Electrocardiographic predictors of left ventricular scar in athletes with right bundle branch block premature ventricular beats.

AIMS: Right bundle branch block (RBBB) morphology non-sustained ventricular arrhythmias (VAs) have been associated with the presence of non-ischaemic left ventricular scar (NLVS) in athletes. The aim of this cross-sectional study was to identify clinical and electrocardiogram (ECG) predictors of the presence of NLVS in athletes with RBBB VAs. METHODS AND RESULTS: Sixty-four athletes [median age 39 (24-53) years, 79% males] with non-sustained RBBB VAs underwent cardiac magnetic resonance (CMR) with late gadolinium enhancement in order to exclude the presence of a concealed structural heart disease. Thirty-six athletes (56%) showed NLVS at CMR and were assigned to the NLVS positive group, whereas 28 athletes (44%) to the NLVS negative group. Family history of cardiomyopathy and seven different ECG variables were statistically more prevalent in the NLVS positive group. At univariate analysis, seven ECG variables (low QRS voltages in limb leads, negative T waves in inferior leads, negative T waves in limb leads I-aVL, negative T waves in precordial leads V4-V6, presence of left posterior fascicular block, presence of pathologic Q waves, and poor R-wave progression in right precordial leads) proved to be statistically associated with the finding of NLVS; these were grouped together in a score. A score ≥2 was proved to be the optimal cut-off point, identifying NLVS athletes in 92% of cases and showing the best accuracy (86% sensitivity and 100% specificity, respectively). However, a cut-off ≥1 correctly identified all patients with NLVS (absence of false negatives). CONCLUSION: In athletes with RBBB morphology non-sustained VAs, specific ECG abnormalities at 12-lead ECG can help in detecting subjects with NLVS at CMR.

In athletes with right bundle branch block (RBBB) morphology non-sustained ventricular arrhythmias (VAs), the presence of a non-ischaemic left ventricular scar (NLVS) may be highly suspected if one or more of the following electrocardiogram (ECG) characteristics are present at the 12-lead resting ECG: low QRS voltages in limb leads, negative T waves in inferior leads, negative T waves in limb leads I­aVL, negative T waves in precordial leads V4­V6, presence of left posterior fascicular block, presence of pathologic Q waves, and poor R-wave progression in right precordial leads. This score should be externally validated in a larger population of athletes with VAs. In athletes with RBBB morphology non-sustained Vas, attention should be placed on the 12-lead resting ECG to suspect the presence of an NLVS. In athletes with RBBB VAs and the presence of one or more of the identified ECG characteristics, a cardiac magnetic resonance with late gadolinium enhancement is useful to rule out an NLVS.

Interpretation and management of premature ventricular beats in athletes: An expert opinion document of the Italian Society of Sports Cardiology (SICSPORT).

Premature ventricular beats (PVBs) are recorded in a sizeable proportion of athletes during pre-participation screening, especially if the evaluation includes both resting and exercise ECG. While in the majority of cases no underlying heart disease is present, in others PVBs may be the sign of a condition at risk of sudden cardiac death, including cardiomyopathies, congenital, coronary artery, heart valves and ion channels diseases. In this expert opinion document of the Italian Society of Sports Cardiology, we propose a multiparametric interpretation approach to PVBs in athletes and a stepwise diagnostic algorithm. The clinical work-up should include the assessment of the probable site of origin based on the ECG pattern of the ectopic QRS and of the arrhythmia behavior (including the number of different PVB morphologies, complexity, response to exercise and reproducibility), as well as first-line tests such as echocardiography. Based on this initial evaluation, most athletes can be reassured of the benign nature of PVBs and cleared for competition under periodical follow-up. However, when the clinical suspicion is high, further investigations with non-invasive (e.g. cardiac magnetic resonance, cardiac computed tomography, genetic testing) and, in very selected cases, invasive (e.g. endocardial voltage mapping and endomyocardial biopsy) tests should be carried out to rule out a high-risk condition. Importantly, such advanced tests should be performed in centers with a consolidated experience not only in the technique, but also in evaluation of athletes.

Electroanatomic mapping in athletes: Why and when. An expert opinion paper from the Italian Society of Sports Cardiology.

Three-dimensional electroanatomical mapping (EAM) has the potential to identify the pathological substrate underlying ventricular arrhythmias (VAs) in different clinical settings by detecting myocardial areas with abnormally low voltages, which reflect the presence of different cardiomyopathic substrates. In athletes, the added value of EAM may be to enhance the efficacy of third-level diagnostic tests and cardiac magnetic resonance (CMR) in detecting concealed arrhythmogenic cardiomyopathies. Additional benefits of EAM in the athlete include the potential impact on disease risk stratification and the consequent implications for eligibility to competitive sports. This opinion paper of the Italian Society of Sports Cardiology aims to guide general sports medicine physicians and cardiologists on the clinical decision when to eventually perform an EAM study in the athlete, highlighting strengths and weaknesses for each cardiovascular disease at risk of sudden cardiac death during sport. The importance of early (preclinical) diagnosis to prevent the negative effects of exercise on phenotypic expression, disease progression, and worsening of the arrhythmogenic substrate is also addressed.

Mitral Valve Prolapse and Sudden Cardiac Death in Athletes at High Risk.

Mitral valve prolapse (MVP) is the most frequent valvulopathy in the general population, with usually a favourable prognosis. Although it can be associated with some complications, ventricular arrhythmias (VA) and sudden cardiac death (SCD) are the most worrying. The estimated risk of SCD in MVP is between 0.2% to 1.9% per year, including MVP patients with and without severe mitral regurgitation (MR). The association between SCD and MVP is expressed by a phenotype called "malignant MVP" characterized by transthoracic echocardiography (TTE) findings such as bileaflet myxomatous prolapse and mitral annulus disjunction (MAD), ECG findings such as repolarization abnormalities, complex ventricular arrhythmias (c-VAs) and LV fibrosis of papillary muscles (PMs) and inferobasal wall visualized by late gadolinium enhancement cardiac magnetic resonance (LGE-CMR). Therefore, attention is raised for patients with "arrhythmic MVP" characterized from an ECG point of view by frequent premature ventricular contractions (PVCs) arising from one or both PMs as well as by T-wave inversion in the inferolateral leads. In athletes, SCD is the most frequent medical cause of death and in young subjects (< 35 years) usually is due to electrical mechanism affecting who has a silent cardiovascular disease and are not considered per se a cause of increased mortality. In MVP, SCD was reported to happen during sports activity or immediately after and valve prolapse was the only pathological aspect detected. The aim of the present paper is to explore the association between SCD and MVP in athletes, focusing attention on ECG, TTE in particular, and CMR findings that could help to identify subjects at high risk for complex arrhythmias and eventually SCD. In addition, it is also examined if sports activity might predispose patients with MVP to develop major arrhythmias.

Sport Related Sudden Death: The Importance of Primary and Secondary Prevention.

Sports are a double-edged sword. On the one hand, cardiovascular benefits from sports activity are well-known, and on the other hand, sports may increase the risk of sudden cardiac death (SCD) in subjects with known or unknown cardiovascular diseases. SCD is rare but has a very strong emotional issue. There are many examples involving famous professional athletes, but this is only scratching the surface of a widespread phenomenon that also involves amateur athletes. The importance of safely performing physical activity appears clear in both professional and amateur athletes. In particular, the former undergo a pre-participation screening for SCD primary prevention with different recommendations in each country. On the other hand, a medical examination is not mandatory for non-professional athletes and, therefore, for people who practice sports as an amateur. Widespread distribution of automatic external defibrillators and people trained for cardiopulmonary resuscitation are necessary to promote secondary prevention of SCD. We briefly report a case series of athletes with aborted SCD during sports activity in order to underline and discuss in this review the previously highlighted issues.

Molecular genetic testing in athletes: Why and when a position statement from the Italian Society of Sports Cardiology.

Molecular genetic testing is an increasingly available test to support the clinical diagnosis of inherited cardiovascular diseases through identification of pathogenic gene variants and to make a preclinical genetic diagnosis among proband's family members (so-called "cascade family screening"). In athletes, the added value of molecular genetic testing is to assist in discriminating between physiological adaptive changes of the athlete's heart and inherited cardiovascular diseases, in the presence of overlapping phenotypic features such as ECG changes, imaging abnormalities or arrhythmias ("grey zone"). Additional benefits of molecular genetic testing in the athlete include the potential impact on the disease risk stratification and the implications for eligibility to competitive sports. This position statement of the Italian Society of Sports Cardiology aims to guide general sports medical physicians and sports cardiologists on clinical decision as why and when to perform a molecular genetic testing in the athlete, highlighting strengths and weaknesses for each inherited cardiovascular disease at-risk of sudden cardiac death during sport. The importance of early (preclinical) diagnosis to prevent the negative effects of exercise on phenotypic expression, disease progression and worsening of the arrhythmogenic substrate is also addressed.

Tailoring the Ablative Strategy for Atrial Fibrillation: A State-of-the-Art Review.

In spite of technological progress and the improving skills of operators, atrial fibrillation (AF) ablation results appear to date to be at a plateau. In any case, the superiority of ablation over pharmacological therapy in terms of effectiveness, reduction of hospitalizations, and improvement has been well demonstrated in recent randomized trials. Triggers, substrate, and modulating factors (elements of Coumel's triangle) play different roles in paroxysmal and persistent AF, so induction and perpetuation mechanisms of arrhythmia may be different in each patient. Although effective ablative strategies are available for the treatment of paroxysmal AF triggers and persistent AF substrates, an adequate clinical evaluation of the patient is crucial in order to increase the chances of success. Recognizing triggers allows not only performing an effective ablation but also to avoid unnecessary lesions and at the same time reducing the risk of complications. AF beginning and triggers could be recorded by 12-lead ECG, continuous Holter monitoring, or implantable devices. In case of an unsuccessful noninvasive evaluation, nonpulmonary vein triggers should be investigated with an electrophysiological study. Persistent AF needs more effort to perform an accurate substrate characterization. Among the many methods proposed, recently the use of high-density mapping and multipolar catheters seems of particular benefit in order to clarify the arrhythmia mechanisms. Surgical and hybrid techniques allow to treat regions such as the posterior wall or Bachmann's bundle, which is fundamental for an ablative strategy that goes beyond just pulmonary vein isolation. Too often, patients are referred to electrophysiology laboratories without adequate preprocedural screening and planning in order to submit them to a standard "ready-made" procedure. The accurate search for triggers in paroxysmal AF and the correct recognition of the link between a possible underlying heart disease and the substrate in persistent AF could allow us to tailor the interventional approach in order to overcome the current plateau, increasing ablative procedure success and minimizing complications.

[Brugada syndrome and early repolarization syndrome: similarities and differences. The lesson from case reports]. / Sindrome di Brugada e sindrome della ripolarizzazione precoce: affinità e differenze. La lezione dei casi clinici.

In subjects with cardiac arrest and aborted sudden death, the presence of an ECG pattern (spontaneous or drug-induced) suggesting a Brugada syndrome (BrS) is generally considered the proof of a BrS. We present three case reports of patients with resuscitated sudden death in whom a BrS was diagnosed, but in whom the real cause was probably an early repolarization syndrome (ERS). This distinction is not only theoretical but has also practical consequences. In fact, the arrhythmic substrate is different in the two conditions: right ventricular outflow tract in BrS, inferior ventricular wall in ERS. As a consequence, catheter ablation of the right ventricular outflow tract may be useful in BrS, whereas it is useless in ERS.

Genotype-Phenotype Correlation of SCN5A Genotype in Patients With Brugada Syndrome and Arrhythmic Events: Insights From the SABRUS in 392 Probands.

BACKGROUND: Brugada syndrome (BrS) is associated with mutations in the cardiac sodium channel gene, SCN5A. However, genetic studies of patients with BrS with arrhythmic events have been limited. We sought to compare various clinical, ECG, and electrophysiological parameters according to SCN5A genotype in a large cohort of BrS probands with first arrhythmic event. METHODS: Survey on Arrhythmic Events in Brugada Syndrome is a survey of 10 Western and 4 Asian countries, gathering 678 patients with BrS with first arrhythmic event. Only probands were included, and SCN5A genotype adjudicated. Patients without appropriate genetic data were excluded. Associations of genotype with clinical features were analyzed. RESULTS: The study group comprised 392 probands: 92 (23.5%) SCN5A+(44 pathogenic/likely pathogenic [P/LP] and 48 variants of unknown significance) and 300 (76.5%) SCN5A-.SCN5A missense variants and the patients hosting them were similar regardless of adjudication. A higher proportion of patients with P/LP were pediatric (<16 years) compared with SCN5A- (11.4% versus 3%, P=0.023). The proportion of females was higher among patients with P/LP compared with SCN5A- (18.2% versus 6.3%, P=0.013). P/LP probands were more likely to have a family history of sudden cardiac death compared with SCN5A- (41.9% versus 16.8%, P<0.001). A higher proportion of patients with P/LP were White compared with SCN5A- (87.5% versus 47%, P<0.001). Ethnicity (odds ratio, 5.41 [2.8-11.19], P<0.001) and family history of sudden cardiac death (odds ratio, 2.73 [1.28-5.82], P=0.009) were independent variables associated with P/LP genotype following logistic regression. CONCLUSIONS: The genetic basis of BrS has a complex relationship with gender, ethnicity, and age. Probands hosting a P/LP variant tended to experience their first arrhythmic event at a younger age and to have events triggered by fever compared with patients with SCN5A-. In addition, they were more likely to be White and to have family history of sudden cardiac death. Among females, a P/LP variant suggests an increased risk of being symptomatic. This association should be further studied on an ethnically specific basis in large prospectively collected international cohorts.

Standard ECG in Brugada Syndrome as a Marker of Prognosis: From Risk Stratification to Pathophysiological Insights.

Background The 12-lead ECG plays a key role in the diagnosis of Brugada syndrome (BrS). Since the spontaneous type 1 ECG pattern was first described, several other ECG signs have been linked to arrhythmic risk, but results are conflicting. Methods and Results We performed a systematic review to clarify the associations of these specific ECG signs with the risk of syncope, sudden death, or equivalents in patients with BrS. The literature search identified 29 eligible articles comprising overall 5731 patients. The ECG findings associated with an incremental risk of syncope, sudden death, or equivalents (hazard ratio ranging from 1.1-39) were the following: localization of type 1 Brugada pattern (in V2 and peripheral leads), first-degree atrioventricular block, atrial fibrillation, fragmented QRS, QRS duration >120 ms, R wave in lead aVR, S wave in L1 (≥40 ms, amplitude ≥0.1 mV, area ≥1 mm2), early repolarization pattern in inferolateral leads, ST-segment depression, T-wave alternans, dispersion of repolarization, and Tzou criteria. Conclusions At least 12 features of standard ECG are associated with a higher risk of sudden death in BrS. A multiparametric risk assessment approach based on ECG parameters associated with clinical and genetic findings could help improve current risk stratification scores of patients with BrS and warrants further investigation. Registration URL: https://www.crd.york.ac.uk/prospero/. Unique identifier: CRD42019123794.

Italian Cardiological Guidelines (COCIS) for Competitive Sport Eligibility in athletes with heart disease: update 2020.

Since 1989, SIC Sport and a FMSI, in partnership with leading Italian Cardiological Scientific Associations (ANCE, ANMCO and SIC) have produced Cardiological Guidelines for Completive Sports Eligibility for athletes with heart disease (COCIS -- 1989, 1995, 2003, 2009 and 2017). The English version of the Italian Cardiological Guidelines for Competitive Sports Eligibility for athletes with heart disease was published in 2013 in this Journal. This publication is an update with respect to the document previously published in English in 2013. It includes the principal innovations that have emerged over recent years, and is divided into five main chapters: arrhythmias, ion channel disorders, congenital heart diseases, acquired valve diseases, cardiomyopathies, myocarditis and pericarditis and ischemic heart disease. Wherever no new data have been introduced with respect to the 2013 publication, please refer to the previous version. This document is intended to complement recent European and American guidelines but an important difference should be noted. The European and American guidelines indicate good practice for people engaging in physical activity at various levels, not only at the competitive level. In contrast, the COCIS guidelines refer specifically to competitive athletes in various sports including those with high cardiovascular stress. This explains why Italian guidelines are more restrictive than European and USA ones. COCIS guidelines address 'sports doctors' who, in Italy, must certify fitness to participate in competitive sports. In Italy, this certificate is essential for participating in any competition.

Clinical and Prognostic Significance of Idiopathic Left Bundle-Branch Block in Young Adults.

AIMS: LBBB is rare in healthy young adults, and its long-term prognosis is uncertain. METHODS: 56 subjects (aged <50 years), in whom an LBBB was discovered by chance in the absence of clinical and echocardiographic evidence of heart disease, were collected in a multicenter registry. RESULTS: 69% were males. Mean age at the time of discovery of LBBB was 37 ± 11 years. Mean QRS duration was 149 ± 17 m sec and 35% had left axis deviation. All patients had a normal echocardiogram, except for left ventricular dyssynchrony; 37 patients underwent coronary angiography (30) or myocardial scintigraphy during effort Eriksson and Wilhelmsen (2005), and in all cases obstructive coronary artery disease was excluded. In 2/30 patients who underwent coronary angiography, an anomalous origin of the CX artery from the right coronary sinus was found. Thirty patients underwent cardiac magnetic resonance; in 60% it was normal, while in 40% it revealed late enhancement, which in 33% was localized in the basal septum, suggesting fibrosis of the left bundle branch. During follow-up (12+/10 years, median 10 years) no sudden death occurred. At the end of follow-up, all patients were alive, except for one who suffered accidental death. Two patients (3.5%) underwent PM implantation owing to syncope. The echocardiogram at the end of follow-up revealed LV dysfunction in only one patient. CONCLUSIONS: In young adults without apparent heart disease, LBBB is a heterogeneous condition. In the vast majority of cases, the prognosis is good and no ventricular dysfunction occurs over time. However, as only 18% of our patients were aged >60 years at the end of follow-up, we cannot establish the prognosis in older age-groups.

Sport activity in patients with cardiac implantable electronic devices: evidence and perspectives.

Demographic characteristics of patients with cardiac implantable electronic devices have significantly changed during the last few years, according to the ageing of the population and the consequent increase in the number of elderly individuals with indication for pacemaker implant and, on the other hand, to the increased number of young individuals implanted with an implantable cardioverter defibrillator for the primary prevention of sudden death. More and more often, both elderly and young patients ask the physician to deal with the device in their daily activities, which often include sport practice. This latter is advisable because of its recognized benefits on cardiovascular prevention, although there are many limitations for patients with a cardiac implantable electronic device. Hence, the need to balance the patient's request with the appropriate precautions emerging from existing evidence. The current article aims to provide an overview of the most recent data on this topic, derived from registries and observational studies. Over the years an attempt to standardize recommendations has been made, but robust evidence is still lacking. Substantial differences exist between countries based on their sports regulations. Official recommendations of European and American Scientific Societies are resumed. The future perspective is to obtain data to allow these patients a safer practice of sport activity also through technological advances in terms of device materials and programming improvement and the possibility of remote monitoring.

Recommendations for participation in leisure-time physical activity and competitive sports in patients with arrhythmias and potentially arrhythmogenic conditions: Part 1: Supraventricular arrhythmias. A position statement of the Section of Sports Cardiology and Exercise from the European Association of Preventive Cardiology (EAPC) and the European Heart Rhythm Association (EHRA), both associations of the European Society of Cardiology.

Symptoms attributable to arrhythmias are frequently encountered in clinical practice. Cardiologists and sport physicians are required to identify high-risk individuals harbouring such conditions and provide appropriate advice regarding participation in regular exercise programmes and competitive sport. The three aspects that need to be considered are: (a) the risk of life-threatening arrhythmias by participating in sports; (b) control of symptoms due to arrhythmias that are not life-threatening but may hamper performance and/or reduce the quality of life; and (c) the impact of sports on the natural progression of the underlying arrhythmogenic condition. In many cases, there is no unequivocal answer to each aspect and therefore an open discussion with the athlete is necessary, in order to reach a balanced decision. In 2006 the Sports Cardiology and Exercise Section of the European Association of Preventive Cardiology published recommendations for participation in leisure-time physical activity and competitive sport in individuals with arrhythmias and potentially arrhythmogenic conditions. More than a decade on, these recommendations are partly obsolete given the evolving knowledge of the diagnosis, management and treatment of these conditions. The present document presents a combined effort by the Sports Cardiology and Exercise Section of the European Association of Preventive Cardiology and the European Heart Rhythm Association to offer a comprehensive overview of the most updated recommendations for practising cardiologists and sport physicians managing athletes with supraventricular arrhythmias, and provides pragmatic advice for safe participation in recreational physical activities, as well as competitive sport at amateur and professional level. A companion text on recommendations in athletes with ventricular arrhythmias, inherited arrhythmogenic conditions, pacemakers and implantable defibrillators is published as Part 2 in Europace.

Sudden Cardiac Death in Patients with Ventricular Preexcitation.

Patients with the Wolff-Parkinson-White syndrome may experience benign and malignant arrhythmias, the most common being atrioventricular reentrant tachycardias. This arrhythmia may degenerate into atrial fibrillation, which can be conducted over an accessory pathway capable of exceptionally fast conduction to the ventricles and degenerate into ventricular fibrillation, leading to sudden cardiac death. These life-threatening events generally affect symptomatic patients in their third or fourth decade. Although rare, ventricular fibrillation may be the first clinical manifestation in subjects who are asymptomatic or unaware of their conditions. Electrophysiologic study may be useful to identify subjects at high risk of sudden cardiac death.

Ethnic differences in patients with Brugada syndrome and arrhythmic events: New insights from Survey on Arrhythmic Events in Brugada Syndrome.

BACKGROUND: There is limited information on ethnic differences between patients with Brugada syndrome (BrS) and arrhythmic events (AEs). OBJECTIVE: The purpose of this study was to compare clinical, electrocardiographic (ECG), electrophysiological, and genetic characteristics between white and Asian patients with BrS and AEs. METHODS: The Survey on Arrhythmic Events in Brugada Syndrome is a multicenter survey from Western and Asian countries, gathering 678 patients with BrS and first documented AE. After excluding patients with other (n = 14 [2.1%]) or unknown (n = 30 [4.4%]) ethnicity, 364 (53.7%) whites and 270 (39.8%) Asians comprised the study group. RESULTS: There was no difference in AE age onset (41.3 ± 16.1 years in whites vs 43.3 ± 12.3 years in Asians; P = .285). Higher proportions of whites were observed in pediatric and elderly populations. Asians were predominantly men (98.1% vs 85.7% in whites; P < .001) and frequently presented with aborted cardiac arrest (71.1% vs 56%; P < .001). Asians tended to display more spontaneous type 1 BrS-ECG pattern (71.5% vs 64.3%; P = .068). A family history of sudden cardiac death was noted more in whites (29.1% vs 11.5%; P < .001), with a higher rate of SCN5A mutation carriers (40.1% vs 13.2% in Asians; P < .001), as well as more fever-related AEs (8.5% vs 2.9%; P = .011). No difference was observed between the 2 groups regarding history of syncope and ventricular arrhythmia inducibility. CONCLUSION: There are important differences between Asian and white patients with BrS. Asian patients present almost exclusively as male adults, more often with aborted cardiac arrest and spontaneous type 1 BrS-ECG. However, they have less family history of sudden cardiac death and markedly lower SCN5A mutation rates. The striking difference in SCN5A mutation rates should be tested in future studies.