Recognition and perception of emotions in juvenile myoclonic epilepsy.

OBJECTIVE: Perception and recognition of emotions are fundamental prerequisites of human life. Patients with juvenile myoclonic epilepsy (JME) may have emotional and behavioral impairments that might influence socially desirable interactions. We aimed to investigate perception and recognition of emotions in patients with JME by means of neuropsychological tests and functional magnetic resonance imaging (fMRI). METHODS: Sixty-five patients with JME (median age = 27 years, interquartile range [IQR] = 23-34) were prospectively recruited at the Department of Neurology, Christian Doppler University Hospital, Paracelsus Medical University, Salzburg, Austria. Patients were compared to 68 healthy controls (median age = 24 years, IQR = 21-31), matched for sex, age, and education. All study participants underwent the Networks of Emotion Processing test battery (NEmo), an fMRI paradigm of "dynamic fearful faces," a structured interview for psychiatric and personality disorders, and comprehensive neuropsychological testing. RESULTS: JME patients versus healthy controls demonstrated significant deficits in emotion recognition in facial and verbal tasks of all emotions, especially fear. fMRI revealed decreased amygdala activation in JME patients as compared to healthy controls. Patients were at a higher risk of experiencing psychiatric disorders as compared to healthy controls. Cognitive evaluation revealed impaired attentional and executive functioning, namely psychomotor speed, tonic alertness, divided attention, mental flexibility, and inhibition of automated reactions. Duration of epilepsy correlated negatively with parallel prosodic and facial emotion recognition in NEmo. Deficits in emotion recognition were not associated with psychiatric comorbidities, impaired attention and executive functions, types of seizures, and treatment. SIGNIFICANCE: This prospective study demonstrated that as compared to healthy subjects, patients with JME had significant deficits in recognition and perception of emotions as shown by neuropsychological tests and fMRI. The results of this study may have importance for psychological/psychotherapeutic interventions in the management of patients with JME.

Electroanatomical mapping-guided left bundle branch area pacing in patients with structural heart disease and advanced conduction abnormalities.

AIMS: Left bundle branch area pacing (LBBAP) can be technically challenging and fluoroscopy-intense. Three-dimensional electroanatomical mapping (EAM) facilitates non-fluoroscopic lead navigation and electrogram mapping. We sought to prospectively evaluate the feasibility, safety, and outcomes of routine EAM-guided LBBAP in patients with structural heart disease (SHD) and advanced conduction abnormalities. METHODS AND RESULTS: Consecutive patients with SHD and conduction abnormalities who underwent an attempt at EAM-guided LBBAP were included. The feasibility, safety, procedural, and mid-term outcomes were evaluated. Electrical, echocardiographic, and clinical parameters were assessed at implantation and last follow-up. Thirty-two patients (68 ± 18 years; 19% female) were included, of which 75% had intrinsic QRS > 150 ms, 53% left bundle branch block, and 25% right bundle branch block. Primary EAM-guided LBBAP was successful in 29 patients (91%). The procedural duration was 95 (70-110) min, total fluoroscopy time 0.93 (0.40-1.73) min, and total fluoroscopy dose 35.4 (20.5-77.2) cGy cm2. Paced QRS duration (QRSd) was significantly shorter than intrinsic QRSd (121.9 ± 10.7 vs. 159.2 ± 34.4 ms; P < 0.001) and remained stable during the mean follow-up of 7.0 ± 5.9 months. The LBBAP capture threshold was 0.57 ± 0.23 V/0.4 ms at implantation and remained low during follow-up (0.58 ± 0.18 V/0.5 ± 0.2 ms; P = 0.877). Overall left ventricular ejection fraction improved significantly from 44.2 ± 14.3% at baseline to 49.4 ± 13.1% at follow-up (P = 0.009), New York Heart Association class from 2.4 ± 0.6 to 1.8 ± 0.6 (P = 0.002), respectively. No complications occurred that required intervention. CONCLUSION: Routine near-zero fluoroscopy EAM-guided LBBAP can safely be performed in patients with SHD and advanced conduction abnormalities with high success rates and favourable mid-term outcomes. Further studies are needed to investigate whether the use of EAM improves the overall outcome of conduction system pacing and to identify specific patient populations who benefit the most from EAM-guided lead implantation.

Emotional Word Processing in Patients With Juvenile Myoclonic Epilepsy.

Objective: According to Panksepp's hierarchical emotion model, emotion processing relies on three functionally and neuroanatomically distinct levels. These levels comprise subcortical networks (primary level), the limbic system (secondary level), and the neocortex (tertiary level) and are suggested to serve differential emotional processing. We aimed to validate and extend previous evidence of discrete and dimensional emotion processing in patient with juvenile myoclonic epilepsy (JME). Methods: We recorded brain activity of patients with JME and healthy controls in response to lexical decisions to words reflecting the discrete emotion fear and the affective dimension negativity previously suggested to rely on different brain regions and to reflect different levels of processing. In all study participants, we tested verbal cognitive functions, as well as the relationship of psychiatric conditions, seizure types and duration of epilepsy and emotional word processing. Results: In support of the hierarchical emotion model, we found an interaction of discrete emotion and affective dimensional processing in the right amygdala likely to reflect secondary level processing. Brain activity related to affective dimensional processing was found in the right inferior frontal gyrus and is suggested to reflect tertiary level processing. Psychiatric conditions, type of seizure nor mono- vs. polytherapy and duration of epilepsy within patients did not have any effect on the processing of emotional words. In addition, no differences in brain activity or response times between patients and controls were observed, despite neuropsychological testing revealed slightly decreased verbal intelligence, verbal fluency and reading speed in patients with JME. Significance: These results were interpreted to be in line with the hierarchical emotion model and to highlight the amygdala's role in processing biologically relevant stimuli, as well as to suggest a semantic foundation of affective dimensional processing in prefrontal cortex. A lack of differences in brain activity of patients with JME and healthy controls in response to the emotional content of words could point to unaffected implicit emotion processing in patients with JME.

A randomized trial of non-fasting vs. fasting for cardiac implantable electronic device procedures (Fast-CIED Study).

AIMS: Data on safety and efficacy of a non-fasting strategy in minimal invasive cardiac procedures are lacking. We assessed a non-fasting strategy compared with a fasting strategy regarding patient's well-being and safety in elective cardiac implantable electronic device (CIED) procedures. METHODS AND RESULTS: In this randomized, single-blinded clinical trial, 201 patients (non-fasting = 100, fasting = 101) with a mean age of 72.0 ± 11.6 years (66.7% male) were assigned to a non-fasting strategy (solids/fluids allowed up to 1 h) or a fasting strategy (at least 6 h no solids and 2 h no fluids) before the procedure and analysed on an intention-to-treat basis. The co-primary outcomes were patients' well-being scores (based on numeric rating scale, 0-10) and incidence of intra-procedural food-related adverse events, including vomiting, perioperative pulmonary aspiration, and emergency intubation. Renal, haematological, and metabolic blood parameters and 30-day follow-up data were gathered. The summed pre-procedural patients' well-being score was significantly lower in the non-fasting group [non-fasting: 13.1 ± 9.6 vs. fasting: 16.5 ± 11.4, 95% confidence interval (CI) of mean difference (MD) -6.35 to -0.46, P = 0.029], which was mainly driven by significantly lower scores for hunger and tiredness in the non-fasting group (non-fasting vs. fasting; hunger: 0.9 ± 1.9 vs. 3.1 ± 3.2, 95% CI of MD -2.86 to -1.42, P < 0.001; tiredness: 1.6 ± 2.3 vs. 2.6 ± 2.7, 95% CI of MD -1.68 to -0.29, P = 0.023). No intra-procedural food-related adverse events were observed. Relevant blood parameters and 30-day follow-up did not show significant differences. CONCLUSION: These results showed that a non-fasting strategy is beneficial to a fasting one regarding patient's well-being and comparable in terms of safety for CIED procedures (NCT04389697).

Impact of electroanatomical mapping-guided lead implantation on procedural outcome of His bundle pacing.

AIMS: Conventional His bundle pacing (HBP) can be technically challenging and fluoroscopy-intense, particularly in patients with His-Purkinje conduction disease (HPCD). Three-dimensional electroanatomical mapping (EAM) facilitates non-fluoroscopic lead navigation and HB electrogram mapping. We sought to assess the procedural outcome of routine EAM-guided HBP compared with conventional HBP in a real-world population and evaluate the feasibility and safety of EAM-guided HBP in patients with HPCD. METHODS AND RESULTS: We included 58 consecutive patients (72 ± 13 years; 71% male) who underwent an attempt to conventional (EAM- group; n = 29) or EAM-guided (EAM+ group; n = 29) HBP between June 2019 and April 2020. The centre's learning curve was initially determined (n = 40 cases) to define the conventional control group and minimize outcome bias favouring EAM-guided HBP. His bundle pacing was successful in 26 patients (90%) in the EAM+ and 27 patients (93%) in the EAM- group (P = 0.64). The procedure time was 90 (73-135) and 110 (70-130) min, respectively (P = 0.89). The total fluoroscopy time [0.7 (0.5-1.4) vs. 3.3 (1.4-6.5) min; P < 0.001] and fluoroscopy dose [21.9 (9.1-47.7) vs. 78.6 (27.2-144.9) cGycm2; P = 0.001] were significantly lower in the EAM+ than EAM- group. There were no significant differences between groups in His capture threshold (1.2 ± 0.6 vs. 1.4 ± 1.0 V/1.0 ms; P = 0.33) and paced QRS duration (113 ± 15 vs. 113 ± 17 ms; P = 0.89). In patients with HPCD, paced QRS duration was similar in both groups (121 ± 15 vs. 123 ± 12 ms; P = 0.77). The bundle branch-block recruitment threshold tended to be lower in the EAM+ than EAM- group (1.3 ± 0.7 vs. 1.8 ± 1.2 V/1.0 ms; P = 0.31). No immediate procedure-related complications occurred. One patient (2%) experienced lead dislodgement during 4-week follow-up. CONCLUSION: Implementation of routine EAM-guided HBP lead implantation is feasible and safe in a real-world cohort of patients with and without HPCD and results in a tremendous reduction in radiation exposure without prolonging procedure time or increasing procedure-related complications.

Predictors of rhythm outcomes after cardiac resynchronization therapy in atrial fibrillation patients: When should we use an atrial lead?

BACKGROUND: Cardiac resynchronization therapy (CRT) is widely used in atrial fibrillation (AF) patients and could impact rhythm stability. HYPOTHESIS: We aimed to identify predictors of sinus rhythm (SR) stability or AF progression in a real-word cohort of CRT-AF patients. METHODS: From 330 consecutive implantable cardioverter-defibrillator implantations due to ischemic or dilated cardiomyopathy, 65 (20%) patients with AF history (paroxysmal, n = 32) underwent a CRT implantation with an atrial electrode and were regularly followed every 4-6 months. Rhythm restoration was attempted for most AF patients based on symptoms, biventricular pacing (BP), and lack of thrombi. RESULTS: After 33 months, 18 (28%) patients progressed to permanent mode switch (MS≥99%) and 20 (31%) patients had stable SR (MS < 1%). Logistic regression showed that history of persistent AF (OR: 8.01, 95%CI: 2.0-31.7, p = .003) is associated with higher risk of permanent MS. In persistent AF patients, a bigger left atrium (OR: 1.2 per mm, 95%CI: 1.03-1.4, p = .025) and older age (OR: 1.15 per life-year, 95%CI: 1.01-1.3, p = .032) were predictors of future permanent MS. Paroxysmal AF at implantation (OR: 5.96, 95%CI: 1.6-21.9, p = .007) and increased BP (OR: 1.4 per 1%, 95%CI: 1.05-1.89, p = .02) were associated with stable SR. In persistent AF patients, stable SR correlated with higher BP (98 ± 2 vs. 92 ± 8%, p < .001). CONCLUSION: In patients with AF undergoing CRT implantation, persistent AF, LA dilatation and advanced age relate to future permanent MS (AF), whereas high BP promotes SR stability. These findings could facilitate the management of CRT-AF patients and guide therapy in order to maximize its effect on rhythm.

Transvenous revision of leads with cardiac perforation following device implantation-Safety, outcome, and complications.

INTRODUCTION: Cardiac perforation is a rare complication of cardiac implantable electronic device (CIED) implantation. Transvenous revision of perforated leads is associated with the risk of cardiac tamponade and death. Little is known about periprocedural complications and outcome of these patients. METHODS AND RESULTS: All patients referred to our department with evidence or suspicion of cardiac perforation following CIED implantation underwent chest X-ray, transthoracic echocardiography, device interrogation, and, if necessary, a cardiac computed tomography (CT)-scan to diagnose lead perforation and associated complications. Transvenous lead revision (TLR) was performed in all patients with evidence of lead perforation. Patient characteristics, procedural complications, and outcome were recorded and analyzed. Fifty-six patients (75 ± 10 years, 43% male) were diagnosed with cardiac perforation, 34 patients (61%) early within 30 days post-implantation, and 22 patients (39%) thereafter. The most frequent perforation site was the right ventricular (RV) apex (75%), followed by the RV free wall (16%) and the right atrial appendage (9%). A total of 16 patients (29%) presented with severe complications; 12 patients (21%) with pericardial effusion treated by pericardiocentesis before lead revision and four patients (7%) with hematothorax requiring drainage. Late perforations showed significantly more frequent cardiac tamponades (P = .041). TLR was performed without further complications in 54 patients (96%). None of the patients required surgical treatment or experienced in-hospital death. CONCLUSIONS: Cardiac perforation following CIED implantation is associated with severe complications in nearly one-third of the cases. Transvenous revision of the perforated lead can safely be performed with a very low complication rate.

Extraction of infected cardiac implantable electronic devices and the need for subsequent re-implantation.

BACKGROUND: Little is known about rates of re-implantation and outcomes of patients not implanted with a device after transvenous lead extraction (TLE) in cardiac device related infections (CDRI). METHODS: All patients with CDRI were included in a prospective registry. After TLE, the indication for re-implantation was evaluated according to the patients' history and most recent cardiac examinations. All patients were followed for complications and mortality. In addition, in patients discharged without device the frequency of device implantations was analyzed. RESULTS: Among 302 patients, only 123 (40.7%) met the indication for implantation of the same cardiac implantable electronic device (CIED), 68 (22.5%) received a different device and 111 (36.8%) patients were discharged without CIED. Reimplanted patients were younger (70 ±â€¯11 vs. 73 ±â€¯13 years; p = 0.004), more often male (83 vs. 69%, p = 0.006), had less systemic infection (38 vs. 60%; p < 0.001) and a higher prevalence of complete heart block (28 vs. 7%, p < 0.001). Reasons against re-implantation were: loss of indication (45%), never met indication (27%), patients' preference (17%), persistent infection (8%) and advanced age (3%). During 26 ±â€¯18 months of follow-up, mortality in both groups was similar after adjusting for cofactors (HR 0.79; 95% CI 0.49-1.29; p = 0.352). CONCLUSION: More than one third of patients undergoing TLE for CDRI in our study are not implanted with a new device. Careful evaluation of the initial CIED indication allows for detection of over treated patients and may avoid unnecessary device-related complications.

Outcome in patients undergoing upgrade to cardiac resynchronization therapy: predictors of outcome after upgrade to CRT.

The advantages of upgrade to cardiac resynchronisation therapy (CRT) have not been explored as carefully as the outcomes of de novo CRT implantations. Furthermore selection criteria for patients with the potential to benefit the most from this therapy are unknown. Therefore, we analyzed the long term outcome and its predictors in a real-world cohort receiving a CRT upgrade from previous pacemaker (PM) and defibrillator devices (ICD). We analyzed 86 patients (mean age 68 ± 9 years; 89% male) undergoing CRT upgrade procedures. Response to CRT as well as long term patient outcome was analyzed. NYHA class improved in majority of the patients during short term period (61%), and this trend remained constant during long term follow-up (54%). The observed all-cause mortality was 54% with mean survival of 49 ± 4 months. 11 patients underwent left ventricular assist device implantation or heart transplantation. In the multivariate analysis, only kidney function assessed by GFR (HR 0.97; 95% CI 0.95-0.99; p = 0.009) and LVEF (HR 0.92; 95% CI 0.87-0.97; p = 0.002) remain predictors for mortality. Patients who undergo an upgrade procedure to CRT demonstrate a significant response rate assessed by improvement in NYHA class, with initial baseline parameters such as LVEF and kidney function remaining significant predictors for mortality.

Deep sedation for transvenous lead extraction: a large single-centre experience.

AIMS: Transvenous lead extraction for cardiac implantable electronic devices (CIED) is of growing importance. Nevertheless, the optimal anaesthetic approach, general anaesthesia vs. deep sedation (DS), remains unresolved. We describe our tertiary centre experience of the feasibility and safety of DS. METHODS AND RESULTS: Extraction procedures were performed in the electrophysiology (EP) laboratory by two experienced electrophysiologists. We used intravenous Fentanyl, Midazolam, and Propofol for DS. A stepwise approach with locking stylets, dilator sheaths, and mechanical sheaths via subclavian, femoral, or internal jugular venous access was utilized. Patient characteristics and procedural data were collected. Logistic regression models were used to identify parameters associated with sedation-related complications. Extraction of 476 leads (dwelling time/patient 88 ± 49 months, 30% ICD leads) was performed in 220 patients (64 ± 17 years, 80% male). Deep sedation was initiated with bolus administration of Fentanyl, Midazolam, and Propofol; mean doses 0.34 ± 0.12 µg/kg, 24.3 ± 6.8 µg/kg, and 0.26 ± 0.13 mg/kg, respectively. Deep sedation was maintained with continuous Propofol infusion (initial dose 3.7 ± 1.1 mg/kg/h; subsequently increased to 4.7 ± 1.2 mg/kg/h with 3.9 ± 2.6 adjustments) and boluses of Midazolam and Fentanyl as indicated. Sedation-related episodes of hypotension, requiring vasopressors, and hypoxia, requiring additional airway management, occurred in 25 (11.4%) and 5 (2.3%) patients, respectively. These were managed without adverse consequences. Five patients (2.3%) experienced major intraprocedural complications; there were no procedure-related deaths. All of our logistic regression models indicated intraprocedural support was associated with administration higher Fentanyl doses. CONCLUSION: Transvenous lead extraction under DS in the EP laboratory is a safe procedure with high success rates when performed by experienced staff.

Implanted defibrillators in trouble: initial device misclassification leads to correct diagnosis-a case series.

AIMS: Supraventricular tachycardia, mistakenly diagnosed as ventricular tachycardia, may lead to inappropriate ICD therapy. Inappropriate ICD therapy associates with increased morbidity and mortality. A reduction of inappropriate therapy is therefore desired. METHODS AND RESULTS: We present three different cases that beautifully illustrate the diagnostic possibilities of stored electrograms. Initial differential diagnoses in all three patients included ventricular tachycardia, focal atrial tachycardia, atrio-ventricular re-entry tachycardia, and atrio-ventricular nodal re-entry tachycardia. The first case was diagnosed as atrio-ventricular nodal re-entry tachycardia. In the second case, atrio-ventricular re-entry tachycardia was revealed. Ectopic atrial tachycardia was the reason for inappropriate therapy in the third patient. CONCLUSION: Thorough analysis of stored electrograms yielded correct diagnosis in all cases, which subsequently was confirmed by electrophysiological study.

Sensor-based electromagnetic navigation to facilitate implantation of left ventricular leads in cardiac resynchronization therapy.

INTRODUCTION: Implantation of cardiac resynchronization therapy (CRT) devices can be challenging, time consuming, and fluoroscopy intense. To facilitate placement of left ventricular (LV) leads, a novel electromagnetic navigation system (MediGuide™, St. Jude Medical, St. Paul, MN, USA) has been developed, displaying real-time 3-D location of sensor-embedded delivery tools superimposed on prerecorded X-ray cine-loops of coronary sinus venograms. We report our experience and advanced progress in the use of this new electromagnetic tracking system to guide LV lead implantation. METHODS AND RESULTS: Between January 2012 and December 2013, 71 consecutive patients (69 ± 9 years, 76% male) were implanted with a CRT device using the new electromagnetic tracking system. Demographics, procedural data, and periprocedural adverse events were gathered. The impact of the operator's experience, optimized workflow, and improved software technology on procedural data were analyzed. LV lead implantation was successfully achieved in all patients without severe adverse events. Total procedure time measured 87 ± 37 minutes and the median total fluoroscopy time (skin-to-skin) was 4.9 (2.5-7.8) minutes with a median dose-area-product of 476 (260-1056) cGy*cm(2) . An additional comparison with conventional CRT device implantations showed a significant reduction in fluoroscopy time from 8.0 (5.8; 11.5) to 4.5 (2.8; 7.3) minutes (P = 0.016) and radiation dose from 603 (330; 969) to 338 (176; 680) cGy*cm(2) , respectively (P = 0.044 ). CONCLUSION: Use of the new navigation system enables safe and successful LV lead placement with improved orientation and significantly reduced radiation exposure during CRT implantation.

Results of catheter ablation of atrial fibrillation in hypertrophied hearts - Comparison between primary and secondary hypertrophy.

BACKGROUND AND PURPOSE: Approximately 20-25% of the patients with hypertrophic cardiomyopathy (HCM) develop atrial fibrillation (AF) during the clinical course of the disease, a percentage significantly larger than that of the general population. The purpose of the present study was to report on the procedural results of patients with AF and either primary or secondary left ventricular hypertrophy (LVH). METHODS AND SUBJECTS: Twenty-two consecutive HCM patients (55% male, mean age 57±8 years) with symptomatic AF, having undergone AF ablation procedures between September 2009 and July 2012 were compared with respect to procedural outcome and follow-up characteristics with 22 matched controls with secondary cardiac hypertrophy (64% male, 63±10 years) from our prospective AF catheter ablation registry. RESULTS AND CONCLUSION: Radiofrequency catheter ablation (RFCA) was successful in restoring long-term sinus rhythm in patients with LVH due to HCM and due to secondary etiology. However, patients with HCM needed more RFCA procedures and frequently additional antiarrhythmic drug therapy in order to maintain sinus rhythm.