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The spectrum of inherited arrhythmogenic diseases (IADs) includes disorders without overt structural abnormalities (i.e. primary inherited arrhythmia syndromes) and structural heart diseases (i.e. arrhythmogenic ventricular cardiomyopathy, hypertrophic cardiomyopathy). The aim of this European Heart Rhythm Association (EHRA) survey was to evaluate current clinical practice and adherence to 2015 European Society of Cardiology Guidelines regarding the management of patients with IADs. A 24-item centre-based online questionnaire was presented to the EHRA Research Network Centres and the European Cardiac Arrhythmia Genetics Focus Group members. There were 46 responses from 20 different countries. The survey revealed that 37% of centres did not have any dedicated unit focusing on patients with IADs. Provocative drug challenges were widely used to rule-out Brugada syndrome (BrS) (91% of centres), while they were used in a minority of centres during the diagnostic assessment of long-QT syndrome (11%), early repolarization syndrome (12%), or catecholaminergic polymorphic ventricular tachycardia (18%). While all centres advised family clinical screening with electrocardiograms for all first-degree family members of patients with IADs, genetic testing was advised in family members of probands with positive genetic testing by 33% of centres. Sudden cardiac death risk stratification was straightforward and in line with current guidelines for hypertrophic cardiomyopathy, while it was controversial for other diseases (i.e. BrS). Finally, indications for ventricular mapping and ablation procedures in BrS were variable and not in agreement with current guidelines in up to 54% of centres., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2020. For permissions, please email: journals.permissions@oup.com.)

Sudden cardiac death (SCD) is a leading cause of death. The advent of the implantable cardioverter‑defibrillator (ICD) has revolutionized prevention of SCD in high‑risk patients with underlying cardiac diseases. However, several challenges remain. Identification of patients at risk who should receive an ICD is suboptimal, and the sole criterion applied in clinical practice is a severely reduced left ventricular ejection fraction despite the fact that SCD occurs mostly in patients with preserved or mildly reduced ejection fraction. Additionally, the majority of patients that do receive the ICD will not benefit from the device at the end. Therefore, improved risk stratification approaches to guide selection of patients for ICD implantation are definitely needed. There are several novel features and developments in the field with the subcutaneous defibrillator being probably the most important one and having the potential to substantially influence clinical practice. The role of catheter ablation of ventricular tachycardia, and particularly the potential to abolish the need for ICD implantation at least in selected patient groups, should be further defined. Internists and general practitioners play a significant role in the management of ICD patients, from identification of candidates for ICD implantation to early detection and appropriate treatment of complications.

Prevention of sudden cardiac death (SCD) remains a partly unsolved task in cardiology. The European Society of Cardiology (ESC) guidelines on management of patients with ventricular arrhythmias and prevention of SCD published in 2015 considered the new insights of the natural history of diseases predisposing to SCD. The guidelines improved strategies for management of patients at risk of SCD and included both drug and device therapies. The intention of this survey was to evaluate the extent of the disparities between daily clinical practice and the 2015 SCD ESC guidelines among electrophysiology centres in Europe. The results suggest that the adherence to guidelines is reasonably high and strategies for the management of ischaemic disease are well-established. Implantable cardioverter-defibrillator indications for primary prevention are a difficult topic, particularly in non-ischaemic dilated cardiomyopathy. Disparities in the use of genetic testing are probably due to differences in local availability.

The aim of this European Heart Rhythm Association (EHRA) prospective snapshot survey is to assess peri-procedural practices, implantation techniques, and short-term procedure-related complications associated with implantation of subcutaneous implantable cardioverter-defibrillator (S-ICD) or transvenous implantable cardioverter-defibrillator (TV-ICD), across tertiary European electrophysiology centres. An internet-based electronic questionnaire concerning implantation settings, peri-procedural routines, techniques, personnel, complications, and patient outcomes was sent to the centres routinely implanting both TV-ICDs and S-ICDs. The centres were requested to prospectively include consecutive patients implanted with either TV-ICD or S-ICD during the 8-week enrolment period. Overall, 20 centres from 6 countries enrolled 429 consecutive patients. Subcutaneous implantable cardioverter-defibrillators (20%) compared with TV-ICD were implanted mainly under general anaesthesia (72% vs. 14%), in the surgical operation room settings (69% vs. 43%), with more frequent prophylactic antibiotic administration (82% vs. 91%), and post-implant defibrillation testing (85% vs. 7%, all P < 0.05). Feasibility (implantation duration of 45 min) and short-term complication rates (4%) were comparable for S-ICDs and TV-ICDs, but the spectrum of complications varied, despite different baseline characteristics of patients undergoing the S-ICD vs. TV-ICD implantation. This EHRA snapshot survey provides important insights into the implantable cardioverter-defibrillator implantation routines and patient outcomes. Our study showed differences between the S-ICD and TV-ICD implantation routines with respect to implantation settings, peri-procedural management, and pre-defined procedural endpoints. However, the comparable duration of S-ICD or TV-ICD implantation and similar rates of peri-procedural complications indicate that both devices can be routinely used in clinical practice.

The purpose of this European Heart Rhythm Association (EHRA) survey is to provide an overview of the current use of leadless pacemakers (LLPM) across a broad range of European centres. An online questionnaire was sent to centres participating in the EHRA Electrophysiology Research Network. Questions dealt with standards of care and policies used for patient management, indications, and techniques of implantation of LLPM. In total, 52 centres participated in the survey. Most (86%) reported using LLPM, although 82% of these centres implanted <30 LLPM devices during the last 12 months. Non-availability (36%), lack of reimbursement (55%), and cost of the device (91%) were factors limiting the use of LLPM. The most commonly reported indications for LLPM were permanent atrial fibrillation (83%), a history of complicated conventional pacemaker (87%), or an anticipated difficult vascular access (91%). Implantation of LLPM is perceived as an easy-to-do and safe procedure by most implanters (64%), while difficult or risky in 28%, and comparable to conventional pacemakers by only a few (8%). Local vascular complications were the most frequently reported major problems (28%), but a significant number of respondents (36%) have never encountered any issue after LLPM implantation. Although cost and reimbursement issues strongly influence the use of LLPM, most respondents (72%) anticipate a significant increase in device utilization within next 2 years.

The purpose of this survey was to assess the current practice in Europe regarding cryoablation for treatment of different cardiac arrhythmias. The data are based on an electronic questionnaire sent to members of the European Heart Rhythm Association Research Network. Responses were received from 49 centres in 18 countries. The results show that cryoablation for supraventricular tachycardia in European centres is an alternative to radiofrequency ablation, which is in accordance with guidelines. There is reasonable consensus regarding clinical results and complications of cryoablation procedure. Some inter-centre variability with respect to patient selection and ablation strategy in cryoablation of atrial fibrillation was demonstrated, underscoring the need for further research.

Background: Incidence and clinical significance of transient, daily fluctuations of biventricular pacing percentage (CRT%) remain unknown. We assessed the value of daily remote monitoring in identifying prognostically critical burden of low CRT%., Methods and Results: Prospective, single-centre registry encompassed 304 consecutive heart failure patients with cardiac resynchronization therapy defibrillators (CRT-D). Patients with 24-h episodes of CRT% loss<95% were assigned to quartiles depending on cumulative time spent in low CRT%: quartile 1 (1-8days), 2 (9-20days), 3 (21-60days) and quartile 4 (>60days). During median follow-up of 35months 51,826 transmissions were analysed, including 15,029 in 208 (68.4%) patients with episodes of low CRT%. Overall, mean CRT%≥95% vs. <95% resulted in a 4-fold lower mortality (17.3 vs. 68.2%; p<0.001). Fifty-four percent of patients experienced episodes of CRT% loss, despite 85.6% having mean CRT%≥95%. Mortality was lowest in quartile 1 (7.7%), while longer periods of CRT% loss resulted in significantly higher death rates (25.0 vs. 34.6 vs. 57.7%; quartiles 2-4 respectively, p<0.001), despite mean CRT% still being ≥95% in quartiles 1-3. Cumulative low CRT% burden was the independent risk factor for death (HR 1.013; 95% CI 1.006-1.021; p<0.001). Mortality rose by 1.3 and 49% with every additional day and quartile of CRT% loss, respectively., Conclusions: Daily remote monitoring allows one to detect 24-h episodes of CRT% loss<95% in over two-thirds of CRT-D recipients during median observation of 3years. Cumulative low CRT% burden (in days) independently predicts mortality before mean CRT% drop., (Copyright © 2016. Published by Elsevier Ireland Ltd.)

Background: The aim of this study was to evaluate the value of device-based diagnostic parameters in predicting ventricular arrhythmias in cardiac resynchronization therapy (CRT) recipients., Methods: Ninety-six CRT-D patients participating in TRUST CRT Trial were analyzed. The inclusion criteria were: heart failure in NYHA ≥ 3 class, QRS ≥ 120 ms, LVEF £ 35% and significant mechanical dyssynchrony. Patients were divided into those with (n = 31, 92 arrhythmias) and without (n = 65) appropriate ICD interventions within follow-up of 12.03 ± 6.7 months. Daily monitored device-based parameters: heart rate (HR), thoracic impedance (TI), HR variability and physical activity were analyzed in 4 time windows: within 10, 7, 3 days and 1 day before appropriate ICD interventions., Results: A consistent pattern of changes in three monitored factors was observed prior to arrhythmia: 1) a gradual increase of day HR (from 103.43% of reference within 10-day window to 105.55% one day before, all p < 0.05 vs. reference); 2) variations in night HR (104.75% in 3 days, 107.65% one day before, all p < 0.05) and 3) TI decrease (from 97.8% in 10 days to 96.81% one day before, all p < 0.05). The combination of three parameters had better predictive value, which improved further after exclusion of patients with atrial fibrillation (AF). The predictive model combining HR and TI together with LVEF and NT-proBNP was more prognostic than the model involving LVEF and NT-proBNP alone (difference in AUC 0.05, 95% CI 0.0005-0.09, p = 0.04)., Conclusions: Daily device-monitored parameters show significant variations prior to ventricular arrhythmia. Combination of multiple parameters improves arrhythmia predictive performance by its additive value to baseline risk factors, while presence of AF diminishes it.

Background: Prediction of recurrent malignant ventricular tachyarrhythmias after insertion of a implantable cardioverter-defibrillator (ICD) is challenging. Microvolt T-wave alternans (MTWA) seems to be a promising marker of such events in ICD recipients., Aim: To assess prognostic significance of MTWA and other noninvasive parameters in the prediction of major arrhythmic events after ICD implantation., Methods: This prospective study included 155 patients (121 male, age 59 ± 11 years) in whom ICD was implanted for secondary prevention of a sudden cardiac death. In all patients, clinical evaluation along with estimation of ejection fraction, MTWA measurement using the HearTwave Cambridge Heart system, and determination of the corrected QT interval (QTc) and QT dispersion (QTd) based on resting ECG were performed 3 days before ICD implantation. Using 24-h Holter monitoring, cardiac arrhythmias, QT interval, QT dynamicity, QT variability (QTSD) and heart rate variability (HRV) time domain parameters were determined. MTWA results were categorised, based on the accepted criteria, as positive, negative or indeterminate. In further analyses, positive and indeterminate MTWA results were grouped together as abnormal or non-negative tests [MTWA+], while negative MTWA results were considered normal [MTWA-]. During the follow-up (mean duration 21.6 ± 11.6 months), major arrhythmic cardiac events (MACE), defined as death and/or the need for ablation and/or heart transplantation due to malignant ventricular tachyarrhythmias, were recorded., Results: During the follow-up, MACE occurred in 17 (11%) patients. Abnormal MTWA before ICD implantation was found significantly more frequently in patients with MACE as compared to patients without MACE. Multivariate Cox regression analysis identified abnormal MTWA and QTSD as independent risk factors for MACE, with hazard ratios of 10.82 (95% CI 9.76-11.88; p〈 0.05) and 1.08 (95% CI 1.05-1.08), respectively. Significant differences in MACE-free survival rate with regard to MTWA results (abnormal vs normal MTWA) were shown during the follow-up (p〈 0.001). The negative predictive value of normal MTWA for MACE was 98.6%. When both MTWA and QTSD were combined, the positive predictive value increased to 35%, with a sensitivity of 82% and specificity of 81%. The probability of MACE with normal results of both these tests was 2.3%., Conclusions: Abnormal MTWA is a strong independent predictor of MACE in ICD recipients, and QTSD is a weaker predictor. In the prediction of MACE after ICD implantation, the highest predictive value was noted for abnormal MTWA combined with QTSD. Normal values of these two parameters were associated with a low probability of MACE. These results suggest that standardised MTWA evaluation can be useful for risk stratification in the clinical practice.

Background: Cardiac resynchronization therapy (CRT) improves the clinical status of patients with heart failure (HF), though its effects on heart rate turbulence (HRT) are unknown., Methods: We measured HRT indices in 58 recipients of CRT systems (mean age = 56 +/- 9 years, 41 men) in New York Heart Association HF functional class III-IV, and with a left ventricular (LV) ejection fraction < or =35%. At 6 months of follow-up, 42 patients were responders and 13 nonresponders to CRT, and three patients died suddenly. The HRT indices turbulence onset (TO%) and turbulence slope (TS ms/RR interval) were calculated from digital 24-hour electrocardiogram before and after 6 months of CRT. TO > or = 0% and TS < or = 2.5 ms/RR interval were considered abnormal., Results: Mean TO in the entire population was 0.4 +/- 1.5 before CRT, and decreased to -0.8 +/- 7.0 during the 6 months of CRT (ns). TS increased significantly from 2.0 +/- 1.7 at baseline, to 3.9 +/- 3.1 (P < 0.05), and a significantly lower proportion of patients had abnormal HRT indices at 6 months. In contrast to the significant increase observed in responders, not significant change in TS was observed among the nonresponders., Conclusions: During 6 months of CRT, improvements in HRT indices and a decrease in the proportion of patients with abnormal HRT were observed. CRT may have beneficial effects on baroreflex sensitivity.

Background: Radiofrequency current ablation (RFCA) is a safe and efficient method of treatment in adult patients with symptomatic arrhythmias. Recently RFCA is being also used in the treatment of children with cardiac arrhythmias, however its value in this set of patients is poorly documented. Aims of our study were to assess the feasibility and safety of RFCA procedures in children with symptomatic supraventricular and ventricular arrhythmias, and to identify the parameters which influence the probability of acute success and RFCA course., Methods: Consecutive 110 patients aged less than 18, who underwent RFCA due to the symptomatic arrhythmias at our center were included into a retrospective analysis. The patients presented mainly with the preexcitation syndrome (57%), 13.6% had ectopic ventricular arrhythmias, in 17.3% nodal reentrant tachycardia, in 8.2% intraatrial reentry tachycardia (IART), whereas in 5.4% ectopic atrial tachycardia (EAT) was diagnosed. Three patients had multiple arrhythmias. In ten subjects congenital heart defect was diagnosed in the past, which was treated surgically in 8. Analyzed parameters included: RFCA success-rate, procedure duration, fluoroscopy-time, perioperative complications and arrhythmia recurrence-rate during 6 months follow-up., Results: Ablation was successful in 101 (91.8%) patients and abolished 104 out of 113 (92%) treated arrhythmias. Out of 9 patients with unsuccessful RFCA, three had congenital heart defect. Two complications occurred intra-operatively in two patients: in one patient pneumothorax was observed following jugular vein puncture, which resolved spontaneously without the need of drainage, in the second patient transient atrioventricular conduction block occurred during radiofrequency current application. Mean procedure duration in the studied population was 118.9 +/- 46 min, fluoroscopy exposure equaled 22.3 +/- 17min. In twelve patients (10.9%) arrhythmia recurred during the follow-up period. The longest procedure duration was observed in patients with EAT and IART, the longest fluoroscopy-time in subjects with IART, whereas the shortest fluoroscopy was observed in patients with the ventricular ectopic arrhythmias. Success-rates, complications, and recurrence-rates did not differ irrespectively from arrhythmia treated. The only independent predictor of unsuccessful RFCA was the presence of congenital cardiac defect (adjusted OR 0.15, p<0.05). The presence of cardiac defect, procedure performed without electroanatomic mapping system and less experienced operators were the parameters associated with longer fluoroscopic exposure., Conclusions: Radiofrequency current ablation is a safe and efficient method of treatment in children with arrhythmias. The presence of congenital heart defect was a factor influencing unfavorably the probability of successful RFCA. Procedural course was related to the experience of electrophysiology team, the use of advanced mapping systems and presence of cardiac defect.

We report on a case of a 62-year-old patient with symptomatic heart failure and severe ventricular electrical and mechanical dyssynchrony, who was implanted percutaneously with a triple site (dual-left single-right) resynchronization device. At 3-months follow-up, the patient's functional status improved significantly as shown by subjective and objective tests. Furthermore, this mode of pacing has allowed nearly complete inter- and intraventricular mechanical resynchronization.