesthesiologist-assisted protocol with propofol in emergency DCC. Methods Single centre, prospective, open blinded, randomized study including all consecutive patients admitted to the Emergency Department requiring urgent or emergency DCC. Patients were randomized in a 1:1 fashion to either propofol or midazolam treatment arm. Patients in the midazolam group were managed by the cardiologist only, while patients treated with propofol group underwent DCC with anaesthesiologist assistance. Results Sixty-nine patients were enrolled and split into two groups. Eighteen patients (26.1%) experienced peri-procedural adverse events (bradycardia, severe hypotension and severe hypoxia), which were similar between the two groups and all successfully managed by the cardiologist. No deaths, stroke or need for invasive ventilation were registered. Patients treated with propofol experienced a greater decrease in systolic and diastolic blood pressure when compared with those treated with midazolam. As the procedure was shorter when midazolam was used, the median cost of urgent/emergency DCC with midazolam was estimated to be 129.0 € (1st-3rd quartiles 114.6–151.6) and 195.6 € (1st-3rd quartiles 147.3–726.7) with propofol (p Conclusions Procedural sedation with midazolam given by the cardiologist alone was feasible, well-tolerated and cost-effective in emergency DCC.

Plakophilin-2 (PKP2) is the most frequently mutated desmosomal gene in arrhythmogenic cardiomyopathy (ACM), a disease characterized by structural and electrical alterations predominantly affecting the right ventricular myocardium. Notably, ACM cases without overt structural alterations are frequently reported, mainly in the early phases of the disease. Recently, the PKP2 p.S183N mutation was found in a patient affected by Brugada syndrome (BS), an inherited arrhythmic channelopathy most commonly caused by sodium channel gene mutations. We here describe a case of a patient carrier of the same BS-related PKP2 p.S183N mutation but with a clear diagnosis of ACM. Specifically, we report how clinical and molecular investigations can be integrated for diagnostic purposes, distinguishing between ACM and BS, which are increasingly recognized as syndromes with clinical and genetic overlaps. This observation is fundamentally relevant in redefining the role of genetics in the approach to the arrhythmic patient, progressing beyond the concept of “one mutation, one disease”, and raising concerns about the most appropriate approach to patients affected by structural/electrical cardiomyopathy. The merging of genetics, electroanatomical mapping, and tissue and cell characterization summarized in our patient seems to be the most complete diagnostic algorithm, favoring a reliable diagnosis.