Management of patients with an electrical storm or clustered ventricular arrhythmias: a clinical consensus statement of the European Heart Rhythm Association of the ESC-endorsed by the Asia-Pacific Heart Rhythm Society, Heart Rhythm Society, and Latin-American Heart Rhythm Society.

Electrical storm (ES) is a state of electrical instability, manifesting as recurrent ventricular arrhythmias (VAs) over a short period of time (three or more episodes of sustained VA within 24 h, separated by at least 5 min, requiring termination by an intervention). The clinical presentation can vary, but ES is usually a cardiac emergency. Electrical storm mainly affects patients with structural or primary electrical heart disease, often with an implantable cardioverter-defibrillator (ICD). Management of ES requires a multi-faceted approach and the involvement of multi-disciplinary teams, but despite advanced treatment and often invasive procedures, it is associated with high morbidity and mortality. With an ageing population, longer survival of heart failure patients, and an increasing number of patients with ICD, the incidence of ES is expected to increase. This European Heart Rhythm Association clinical consensus statement focuses on pathophysiology, clinical presentation, diagnostic evaluation, and acute and long-term management of patients presenting with ES or clustered VA.

Predictors of ventricular tachyarrhythmia in patients with implantable cardioverter-defibrillator and non-ischemic systolic heart failure.

BACKGROUND: The benefit derived from implantable cardioverter-defibrillators (ICD) in subjects with non-ischemic systolic HF (NICM) is less well-established. AIM: The study aimed to determine the incidence, predictors, and prognostic impact of ventricular arrhythmias in patients with ICD and NICM. METHODS: The study sample included 377 consecutive patients with ICD or cardiac resynchronization cardioverter-defibrillators (CRT-D, 74% of patients) and NICM implanted and monitored remotely in a university hospital. RESULTS: During the median (interquartile range [IQR]) follow-up of 1645 (960-2675) days, sustained ventricular arrhythmia occurred in 92 patients (24.4%). Of those, ventricular fibrillation (VF), ventricular tachycardia (VT), and both VT and VF occurred in 10 (10.9%), 72 (78.3%), and 10 (10.9%) patients, respectively. Patients with vs. those without ventricular arrhythmia differed concerning sex, left ventricular end-diastolic diameter (LVEDD), left ventricular end-systolic diameter (LVESD), left ventricular ejection fraction (LVEF), post-inflammatory etiology, atrial fibrillation/flutter occurrence, and supraventricular arrhythmia (SVT) other than AF/AFL during follow-up. In multivariable Cox regression, LVEDD (hazard ratio [HR], 1.05; 95% confidence interval [CI], 1.003-1.09; P = 0.03), AF/AFL (HR, 1.86; 95% CI, 1.21-2.85; P = 0.004), and SVT (HR, 1.77; 95% CI, 1.10-2.87; P = 0.02) were independent predictors of sustained VT, while AF/AFL (HR, 1.65; 95% CI, 1.07-2.56; P = 0.02) was independent predictor of VF. All-cause mortality in patients with VT/VF was significantly higher than in subjects without sustained ventricular arrhythmias (35.9% vs. 22.4%; P = 0.01). CONCLUSIONS: Ventricular arrhythmia occurred in every fourth patient with NICM and ICD during 4.5 years of observation and was associated with significantly worse prognosis than in subjects free of VT/VF. Higher LVEDD, atrial fibrillation/atrial flutter, and supraventricular tachycardia flag patients at risk of ventricular arrhythmia.

Impact of cryoballoon application abortion due to phrenic nerve injury on reconnection rates: a YETI subgroup analysis.

AIMS: Cryoballoon (CB)-based pulmonary vein isolation (PVI) is an effective treatment for atrial fibrillation (AF). The most frequent complication during CB-based PVI is right-sided phrenic nerve injury (PNI) which is leading to premature abortion of the freeze cycle. Here, we analysed reconnection rates after CB-based PVI and PNI in a large-scale population during repeat procedures. METHODS AND RESULTS: In the YETI registry, a total of 17 356 patients underwent CB-based PVI in 33 centres, and 731 (4.2%) patients experienced PNI. A total of 111/731 (15.2%) patients received a repeat procedure for treatment of recurrent AF. In 94/111 (84.7%) patients data on repeat procedures were available. A total of 89/94 (94.7%) index pulmonary veins (PVs) have been isolated during the initial PVI. During repeat procedures, 22 (24.7%) of initially isolated index PVs showed reconnection. The use of a double stop technique did non influence the PV reconnection rate (P = 0.464). The time to PNI was 140.5 ± 45.1 s in patients with persistent PVI and 133.5 ± 53.8 s in patients with reconnection (P = 0.559). No differences were noted between the two populations in terms of CB temperature at the time of PNI (P = 0.362). The only parameter associated with isolation durability was CB temperature after 30 s of freezing. The PV reconnection did not influence the time to AF recurrence. CONCLUSION: In patients with cryoballon application abortion due to PNI, a high rate of persistent PVI rate was found at repeat procedures. Our data may help to identify the optimal dosing protocol in CB-based PVI procedures. CLINICAL TRIAL REGISTRATION: https://clinicaltrials.gov/ct2/show/NCT03645577?term=YETI&cntry=DE&draw=2&rank=1 ClinicalTrials.gov Identifier: NCT03645577.

The implantation of AV leadless pacemaker - a case report.

In early 2020 Food and Drug Administration (FDA) approved Micra AV - a new type of leadless pacemaker with atrioventricular synchrony, to treat patients with atrio-ventricular (AV) blocks. We describe one of the first in Poland case of a patient who was implanted with Micra AV pacemaker. CASE REPORT: A 38-year-old female patient was admitted to the clinic due to the 29-seconds event of a complete AV block without an escape rhythm and was implanted with a dual chamber pacemaker without any complications. After several months she was admitted again with suspicion of ventricular perforation by the pacemaker electrode and underwent a replacement procedure of both pacemaker's leads. Nevertheless, one week later the patient developed a fever with significantly elevated inflammatory markers. The blood cultures were negative but in the transesophageal echocardiography features of cardiac device-related infective endocarditis were observed. Empirical antibiotic therapy was administered, and the device was removed. The Heart Team qualified the patient for the implantation of a MicraTM AV leadless pacemaker. The procedure was performed without any complications and the device was implanted to the right ventricle. All parameters were correct, and the patient was discharged. CONCLUSIONS: Micra AV may be a feasible and safe option for young patients with paroxysmal AV block after device-related complications.

Quality of life in patients with a subcutaneous vs. transvenous implantable cardioverter-defibrillator.

BACKGROUND: The implantable cardioverter-defibrillator (ICD) and subcutaneous ICD (S-ICD) are well-accepted life-saving devices for treating potentially lethal ventricular arrhythmia, but little is known about quality of life (QoL) in patients with S-ICD and ICD. AIMS: Our study aimed to compare QoL in patients with S-ICD and ICD. METHODS: All consecutive patients who had S-ICD implanted between October 2015 and September 2021 were included in the study. A cohort of transvenous ICD (TV-ICD) patients was matched to S-ICD subjects by sex, age, indications for the device, and type of prevention. All patients were requested to fulfill two standardized questionnaires to assess QoL: 36-Item Short Form Health Survey (SF-36) and Minnesota Living with Heart Failure Questionnaire (MLHFQ) 6 months after device implantation. RESULTS: Patients with S-ICD (n = 49) and TV-ICD (n = 49) did not differ regarding baseline characteristics. There were no statistically significant differences between S-ICD and TV-ICD subgroup, both for mental and physical QoL assessed in SF-36 and MLHFQ (all P = NS). The median MLHFQ total score was 24 (9-41) for S-ICD and 28 (14-43) for TV-ICD (P = 0.83). The median total score for the SF-36 questionnaire was 62.5 (29-86) vs. 59 (38-77) for S-ICD and TV-ICD, respectively (P = 0.78). CONCLUSIONS: Quality of life after device implantation does not differ significantly between the groups of patients with subcutaneous and conventional implantable cardioverter-defibrillator.

Phrenic Nerve Injury During Cryoballoon-Based Pulmonary Vein Isolation: Results of the Worldwide YETI Registry.

BACKGROUND: Cryoballoon-based pulmonary vein isolation (PVI) has emerged as an effective treatment for atrial fibrillation. The most frequent complication during cryoballoon-based PVI is phrenic nerve injury (PNI). However, data on PNI are scarce. METHODS: The YETI registry is a retrospective, multicenter, and multinational registry evaluating the incidence, characteristics, prognostic factors for PNI recovery and follow-up data of patients with PNI during cryoballoon-based PVI. Experienced electrophysiological centers were invited to participate. All patients with PNI during CB2 or third (CB3) and fourth-generation cryoballoon (CB4)-based PVI were eligible. RESULTS: A total of 17 356 patients underwent cryoballoon-based PVI in 33 centers from 10 countries. A total of 731 (4.2%) patients experienced PNI. The mean time to PNI was 127.7±50.4 seconds, and the mean temperature at the time of PNI was -49±8°C. At the end of the procedure, PNI recovered in 394/731 patients (53.9%). Recovery of PNI at 12 months of follow-up was found in 97.0% of patients (682/703, with 28 patients lost to follow-up). A total of 16/703 (2.3%) reported symptomatic PNI. Only 0.06% of the overall population showed symptomatic and permanent PNI. Prognostic factors improving PNI recovery are immediate stop at PNI by double-stop technique and utilization of a bonus-freeze protocol. Age, cryoballoon temperature at PNI, and compound motor action potential amplitude loss >30% were identified as factors decreasing PNI recovery. Based on these parameters, a score was calculated. The YETI score has a numerical value that will directly represent the probability of a specific patient of recovering from PNI within 12 months. CONCLUSIONS: The incidence of PNI during cryoballoon-based PVI was 4.2%. Overall 97% of PNI recovered within 12 months. Symptomatic and permanent PNI is exceedingly rare in patients after cryoballoon-based PVI. The YETI score estimates the prognosis after iatrogenic cryoballoon-derived PNI. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT03645577. Graphic Abstract: A graphic abstract is available for this article.

Radiofrequency catheter ablation as a treatment option in a patient with hypoplastic left heart syndrome and atrial flutter after Fontan operation-Case report.

We report a 15-year-old male with hypoplastic left heart syndrome (HLHS) after Fontan operation with recurrent, drug-resistant atrial tachycardia. With the use of electro-anatomical mapping system (EnSite) an atrial flutter (AFl) with reentry activation around the tricuspid valve was diagnosed. Successful radiofrequency catheter ablation (RFCA) was performed.

Utilization of Subcutaneous Cardioverter-Defibrillator in Poland and Europe-Comparison of the Results of Multi-Center Registries.

The implantation of a subcutaneous cardioverter-defibrillator (S-ICD) may be used instead of a traditional transvenous system to prevent sudden cardiac death. Our aim was to compare the characteristics of S-ICD patients from the multi-center registry of S-ICD implantations in Poland with the published results of the European Snapshot Survey on S-ICD Implantation (ESSS-SICDI). We compared data of 137 Polish S-ICD patients with 68 patients from the ESSS-SICDI registry. The groups did not differ significantly in terms of sex, prevalence of ischemic cardiomyopathy, concomitant diseases, and the rate of primary prevention indication. Polish patients had more advanced heart failure (New York Heart Association (NYHA) class III: 11.7% vs. 2.9%, NYHA II: 48.9% vs. 29.4%, NYHA I: 39.4% vs. 67.7%, p < 0.05 each). Young age (75.9% vs. 50%, p < 0.05) and no vascular access (7.3% vs. 0%, p < 0.05) were more often indications for S-ICD. The percentage of patients after transvenous system removal due to infections was significantly higher in the Polish group (11% vs. 1.5%, p < 0.05). In the European population, S-ICD was more frequently chosen because of patients' active lifestyle and patients' preference (both 10.3% vs. 0%, p < 0.05). Our analysis shows that in Poland, compared to other European countries, subcutaneous cardioverters-defibrillators are being implanted in patients at a more advanced stage of chronic heart failure. The most frequent reason for choosing a subcutaneous system instead of a transvenous ICD is the young age of a patient.

Clinical manifestations of device-related infective endocarditis in cardiac resynchronization therapy recipients.

INTRODUCTION: The aim of the study was to analyse microbiological characteristics and clinical manifestations of cardiac device-related infective endocarditis (CDRIE) in cardiac resynchronization therapy (CRT) recipients, and to compare the diagnostic value of modified Duke (MDC) versus modified Duke lead criteria (MDLC; including to MDC local infection and pulmonary infection or embolism as major criteria). MATERIAL AND METHODS: The study population comprised 765 consecutive CRT patients from a high-volume, tertiary care centre from 2002 to 2015. All patients were screened for CDRIE. RESULTS: During a median follow-up of 1692 days (range: 457-3067) 5.36% of patients (n = 41) developed CDRIE, which was accompanied by CRT pocket infection in 17.1% (n = 7) and recurrent pulmonary infection or pulmonary embolism in 29.3% (n = 12). Fever was present in 95.1% of patients (n = 39), whereas blood cultures were positive in 65.9% (n = 27). Staphylococcus was the most prevalent pathogen in 59.3% (n = 16), Gram-negative bacteria in 25.9% (n = 7). Transoesophageal echocardiography showed intracardiac vegetations in 73.2% of patients (n = 30). Non-different pathogen types with the most common methicillin-sensitive Staphylococcus aureus were observed for early versus late CDRIE (endocarditis ≤ 6 vs. > 6 months from CRT or other device-related procedure). All 3 inflammatory markers (C-reactive protein, white blood cells, procalcitonin) were normal in 4.9% of patients (n = 2). MDC versus MDLC indicated definite CDRIE in 48.8% versus 80.5%, respectively (p = 0.003). CONCLUSIONS: Fever is the most common symptom of CRT-related CDRIE, and transoesophageal echocardiography allows vegetations to be visualised in nearly 3/4 of patients with CDRIE. Although the most common pathogens were Staphylococci, Gram-negative bacteria accounted for a quarter of CDRIE. Modified Duke lead criteria proved superior to MDC.

Upgrade from implantable cardioverter-defibrillator vs. de novo implantation of cardiac resynchronization therapy: long-term outcomes.

AIMS: To assess and compare long-term mortality and predictors thereof in de novo cardiac resynchronization therapy defibrillators (CRT-D) vs. upgrade from an implantable cardioverter-defibrillator (ICD) to CRT-D. METHODS AND RESULTS: Study population consisted of 595 consecutive patients with CRT-D implanted between 2002 and 2015 in a tertiary care, university hospital, in a densely inhabited, urban region of Poland [480 subjects (84.3%) with CRT-D de novo implantation; 115 patients (15.7%) upgraded from ICD to CRT-D]. In a median observation of 1692 days (range 457-3067), all-cause mortality for de novo CRT-D vs. CRT-D upgrade was 35.5% vs. 43.5%, respectively (P = 0.045). On multivariable regression analysis including all CRT recipients, the previously implanted ICD was an independent predictor for death [hazard ratio (HR) 1.58, 95% confidence interval (CI) 1.10-2.29, P = 0.02]. For those, who were upgraded from ICD to CRT-D, the independent predictors for all-cause death were as follows: creatinine level (HR 1.01, 95% CI 1.00-1.02, P = 0.01), left ventricular end-systolic diameter (HR 1.07, 95% CI 1.02-1.11, P = 0.002), New York Heart Association (NYHA) IV class at baseline (HR 2.36, 95% CI 1.00-5.53, P = 0.049) and cardiac device-related infective endocarditis during follow-up (HR 2.42, 95% CI 1.02-5.75, P = 0.046). A new CRT scale (Creatinine ≥150 µmol/L; Remodelling, left ventricular end-systolic ≥59 mm; Threshold for NYHA, NYHA = IV) showed high prediction for mortality in CRT-D upgrades (AUC 0.70, 95% CI 0.59-0.80, P = 0.0007). CONCLUSION: All-cause mortality in patients upgraded from ICD is significantly higher compared with de novo CRT-D implantations and reaches almost 45% within 4.5 years. A new CRT scale (Creatinine; Remodelling; Threshold for NYHA) has been proposed to help survival prediction following CRT upgrade.

Successful implantation of leadless pacemakers in children: a case series.

BACKGROUND: A leadless pacemaker is a new concept in which a miniaturized pacing device is self-contained within the heart. Recently published data show that leadless pacemakers are associated with a decreased risk of major complications when compared with transvenous cardiac pacemakers. This seems to be of particular importance in children and young adults in whom various complications may occur during their lifetime. CASE SUMMARY: Herein, we report the successful implantation of Micra™ Transcatheter Pacing System in two children: 12-year-old boy and 13-year-old girl, along with a long-term follow-up. The children had indications for pacemaker implantation, however, with an expected low percentage of pacing due to paroxysmal nature of the third-degree atrioventricular block. The implantation procedures were performed in general anaesthesia. There were no complications. During the 2-year follow-up, there were no adverse events and the electrical parameters of the device remained stable. Pacing percentage was below 0.1%. DISCUSSION: Transvenous cardiac pacemakers improve quality of life and reduce mortality but may be associated with various short- and long-term complications, mainly related to the presence of transvenous leads and the pulse generator. Compared with adult patients, the implantation of conventional pacemakers in children is still a challenge, not only because of their smaller size but also due to continuing growth, as well as a higher rate of lead and device-related complications. We demonstrate that the implantation of leadless pacemakers in children is feasible and could be worth considering in certain clinical scenarios, especially when ventricular pacing is required rarely.

Contemporary management of patients with syncope in clinical practice: an EHRA physician-based survey.

Syncope is a heterogeneous syndrome encompassing a large spectrum of mechanisms and outcomes. The European Society of Cardiology published an update of the Syncope Guidelines in 2018. The aim of the present survey was to capture contemporary management of syncope and guideline implementation among European physicians. A 23-item questionnaire was presented to 2588 European Heart Rhythm Association (EHRA) members from 32 European countries. The response rate was 48%, but only complete responses (n = 161) were included in this study. The questionnaire contained specific items regarding syncope facilities, diagnostic definitions, diagnostic tools, follow-up, and therapy. The survey revealed that many respondents did not have syncope units (88%) or dedicated management algorithms (44%) at their institutions, and 45% of the respondents reported syncope-related hospitalization rates >25%, whereas most (95%) employed close monitoring and hospitalization in syncope patients with structural heart disease. Carotid sinus massage, autonomic testing, and tilt-table testing were inconsistently used. Indications were heterogeneous for implanted loop recorders (79% considered them for recurrent syncope in high-risk patients) or electrophysiological studies (67% considered them in bifascicular block and inconclusive non-invasive testing). Non-pharmacological therapy was consistently considered by 68% of respondents; however, there was important variation regarding the choice of drug and device therapy. While revealing an increased awareness of syncope and good practice, our study identified important unmet needs regarding the optimal management of syncope and variable syncope guideline implementation.

The effect of sacubitril / valsartan on the occurrence of ventricular arrhythmia and the risk of sudden cardiac death in patients with chronic heart failure with reduced left ventricular ejection fraction. Expert opinion of the Heart Rhythm and Heart Failure Sections of the Polish Cardiac Society.

Exacerbation of chronic heart failure (HF) is the most common cause of hospitalization in adults, which is associated with high morbidity and mortality rates, mainly due to HF exacerbation or sudden cardiac death (SCD). A novelty in the treatment of HF with reduced left ventricular ejection fraction (HFrEF) in recent years has been the approval of sacubitril / valsartan, a drug belonging to angiotensin receptor-neprilysin inhibitors (ARNIs). Sacubitril / valsartan significantly reduces the severity of HF symptoms as well as the risk of hospitalization and death and is characterized by a good safety profile. Therefore, it has a strong position in the guidelines of international cardiac societies. However, the precise mechanism underlying the beneficial effects of ARNIs on cardiovascular mortality is unknown. The advantages of ARNIs are likely to result from improved left ventricular ejection fraction, reduced myocardial remodeling, and increased natriuretic peptide availability. Therefore, sacubitril / valsartan may exhibit antiarrhythmic properties and reduce the risk of ventricular arrhythmias and SCD in patients with HFrEF. Importantly, the improvement of the function and electrical stabilization of cardiomyocytes may translate into a reduced risk of appropriate and inappropriate implantable cardioverter­defibrillator interventions and improvement in the percentage of biventricular pacing. In this expert opinion of the Heart Rhythm and Heart Failure Sections of the Polish Cardiac Society, we summarize and discuss the current knowledge on the effect of sacubitril / valsartan on the occurrence of ventricular arrhythmias and the risk of SCD in patients with chronic HFrEF.

Cardiac arrhythmias in the emergency settings of acute coronary syndrome and revascularization: an European Heart Rhythm Association (EHRA) consensus document, endorsed by the European Association of Percutaneous Cardiovascular Interventions (EAPCI), and European Acute Cardiovascular Care Association (ACCA).

Despite major therapeutic advances over the last decades, complex supraventricular and ventricular arrhythmias (VAs), particularly in the emergency setting or during revascularization for acute myocardial infarction (AMI), remain an important clinical problem. Although the incidence of VAs has declined in the hospital phase of acute coronary syndromes (ACS), mainly due to prompt revascularization and optimal medical therapy, still up to 6% patients with ACS develop ventricular tachycardia and/or ventricular fibrillation within the first hours of ACS symptoms. Despite sustained VAs being perceived predictors of worse in-hospital outcomes, specific associations between the type of VAs, arrhythmia timing, applied treatment strategies and long-term prognosis in AMI are vague. Atrial fibrillation (AF) is the most common supraventricular tachyarrhythmia that may be asymptomatic and/or may be associated with rapid haemodynamic deterioration requiring immediate treatment. It is estimated that over 20% AMI patients may have a history of AF, whereas the new-onset arrhythmia may occur in 5% patients with ST elevation myocardial infarction. Importantly, patients who were treated with primary percutaneous coronary intervention for AMI and developed AF have higher rates of adverse events and mortality compared with subjects free of arrhythmia. The scope of this position document is to cover the clinical implications and pharmacological/non-pharmacological management of arrhythmias in emergency presentations and during revascularization. Current evidence for clinical relevance of specific types of VAs complicating AMI in relation to arrhythmia timing has been discussed.

Comparison of transesophageal and intracardiac echocardiography in guiding percutaneous left atrial appendage closure with an Amplatzer Amulet device.

INTRODUCTION: Percutaneous occlusion of the left atrial appendage (LAAO) is becoming an extensively used method of stroke prevention in individuals with contraindications to oral anticoagulants. Transesophageal echocardiography (TOE) is the gold standard for LAAO guiding, but intracardiac echocardiography (ICE) appears to be a potential alternative. AIM: To compare the LAAO procedure guided by TOE or ICE with respect to procedural success and safety. MATERIAL AND METHODS: TOE-guided LAAO was performed in 12 patients and ICE-guided LAAO in 11 patients. ICE was performed using an 8F AcuNav probe and the ACUSON SC2000 system. For LAAO the Amplatzer Amulet was used. After 1 month TOE was performed. RESULTS: Procedural success was achieved in all patients in TOE and ICE groups. There was 1 complication (groin hematoma). The procedure time was significantly longer in the TOE group (43 to 80 min; median: 54 min) compared to the ICE group (28 to 67 min; median: 45 min), (p = 0.02) The time needed to puncture the interatrial septum and time needed to remove the sheath did not differ between groups. Fluoroscopic time was insignificantly longer in the ICE group (9.91 ±4.01s) compared to the TOE group (7.69 ±3.21s), and a significantly larger contrast media volume was used in the ICE group (30.00 ±6.67 ml vs. 40.45 ±23.18 ml, p = 0.03). There were no statistically significant differences in the results between TOE and ICE groups in follow-up assessments. CONCLUSIONS: LAAO using the Amplatzer Amulet may be successfully and safely guided by ICE. ICE offered shorter procedure time and similar results irrespectively of left atrial appendage anatomy compared to TOE guidance.

The first successful implantation of an intravenous AAIR pacemaker into autologous extracardiac lateral tunnel Fontan in the child.

Patients with a single ventricle have complex anatomy that requires staged palliation which is usually the Fontan procedure. This procedure has undergone a lot of modifications to improve hemodynamics. Despite these efforts, sinus node dysfunction (SND) and bradyarrythmias are still common complications after Fontan operation, therefore there is a need of pacemakers implantation. Unfortunately, the most frequent technique of creating Fontan cannale - the extracardiac lateral tunnel makes the transvenous access to the atrium difficult or impossible to achieve. We report a case of successful implantation of an endocardial atrial lead for SND in patient with an extracardiac autologous Fontan tunnel.