Therapy duration and improvement of ventricular function in de novo heart failure: the Heart Failure Optimization study.

BACKGROUND AND AIMS: In patients with de novo heart failure with reduced ejection fraction (HFrEF), improvement of left ventricular ejection fraction (LVEF) is expected to occur when started on guideline-recommended medical therapy. However, improvement may not be completed within 90 days. METHODS: Patients with HFrEF and LVEF ≤ 35% prescribed a wearable cardioverter-defibrillator between 2017 and 2022 from 68 sites were enrolled, starting with a registry phase for 3 months and followed by a study phase up to 1 year. The primary endpoints were LVEF improvement > 35% between Days 90 and 180 following guideline-recommended medical therapy initiation and the percentage of target dose reached at Days 90 and 180. RESULTS: A total of 598 patients with de novo HFrEF [59 years (interquartile range 51-68), 27% female] entered the study phase. During the first 180 days, a significant increase in dosage of beta-blockers, renin-angiotensin system inhibitors, and mineralocorticoid receptor antagonists was observed (P < .001). At Day 90, 46% [95% confidence interval (CI) 41%-50%] of study phase patients had LVEF improvement > 35%; 46% (95% CI 40%-52%) of those with persistently low LVEF at Day 90 had LVEF improvement > 35% by Day 180, increasing the total rate of improvement > 35% to 68% (95% CI 63%-72%). In 392 patients followed for 360 days, improvement > 35% was observed in 77% (95% CI 72%-81%) of the patients. Until Day 90, sustained ventricular tachyarrhythmias were observed in 24 wearable cardioverter-defibrillator carriers (1.8%). After 90 days, no sustained ventricular tachyarrhythmia occurred in wearable cardioverter-defibrillator carriers. CONCLUSIONS: Continuous optimization of guideline-recommended medical therapy for at least 180 days in HFrEF is associated with additional LVEF improvement > 35%, allowing for better decision-making regarding preventive implantable cardioverter-defibrillator therapy.

Utilization of 3D mapping systems in interventional electrophysiology and its impact on procedure time and fluoroscopy-Insights from the "Go for Zero Fluoroscopy" project.

AIM: The implementation of 3D mapping systems plays an important role in interventional electrophysiology (EP) in recent years. The aim of the present study was to evaluate use of 3D mapping systems regarding fluoroscopy and procedure duration. METHOD: In the "Go for Zero Fluoroscopy" project 25 European centers provided data of consecutive EP procedures. Data on use of 3D mapping systems as well as utilization of contact force catheters and multipolar mapping catheters were associated with fluoroscopy time, dose area product (DAP), and procedure duration. RESULT: A 3D mapping system was used in 966 (54%) cases. Use of 3D mapping for atrioventricular nodal reentry tachycardia (AVNRT) was associated with reduced fluoroscopy time (p < 0.001), DAP (p = 0.04) but increased procedure time (p = 0.029). Moreover, fluoroscopy time (p < 0.001) and DAP (p = 0.005) were significantly lower in the 3D mapping group in ablation of typical atrial flutter. However, the procedure time (p < 0.001) increased. Use of 3D mapping in the ablation of accessory pathway (AP) was associated with reduced fluoroscopy time (p < 0.001) and DAP (p < 0.001) with no significant increase in procedure time (p = 0.066). In the case of atrial fibrillation, a 3D mapping system was used in 485 patients (75.8%). Additional use of a contact force catheter was associated with lower fluoroscopy time (p < 0.001) and DAP (p < 0.001). Use of a multipolar mapping catheter was associated with lower fluoroscopy time (p = 0.002). The implementation of 3D mapping systems in the ablation of ventricular tachycardias resulted in a significant increase in the procedure time (p = 0.001) without significant differences regarding the DAP (p = 0.773) and fluoroscopy time (p = 0.249). CONCLUSION: Use of 3D mapping systems in ablation of supraventricular tachycardias is associated with lower radiation exposure. Nevertheless, the procedure time often increases, except in the case of ablation for AP. Use of contact force catheters and multipolar mapping catheters is associated with yet lower radiation exposure values. Prospective randomized studies are needed to further elucidate potential benefit of these technological tools.

Delayed Improvement of Left Ventricular Function in Newly Diagnosed Heart Failure Depends on Etiology-A PROLONG-II Substudy.

In patients with newly diagnosed heart failure with reduced ejection fraction (HFrEF), three months of optimal therapy are recommended before considering a primary preventive implantable cardioverter-defibrillator (ICD). It is unclear which patients benefit from a prolonged waiting period under protection of the wearable cardioverter-defibrillator (WCD) to avoid unnecessary ICD implantations. This study included all patients receiving a WCD for newly diagnosed HFrEF (n = 353) at our center between 2012 and 2017. Median follow-up was 2.7 years. From baseline until three months, LVEF improved in patients with all peripartum cardiomyopathy (PPCM), myocarditis, dilated cardiomyopathy (DCM), or ischemic cardiomyopathy (ICM). Beyond this time, LVEF improved in PPCM and DCM only (10 ± 8% and 10 ± 12%, respectively), whereas patients with ICM showed no further improvement. The patients with newly diagnosed HFrEF were compared to 29 patients with a distinct WCD indication, which is an explantation of an infected ICD. This latter group had a higher incidence of WCD shocks and poorer overall survival. All-cause mortality should be considered when deciding on WCD prescription. In patients with newly diagnosed HFrEF, the potential for delayed LVEF recovery should be considered when timing ICD implantation, especially in patients with PPCM and DCM.

Leadless pacemakers in critically ill patients requiring prolonged cardiac pacing: A multicenter international study.

BACKGROUND: Temporary transvenous pacing in critically ill patients requiring prolonged cardiac pacing is associated with a high risk of complications. We sought to evaluate the safety and efficacy of self-contained intracardiac leadless pacemaker (LPM) implantation in this population. METHODS AND RESULTS: Consecutive patients implanted with a Micra LPM during the hospitalization in an intensive care unit were retrospectively included. Inclusion criteria were: more than or equal to 1 supracaval central venous line, or a ventilation tube, or intravenous antibiotic therapy for ongoing sepsis or bacteremia. Patients with a history of the previous implantation of a pacemaker were excluded. Out of 1016 patients implanted with an LPM, 99 met the inclusion criteria. Mean age was 75 years and Charlson comorbidity index 7. LPM implantation was successfully performed in 98% of cases, with a perioperative complication rate of 5%, mainly cardiac injuries. In-hospital mortality rate was 6%. No late (>30 days) device-related complication occurred, especially no infection. CONCLUSIONS: LPM appears as an acceptable alternative to conventional temporary transvenous pacing in selected critically ill patients requiring prolonged cardiac pacing, especially regarding the risk of infection.

A Novel SCN5A Variant Causes Temperature-Sensitive Loss Of Function in a Family with Symptomatic Brugada Syndrome, Cardiac Conduction Disease, and Sick Sinus Syndrome.

INTRODUCTION: Brugada syndrome (BrS) is an inherited arrhythmia syndrome associated with an increased risk of sudden cardiac death. SCN5A is the most important disease-modifying gene for BrS, but many SCN5A variants have not been functionally characterized. Furthermore, the temperature dependency of SCN5A is only rarely explored in in vitro analyses. METHODS: The clinical phenotype of the affected family was assessed by medical history, ECGs and ajmaline challenge. Whole-cell patch clamp recordings were performed on HEK 293T cells expressing Nav1.5-G1712S, a novel SCN5A variant found in the symptomatic family. RESULTS: Three male family members had experienced sudden cardiac death, sudden cardiac arrest, and rhythmogenic syncopes. Beside a positive ajmaline challenge with demarcation of a Brugada type 1 ECG, 1 patient also showed evidence of symptomatic cardiac conduction disease and sick sinus syndrome (SSS). In patch clamp analyses, Nav1.5-G1712S generated reduced peak currents as compared to the wild type. At body temperature, Nav1.5-G1712S additionally exhibited an enhanced slow inactivation and an impaired recovery from inactivation. CONCLUSION: We conclude that G1712S is a pathogenic SCN5A loss-of function mutation at physiological temperature associated with an overlapping presentation of BrS, SSS, and cardiac conduction disease.

Increased Quality of Life Among Newly Diagnosed Patients With Heart Failure With Reduced Ejection Fraction in the Months After Initiation of Guideline-Directed Medical Therapy and Wearable Cardioverter Defibrillator Prescription.

BACKGROUND: The patient experience of heart failure involves a multi-impact symptom response with functional limitations, psychological changes, and significant treatment burden. OBJECTIVE: The aim of this study was to examine the change in patient-reported outcomes in newly diagnosed patients with heart failure and reduced ejection fraction (HFrEF) prescribed a wearable cardioverter defibrillator. METHODS: Adults hospitalized for new-onset heart failure, due to ischemic or nonischemic cardiomyopathy, and prescribed a wearable cardioverter defibrillator within 10 days post discharge were approached for inclusion. Participants completed the Kansas City Cardiomyopathy Questionnaire at 3 time points: baseline, day 90, and day 180. RESULTS: A total of 210 patients (26% female) were included. All Kansas City Cardiomyopathy Questionnaire subscales (physical limitation, symptom frequency, quality of life, and social limitation) showed improvement from baseline to day 90 (all Ps < .001). Only quality of life continued to improve from day 90 to day 180 (P < .001). By day 90, nearly 70% of patients showed an improvement in quality of life (67.9%, n = 91), and by day 180, more than 80% (82.8%, n = 111) reported a net improvement. Five patients (3.7%) reported a net decrease, and 18 patients (13.4%) had no net change in quality of life during the 180-day period. CONCLUSION: Patient-reported quality of life improved significantly among patients newly diagnosed with HFrEF and prescribed a wearable cardioverter defibrillator. These results suggest that pursuing guideline-directed medical therapy for HFrEF, while being protected by the wearable cardioverter defibrillator, is likely to provide symptom relief and improve quality of life.

Feasibility and First Results of Heart Failure Monitoring Using the Wearable Cardioverter-Defibrillator in Newly Diagnosed Heart Failure with Reduced Ejection Fraction.

The wearable cardioverter-defibrillator (WCD) is used in patients with newly diagnosed heart failure and reduced ejection fraction (HFrEF). In addition to arrhythmic events, the WCD provides near-continuous telemetric heart failure monitoring. The purpose of this study was to evaluate the clinical relevance of additionally recorded parameters, such as heart rate or step count. We included patients with newly diagnosed HFrEF prescribed with a WCD. Via the WCD, step count and heart rate were acquired, and an approximate for heart rate variability (HRV5) was calculated. Multivariate analysis was performed to analyze predictors for an improvement in left ventricular ejection fraction (LVEF). Two hundred and seventy-six patients (31.9% female) were included. Mean LVEF was 25.3 ± 8.5%. Between the first and last seven days of usage, median heart rate fell significantly (p < 0.001), while median step count and HRV5 significantly increased (p < 0.001). In a multivariate analysis, a delta of HRV5 > 23 ms was an independent predictor for LVEF improvement of ≥10% between prescription and 3-month follow-up. Patients with newly diagnosed HFrEF showed significant changes in heart rate, step count, and HRV5 between the beginning and end of WCD prescription time. HRV5 was an independent predictor for LVEF improvement and could serve as an early indicator of treatment response.

Smart Wearables for Cardiac Monitoring-Real-World Use beyond Atrial Fibrillation.

The possibilities and implementation of wearable cardiac monitoring beyond atrial fibrillation are increasing continuously. This review focuses on the real-world use and evolution of these devices for other arrhythmias, cardiovascular diseases and some of their risk factors beyond atrial fibrillation. The management of nonatrial fibrillation arrhythmias represents a broad field of wearable technologies in cardiology using Holter, event recorder, electrocardiogram (ECG) patches, wristbands and textiles. Implementation in other patient cohorts, such as ST-elevation myocardial infarction (STEMI), heart failure or sleep apnea, is feasible and expanding. In addition to appropriate accuracy, clinical studies must address the validation of clinical pathways including the appropriate device and clinical decisions resulting from the surrogate assessed.

A novel open-source software-based high-precision workflow for target definition in cardiac radioablation.

INTRODUCTION: Noninvasive ablative radiotherapy of cardiac arrhythmias (stereotactic ablative body radiation) has shown promising initial results. Precise targeting of the arrhythmogenic substrate is paramount to limit adverse effects to healthy myocardium, organs at risk, and cardiac implantable electronic devices. Using electroanatomic maps for treatment planning is technically challenging. METHODS AND RESULTS: Using the free open-source 3D Slicer software platform we established a workflow for high-precision target definition based on electroanatomic maps. An import plug-in for 3D Slicer has been designed that reads electroanatomic maps generated with three mapping systems in widespread clinical use. Using our proposed workflow in a real-world patient case we were able to align the map to the computed tomography (CT) with a mean distance of 3.1 mm. Thus, points defined on the map were translated into CT space with high accuracy and a radiotherapy treatment volume was defined in CT space based on these map-derived points. CONCLUSION: We describe a novel high-precision target definition method for stereotactic ablation of cardiac arrhythmias. Multimodal integration of the electroanatomic map with the planning CT allows for highly accurate localization of previously identified electrophysiological features in CT space. It remains to be shown whether this novel planning workflow leads to superior ablation outcomes when compared with other approaches.

A novel screening tool to unmask potential interference between S-ICD and left ventricular assist device.

INTRODUCTION: In patients with a left ventricular assist device (LVAD), the subcutaneous implantable cardioverter-defibrillator (S-ICD) can be an alternative to transvenous ICD systems due to reduced risk of systemic infection, which could lead to extraction of the ICD as well as the LVAD. S-ICD eligibility is lower in patients with LVAD than in patients with end-stage heart failure without LVAD. Several reports have shown inappropriate S-ICD therapy in the coexistence of LVAD and S-ICD. The aim of the present study was to evaluate S-ICD eligibility in patients with LVAD using the established electrocardiogram (ECG)-based screening test as well as a novel device-based screening test to identify potentially inappropriate S-ICD sensing in this specific patient cohort. METHODS AND RESULTS: The present study included 115 patients implanted with an LVAD. The standard ECG-based screening test and a novel device-based screening test were performed in all patients. Eighty patients (70%) were eligible for S-ICD therapy with the standard ECG-based screening test. Performance of the novel device-based screening test identified device-device interference in 14 of these 80 patients (12%). CONCLUSION: Using a novel extended device-based S-ICD screening method, a small number of patients with LVAD deemed eligible for S-ICD with the standard ECG-based screening test exhibit device-device interference. Careful S-ICD screening should be performed in patients with LVAD, who are candidates for S-ICD therapy, to prevent inappropriate sensing or ICD therapy.

One-Year Course of Periprocedural Anticoagulation in Atrial Fibrillation Ablation: Results of a German Nationwide Survey.

INTRODUCTION: Periprocedural oral anticoagulation (OAC) strategies for atrial fibrillation (AF) ablation procedures are changing rapidly. OBJECTIVE: To assess the management and course of periprocedural OAC for AF ablation procedures in experienced electrophysiology (EP) centers in Germany over the last 12 months. METHODS: The data are based on an electronic questionnaire, which was sent to 35 experienced EP centers in September 2018 and then exactly 1 year later. Participants provided information on their periprocedural OAC management, the handling with dual therapy (OAC plus single antiplatelet therapy), the availability of specific antidotes, the transseptal puncture approach, and noteworthy complications. RESULTS: Responses were received from all 35 centers and represent 10,010 AF ablation procedures annually. In 2018, the administration of vitamin K antagonist (VKA) was continued throughout the procedure at all centers (100%). In contrast, the majority of centers used minimally interrupted periprocedural non-vitamin K antagonist oral anticoagulants (NOAC) (54.3%), 13 centers (37.2%) completely interrupted NOAC, and only 3 centers (8.5%) continued NOAC throughout the procedure. At the 1-year follow-up survey, 32 centers were found to have continued their previous strategy of periprocedural OAC and 3 changed from a minimally interrupted to a continued NOAC strategy. Of note, 30 centers (85.7%) performed transseptal puncture fluoroscopically without additional cardiac imaging. In the setting of uninterrupted periprocedural OAC management, no relevant complications were noted. CONCLUSION: Our survey shows marked heterogeneous periprocedural OAC management at experienced EP centers in Germany. Whereas continuation of VKA has already been integrated into clinical practice, the majority of centers still use a minimally interrupted NOAC strategy.

Autophagy alleviates amiodarone-induced hepatotoxicity.

Amiodarone is a widely used antiarrhythmic drug that can cause the development of steatohepatitis as well as liver fibrosis and cirrhosis. The molecular mechanisms of amiodarone-mediated liver injury remain largely unknown. We therefore analyzed amiodarone-mediated hepatocellular injury in patients with chronic heart failure, in primary hepatocytes and HepG2 cells. We found that amiodarone-treated patients with chronic heart failure revealed significantly higher serum levels of caspase-cleaved keratin-18, an apoptosis biomarker, compared to healthy individuals or patients not receiving amiodarone. Furthermore, amiodarone treatment of hepatocytes resulted in apoptosis associated with lipid accumulation and ER-stress induction. Liver cell steatosis was accompanied by enhanced de novo lipogenesis which, after reaching peak levels, declined together with decreased activation of ER stress. The decline of amiodarone-mediated lipotoxicity was associated with protective autophagy induction. In contrast, in hepatocytes treated with the autophagy inhibitor chloroquine as well as in autophagy gene (ATG5 or ATG7)-deficient hepatocytes, amiodarone-triggered toxicity was increased. In conclusion, we demonstrate that amiodarone induces lipid accumulation associated with ER stress and apoptosis in hepatocytes, which is mirrored by increased keratin-18 fragment serum levels in amiodarone-treated patients. Autophagy reduces amiodarone-mediated lipotoxicity and could provide a therapeutic strategy for protection from drug-induced liver injury.

Time-to-first appropriate shock in patients implanted prophylactically with an implantable cardioverter-defibrillator: data from the Survey on Arrhythmic Events in BRUgada Syndrome (SABRUS).

AIMS: Data on predictors of time-to-first appropriate implantable cardioverter-defibrillator (ICD) therapy in patients with Brugada Syndrome (BrS) and prophylactically implanted ICD's are scarce. METHODS AND RESULTS: SABRUS (Survey on Arrhythmic Events in BRUgada Syndrome) is an international survey on 678 BrS patients who experienced arrhythmic event (AE) including 252 patients in whom AE occurred after prophylactic ICD implantation. Analysis was performed on time-to-first appropriate ICD discharge regarding patients' characteristics. Multivariate logistic regression models were utilized to identify which parameters predicted time to arrhythmia ≤5 years. The median time-to-first appropriate ICD therapy was 24.8 ± 2.8 months. A shorter time was observed in patients from Asian ethnicity (P < 0.05), those with syncope (P = 0.001), and those with Class IIa indication for ICD (P = 0.001). A longer time was associated with a positive family history of sudden cardiac death (P < 0.05). Multivariate Cox regression revealed shorter time-to-ICD therapy in patients with syncope [odds ratio (OR) 1.65, P = 0.001]. In 193 patients (76.6%), therapy was delivered during the first 5 years. Factors associated with this time were syncope (OR 0.36, P = 0.001), spontaneous Type 1 Brugada electrocardiogram (ECG) (OR 0.5, P < 0.05), and Class IIa indication (OR 0.38, P < 0.01) as opposed to Class IIb (OR 2.41, P < 0.01). A near-significant trend for female gender was also noted (OR 0.13, P = 0.052). Two score models for prediction of <5 years to shock were built. CONCLUSION: First appropriate therapy in BrS patients with prophylactic ICD's occurred during the first 5 years in 76.6% of patients. Syncope and spontaneous Type 1 Brugada ECG correlated with a shorter time to ICD therapy.

When two hearts do not beat as one - An unusual cause of pacemaker related tachycardia.

We describe an unusual cause of intermittent rapid ventricular paced rhythm in a patient implanted with a dual chamber pacemaker due to sinus node dysfunction after heart transplantation. During implantation of the pacemaker lead measurements were reported normal, atrial sensing was not documented because of sinus arrest. After implantation the patient complained about intermittent palpitations. Via pacemaker interrogation we could demonstrate electrical isolation of the atrial lead, which was implanted in the donor's atrial myocardium. This led to intermittent pacemaker related tachycardia and AV-dissociation. This case report highlights the difficulty of atrial lead placement in heart transplanted patients using the biatrial surgical technique.

Electrocardiographic changes after implantation of a left ventricular assist device - Potential implications for subcutaneous defibrillator therapy.

BACKGROUND: Implantation of a left ventricular assist device (LVAD) leads to a diverse spectrum of changes on the twelve-lead surface electrocardiogram (ECG). We aimed to elucidate the changes of the surface ECG in patients after LVAD implantation potentially impacting ECG based screening tests of subcutaneous implantable cardioverter-defibrillators (S-ICD). METHODS: Patients from 2005 until 2017 with a documented twelve-lead ECG before and after LVAD implantation were included. Baseline parameters were obtained through hospital records. The twelve-lead ECGs registered before and after LVAD implantation were analyzed. RESULTS: From 415 patients undergoing an LVAD implantation, complete datasets were available for 253 patients. 216 patients (85%) were male. Mean age at time of LVAD implantation was 54.7 ±â€¯12.4 years. The underlying etiology was ischemic cardiomyopathy in 119 (47%), dilated cardiomyopathy in 112 (44%), myocarditis in 8 (3%) and other in 14 (6%). We observed a reduction in the amplitude of the R wave in lead I (p < 0.0001), lead II (p < 0.0001), lead III (p < 0.004), lead aVL (p < 0.001) and lead aVF (p < 0.0001) as well as of the S wave in lead III (p < 0.001) and lead aVR (p < 0.0001) after LVAD implantation. We also noticed a reduction of the R:T ratio in lead I (p < 0.0001) as well as in lead II (p = 0.100) and lead aVF (p = 0.292) although statistically non-significant. CONCLUSION: LVAD implantation leads to significant alterations of the surface ECG, especially the R:T ratio in leads I, II and aVF. These leads correlate with the vectors of the ECG based S-ICD screening test. Thus, these ECG changes may impact the continuous eligibility for subcutaneous ICD therapy in patients after LVAD implantation.

Role of the Wearable Defibrillator in Newly Diagnosed Heart Failure.

PURPOSE OF REVIEW: The wearable defibrillator (WCD) was shown to be safe and effective in detecting and terminating ventricular tachyarrhythmias and therefore allows temporary protection from sudden cardiac death. This review gives an overview of the current data on WCD in newly diagnosed cardiomyopathy. RECENT FINDINGS: Patients with newly diagnosed heart failure and reduced LVEF appear to have an increased risk of ventricular tachyarrhythmias, which may decrease over time when heart failure medication is optimized and left ventricular function improves. This was shown to apply for patients with ischemic and non-ischemic cardiomyopathy, including peripartum cardiomyopathy. Prolongation of the WCD period may support to further optimization of heart failure medication, by protecting the patient from sudden cardiac death during this time and to avoid untimely ICD implantation. The WCD should be considered in structured patient management for newly diagnosed heart failure during the early phase of the disease. Careful patient selection, structured patient management, and patient's compliance is crucial for a successful WCD strategy.

Systematic ajmaline challenge in patients with long QT 3 syndrome caused by the most common mutation: a multicentre study.

AIMS: Overlap syndromes of long QT 3 syndrome (LQT3) and the Brugada syndrome (BrS) have been reported. Identification of patients with an overlapping phenotype is crucial before initiation of Class I antiarrhythmic drugs for LQT3. Aim of the present study was to elucidate the yield of ajmaline challenge in unmasking the Brugada phenotype in patients with LQT3 caused by the most common mutation, SCN5A-E1784K. METHODS AND RESULTS: Consecutive families in tertiary referral centres diagnosed with LQT3 caused by SCN5A-E1784K were included in the study. Besides routine clinical work-up, ajmaline challenge was performed after informed consent. A total of 23 subjects (11 female, mean age 27 ± 14 years) from 4 unrelated families with a family history of sudden cardiac death and familial diagnosis of the SCN5A-E1784K mutation underwent ajmaline challenge and genetic testing. Sixteen subjects (9 female) were found to be heterozygous carriers of SCN5A-E1784K. Ajmaline challenge was positive in 12 out of the 16 (75%) mutation carriers, but negative in all non-carriers. Following ajmaline, a significant shortening of the rate-corrected JT (JTc) interval was observed in mutation carriers. The baseline JTc interval was significantly longer in mutation carriers with a positive ajmaline challenge compared with those with a negative one. CONCLUSION: Overlap of LQT3 and BrS in patients carrying the most common mutation is high. Therefore, ajmaline challenge represents an important step to rule out potential BrS overlap in these patients before starting sodium channel blockers for the beneficial effect of QT shortening in LQT3.

One symptom, two arrhythmias: the rare and the even rarer.

BACKGROUND: Wolff-Parkinson-White (WPW) syndrome and idiopathic left ventricular tachycardia (ILVT) are rare and up to now the coexistence of both entities has rarely been reported. In patients with ventricular preexcitation the underlying mechanism of paroxysmal tachycardia most likely is atrioventricular reentrant tachycardia (AVRT). However, without ECG documentation of the tachycardia diagnosis of the underlying mechanism cannot be made due to similar clinical presentation of AVRT and ILVT. CASE PRESENTATION: We report a case of a two-staged occurrence of two rare arrhythmias in a young adult, who was admitted to our hospital twice within 6 months because of paroxysmal tachycardia. WPW syndrome and ILVT as underlying arrhythmias have been diagnosed and were ablated successfully. CONCLUSIONS: This case highlights the diagnostic defiance of rare tachycardia entities and the paramount importance of ECG documentation and analysis of all available tachycardia ECGs.

Brugada syndrome: clinical presentation and genotype-correlation with magnetic resonance imaging parameters.

AIMS: The purpose of the this study was to evaluate a possible genotype-phenotype correlation in BrS patients and to analyze possible associations with clinical events in affected patients. SCN5A gene encodes the alpha-subunit of the voltage-gated sodium channel NaV1.5. Its mutations are associated with a broad spectrum of hereditary arrhythmias such as long-QT syndrome, cardiac conduction diseases, and Brugada syndrome (BrS). Experimental studies have shown an interaction between SCN5A and cellular cytoskeleton, explaining its functional role in cellular integrity of heart cells. METHODS AND RESULTS: Cardiovascular magnetic resonance was performed on 81 consecutive genetically screened BrS patients and 30 healthy controls. Left ventricular (LV) and right ventricular (RV) volumes and dimensions were assessed and compared with respect to the genotype. Brugada syndrome patients with an SCN5A mutation (16 patients; 20%) revealed significantly larger RV volumes, along with lower RV ejection fraction, than patients without a mutation or controls, indicating a more severe phenotype in patients with a mutation. Furthermore, patients with an SCN5A mutation showed significantly more often a spontaneous type 1 BrS-electrocardiogram (ECG). In multivariate analysis, the presence of a spontaneous type 1 BrS-ECG showed the strongest association with cardiac events. Receiver-operating characteristic curve analysis indicated good predictive performance of RV end-diastolic volume, RV end-systolic, and LV cardiac output (area under the curve = 0.81, 0.81, and 0.2), with respect to the presence of an SCN5A mutation. CONCLUSION: Brugada syndrome patients with an SCN5A mutation reveal distinct changes in RV volumes and function when compared with those without an SCN5A mutation. Furthermore, mutation-positive patients have a higher likelihood of a spontaneous type 1 BrS-ECG, which is associated with a higher incidence of clinical events. Cardiovascular magnetic resonance may provide additional insight to distinguish between SCN5A mutation-positive and -negative BrS patients.

Early repolarization pattern: a marker of increased risk in patients with catecholaminergic polymorphic ventricular tachycardia.

AIMS: The early repolarization pattern (ERP) has been shown to be associated with arrhythmias in patients with short QT syndrome, Brugada syndrome, and ischaemic heart disease. Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome and related to malignant ventricular tachyarrhythmias in a structurally normal heart. The aim of this study was to evaluate the prevalence of ERP and clinical events in patients with CPVT. METHODS AND RESULTS: Digitalized resting 12-lead ECGs of patients were analysed for ERP and for repolarization markers (QT and Tpeak-Tend interval). The ERP was diagnosed as 'notching' or 'slurring' at the terminal portion of QRS with ≥0.1 mV elevation in at least two consecutive inferior (II, III, aVF) and/or lateral leads (V4-V6, I, aVL). Among 51 CPVT patients (mean age 36 ± 15 years, 11 males), the ERP was present in 23 (45%): strictly in the inferior leads in 9 (18%) patients, in the lateral leads in 9 (18%) patients, and in infero-lateral leads in 5 (10%) patients. All patients with ERP were symptomatic at presentation (23 of 23 patients with ERP vs. 19 of 28 patients without ERP, P = 0.003). Syncope was also more frequent in patients with ERP (18 of 23 patients with ERP vs. 11 of 28 patients without ERP, P = 0.005). CONCLUSION: A pathologic ERP is present in an unexpected large proportion (45%) of patients and is associated with an increased frequency of syncope. In patients with unexplained syncope and ERP at baseline, exercise testing should be performed to detect CPVT.