• Athletes
• ECG
• Non-ischaemic left ventricular scar
• Predictors
• Ventricular arrhythmias

• Male
• Humans
• Adult
• Female
• Bundle-Branch Block/diagnosis
• Bundle-Branch Block/etiology
• Ventricular Premature Complexes/diagnosis
• Ventricular Premature Complexes/etiology
• Cicatrix/pathology
• Contrast Media
• Cross-Sectional Studies
• Gadolinium
• Electrocardiography

• Cardiac magnetic resonance
• Late gadolinium enhancement
• Premature ventricular complex
• Right bundle branch block morphology

• Male
• Humans
• Female
• Cicatrix
• Contrast Media
• Gadolinium
• Heart Ventricles/diagnostic imaging
• Electrocardiography/methods
• Ventricular Premature Complexes
• Bundle-Branch Block
• Athletes

• LVEF
• Morphology
• PVC ablation
• PVC burden
• Premature ventricular complex (PVC)

• Humans
• Catheter Ablation/adverse effects
• Catheter Ablation/methods
• Stroke Volume/physiology
• Ventricular Dysfunction, Left
• Ventricular Function, Left/physiology
• Ventricular Premature Complexes/diagnosis
• Ventricular Premature Complexes/surgery
• Ventricular Premature Complexes/complications

• Anti-arrhythmic drugs
• Cardiac magnetic resonance
• Cardiomyopathies
• Catheter ablation
• Chronic coronary artery disease
• Genetic testing
• Guidelines
• Implantable cardioverter defibrillator
• Premature ventricular complex
• Primary electrical disease
• Recommendations
• Risk calculator
• Risk stratification
• Sudden cardiac death
• Sudden death
• Ventricular arrhythmia
• Ventricular fibrillation
• Ventricular tachycardia

• Humans
• Death, Sudden, Cardiac/prevention & control
• Arrhythmias, Cardiac/complications
• Arrhythmias, Cardiac/therapy

• Cardiac magnetic resonance
• Electrocardiogram
• Late gadolinium enhancement
• Myocardial fibrosis

• MRI
• cohort study
• outcomes
• premature ventricular complexes
• ventricular tachycardia

• Ambulatory
• Arrhythmias
• Cardiac
• Electrocardiography
• Fibrosis
• Magnetic resonance imaging

• 81701651 National Natural Science Foundation of China

Premature ventricular contractions in the absence of structural heart disease are among the most common arrhythmias in clinical practice, with well-defined sites of origin in the right and left ventricle. In this review, starting from the electrocardiographic localization of premature ventricular contractions, we investigated the mechanisms, prevalence in the general population, diagnostic work-up, prognosis and treatment of premature ventricular contractions, according to current scientific evidence.

• antiarrhythmic drugs
• premature ventricular contractions
• transcatheter ablation

• Electrocardiography, Ambulatory
• Heart Ventricles
• Humans
• Melissa
• Tea
• Ventricular Premature Complexes/drug therapy

• cardiac magnetic resonance
• cardiovascular imaging
• premature ventricular contractions
• ventricular arrhythmia

• ALF Marten Rosnqvist Stockholms Läns Landsting

• ablation techniques
• ventricular premature complexes

• cardiac magnetic resonance
• feature-tracking CMR
• myocardial strain
• premature ventricular contractions
• ventricular arrhythmia

• CH/F/21/90009 British Heart Foundation
• RG/F/21/110040 British Heart Foundation
• TG/16/3/32687 British Heart Foundation

• Arrhythmias
• Cardiac magnetic resonance imaging
• Catheter ablation
• Late gadolinium enhancement
• Sudden cardiac death

• Contrast Media
• Gadolinium
• Humans
• Magnetic Resonance Imaging
• Ventricular Function, Left
• Ventricular Premature Complexes/diagnostic imaging

• arrhythmias
• cardiac gadolinium
• magnetic resonance angiography
• prognosis

• Catheter ablation
• Clinical document
• Electrical storm
• Electroanatomical mapping
• Electrocardiogram
• Expert consensus statement
• Imaging
• Premature ventricular complex
• Radiofrequency ablation
• Ventricular arrhythmia
• Ventricular tachycardia

Patients undergoing ablation of premature ventricular complexes (PVCs) can have cardiac scar. Risk factors for the presence of scar are not well defined.

To determine the prevalence of scarring detected by delayed enhancement cardiac magnetic resonance imaging (DE-CMR) in patients undergoing ablation of PVCs, to create a risk score predictive of scar, and to explore correlations between the scoring system and long-term outcomes.

DE-CMR imaging was performed in consecutive patients with frequent PVCs referred for ablation. The full sample was used to develop a prediction model for cardiac scar based on demographic and clinical characteristics, and internal validation of the prediction model was done using bootstrap samples.

The study consisted of 333 patients (52% male, aged 53.2 ± 14.5 years, preablation ejection fraction 50.9% ± 12.2%, PVC burden 20.7 ± 13.14), of whom 112 (34%) had DE-CMR scarring. Multiple logistic regression analysis showed age (odds ratio [OR] 1.02 [1.01–1.04]/year, P = .019) and preablation ejection fraction (OR 0.92 [0.89–0.94]/%, P < .001) to be predictive of scar. A weighted risk score incorporating age and ejection fraction was used to stratify patients into low-, medium-, and high-risk groups. Scar prevalence was around 86% in the high-risk group and 12% in the low-risk group; high-risk patients had worse survival free of arrhythmia.

Cardiac scar was present in one-third of patients referred for PVC ablation. A weighted risk score based simply on patient age and preprocedural ejection fraction can help discriminate between patients at high and low risk for the presence of cardiac scar and worse arrhythmia outcomes.

• Cardiac scar
• Catheter ablation
• Delayed enhancement cardiac magnetic resonance imaging
• Premature ventricular complexes
• Risk stratification

• Catheter ablation
• Clinical document
• Electrical storm
• Electroanatomical mapping
• Electrocardiogram
• Expert consensus statement
• Imaging
• Premature ventricular complex
• Radiofrequency ablation
• Ventricular arrhythmia
• Ventricular tachycardia

• Cardiac Electrophysiology
• Catheter Ablation
• Consensus
• Humans
• Tachycardia, Ventricular/surgery
• Ventricular Premature Complexes

• R21 DC020005 NIDCD NIH HHS

• Cardiovascular magnetic resonance imaging
• Diagnostic reclassification
• Implanted cardioverter defibrillator
• Treatment decision
• Ventricular tachycardia ablation

• Aged
• Catheter Ablation
• Clinical Decision-Making
• Death, Sudden, Cardiac/prevention & control
• Defibrillators, Implantable
• Disease Progression
• Electric Countershock/instrumentation
• Female
• Heart Failure/diagnostic imaging
• Heart Failure/physiopathology
• Heart Failure/therapy
• Humans
• Magnetic Resonance Imaging, Cine
• Male
• Middle Aged
• Predictive Value of Tests
• Primary Prevention
• Secondary Prevention
• Tachycardia, Ventricular/diagnostic imaging
• Tachycardia, Ventricular/physiopathology
• Tachycardia, Ventricular/therapy
• Time Factors
• Treatment Outcome
• Ventricular Function, Left

• Children
• Premature ventricular contractions
• Screening
• Ventricular tachicardia

The athletic preparticipation evaluation (PPE) protocol proposed by the European Society of Cardiology includes history, physical examination and resting electrocardiogram (ECG). The aim of this study was to assess the results of adding constant-load ECG stress testing (EST) to the protocol for the evaluation of ventricular arrhythmias (VA) inducibility.

We evaluated a consecutive cohort of young athletes with history, physical examination, resting ECG and EST. Athletes with VA induced by EST underwent 24-hour 12-lead Holter monitoring and echocardiography. Cardiac magnetic resonance (CMR) was reserved for those with frequent, repetitive or exercise-worsened VA, and for athletes with echocardiographic abnormalities.

Of 10,985 athletes (median age 15 years, 66% males), 451 (4.1%) had an abnormal history, physical examination or resting ECG and 31 (0.28%) were diagnosed with a cardiac disease and were at risk of sudden cardiac death. Among the remaining 10,534 athletes, VA at EST occurred in 524 (5.0%) and a previously missed at-risk condition was identified in 23 (0.22%); the most common (N = 10) was an echocardiographically silent non-ischaemic left-ventricular fibrosis evidenced by CMR. The addition of EST increased the diagnostic yield of PPE by 75% (from 0.28% to 0.49%) and decreased the positive predictive value by 20% (from 6.9% to 5.5%). During a 32 ± 21 months follow-up, no cardiac arrests occurred among either eligible athletes or non-eligible athletes with cardiovascular disease.

The addition of exercise testing for the evaluation of VA inducibility to history, physical examination and ECG resulted in an increase of the diagnostic yield of PPE at the expense of an increase in false-positive findings.

• Cardiac magnetic resonance
• late enhancement
• premature ventricular beats
• sports cardiology
• sudden cardiac death

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.

Routine diagnostic work‐up not infrequently miss subtle concealed arrhythmic substrate, leading to erroneously refer to such arrhythmias as to “idiopathic”.

Literature search of PVCs articles was conducted in PubMed and Scopus electronic database.

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.

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.

• cardiac magnetic resonance
• premature ventricular complexes
• risk stratification

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.

• Catheter ablation
• Clinical document
• Electrical storm
• Electroanatomical mapping
• Electrocardiogram
• Expert consensus statement
• Imaging
• Premature ventricular complex
• Radiofrequency ablation
• Ventricular arrhythmia
• Ventricular tachycardia

The clinical effect of copper accumulation on the heart of patients suffering from Wilson’s disease (WD) is not completely understood. We aimed to determine if patients with WD show signs of cardiac involvement, structural heart disease or autonomic dysfunction. In this prospective trial, we studied 61 patients (mean age 44.3 ± 15.2 years, 51% males) with WD and compared them to 61 age- and gender-matched healthy controls. All subjects underwent clinical examination, blood tests, echocardiography and 24 h electrocardiographic (ECG) recording.

Left- and right ventricular systolic function did not differ significantly between WD patients and controls. However, 5 of the 61 patients had a reduced left ventricular ejection fraction (LVEF). Furthermore, diastolic dysfunction was more prevalent in WD patients (9 of 61 vs. 0 of 61, p = 0.001).

The severity of WD based on the Unified Wilson’s Disease Rating Scale was significantly correlated to NT-pro BNP (r = 0.34, P = 0.013). Patients with an exacerbation of WD in medical history had higher troponin levels compared to those without (11.3 ± 4.7 vs 4.6 ± 1.2).

The autonomic function assessed by triangular index (TI) and SDNN-index was significantly reduced in WD patients compared to controls in most in almost every age category (p-value TI and SDNN: age 20–29, p < 0.001 and 0.05; age 30–39, p < 0.01 and not significant (ns); age 40–49, p < 0,01 and 0.001; age 50–59, p = ns and < 0.001, age 60–70, p < 0.05 and ns).

Our data demonstrate that cardiac involvement and autonomic dysfunction in WD is possible, however the underlying cause is still not known. We suggest that patients with signs and symptoms of structural heart disease should be examined by a cardiologist in addition to the interdisciplinary treatment team of WD.

The online version of this article (10.1186/s13023-019-1007-7) contains supplementary material, which is available to authorized users.

• Autonomic dysfunction
• Cardiac involvement
• Echocardiography
• Heart failure
• Unified Wilson’s disease rating scale
• Wilson’s disease

• Cardiac magnetic resonance
• idiopathic
• late gadolinium enhancement
• structural heart disease
• tissue characterization
• ventricular arrhythmias.

• Arrhythmias, Cardiac/diagnosis
• Arrhythmias, Cardiac/diagnostic imaging
• Arrhythmias, Cardiac/pathology
• Female
• Humans
• Magnetic Resonance Imaging/methods
• Male
• Tachycardia, Ventricular/diagnosis
• Tachycardia, Ventricular/diagnostic imaging
• Tachycardia, Ventricular/pathology

Left ventricular non-compaction (LVNC) is a rare cardiomyopathy associated with a hypertrabeculated phenotype and a large spectrum of symptoms. It is still unclear whether LVNC results from a defect of ventricular trabeculae development and the mechanistic basis that underlies the varying severity of this pathology is unknown. To investigate these issues, we inactivated the cardiac transcription factor Nkx2-5 in trabecular myocardium at different stages of trabecular morphogenesis using an inducible Cx40-creERT2 allele. Conditional deletion of Nkx2-5 at embryonic stages, during trabecular formation, provokes a severe hypertrabeculated phenotype associated with subendocardial fibrosis and Purkinje fiber hypoplasia. A milder phenotype was observed after Nkx2-5 deletion at fetal stages, during trabecular compaction. A longitudinal study of cardiac function in adult Nkx2-5 conditional mutant mice demonstrates that excessive trabeculation is associated with complex ventricular conduction defects, progressively leading to strain defects, and, in 50% of mutant mice, to heart failure. Progressive impaired cardiac function correlates with conduction and strain defects independently of the degree of hypertrabeculation. Transcriptomic analysis of molecular pathways reflects myocardial remodeling with a larger number of differentially expressed genes in the severe versus mild phenotype and identifies Six1 as being upregulated in hypertrabeculated hearts. Our results provide insights into the etiology of LVNC and link its pathogenicity with compromised trabecular development including compaction defects and ventricular conduction system hypoplasia.

During fetal heart morphogenesis, formation of the mature ventricular wall requires coordinated compaction of the inner trabecular layer and growth of the outer layer of myocardium. Arrested trabecular development has been implicated in the pathogenesis of hypertrabeculation associated with ventricular non-compaction cardiomyopathy. However much uncertainty still exists among clinicians concerning the physiopathology of ventricular non-compaction cardiomyopathy, including its clinical characteristics, prognosis, classification and even the definition of hypertrabeculation. In particular, distinguishing between pathological and non-pathological subtypes of non-compaction is currently a major issue. Here we show that deletion of the gene encoding the transcription factor Nkx2-5 at critical steps during trabecular development recapitulates pathological features of hypertrabeculation, providing the first model of ventricular non-compaction cardiomyopathy in adult mice. We demonstrate that excessive trabeculation due to failure of trabecular compaction during fetal development is associated with Purkinje fiber hypoplasia and subendocardial fibrosis. Longitudinal functional studies reveal that these mice present all the clinical signs of symptomatic left ventricular non-compaction cardiomyopathy, including conduction defects, strain defects and progressive heart failure. Our results, including transcriptomic analysis, suggest that pathological features of non-compaction are primarily developmental defects. This study clarifies the origin of the pathological outcomes associated with LVNC and may provide helpful information for clinicians concerning the etiology of this rare cardiomyopathy.

• cardiac magnetic resonance
• sports cardiology
• sudden cardiac death
• ventricular arrhythmias

• Adolescent
• Adult
• Age Factors
• Arrhythmias, Cardiac/diagnosis
• Arrhythmias, Cardiac/epidemiology
• Arrhythmias, Cardiac/mortality
• Arrhythmias, Cardiac/physiopathology
• Athletes
• Case-Control Studies
• Competitive Behavior
• Death, Sudden, Cardiac/epidemiology
• Female
• Heart Rate
• Humans
• Italy/epidemiology
• Male
• Prevalence
• Risk Factors
• Young Adult