Background and Objectives: Aortic stenosis (AS) is a frequent valvular disease characterized by the obstruction of left ventricular outflow. The resulting hemodynamic and structural changes create an arrhythmogenic substrate, with sudden cardiac death (SCD) often caused by ventricular arrhythmias (VAs) being a feared complication. This review examines the relationship between severe AS and VA, detailing the epidemiology, pathophysiological mechanisms, risk factors, and management approaches prior to aortic valve replacement (AVR). Materials and Methods: We conducted a comprehensive narrative review of the historical and contemporary literature investigating ventricular arrhythmias in severe aortic stenosis. Literature searches were performed in PubMed, MEDLINE, and Scopus databases using keywords, including "aortic stenosis", "ventricular arrhythmia", "sudden cardiac death", and "aortic valve replacement". Both landmark historical studies and modern investigations utilizing advanced monitoring techniques were included to provide a complete evolution of the understanding. Results: The prevalence of ventricular ectopy and non-sustained ventricular tachycardia increases with AS severity and symptom onset. Left ventricular hypertrophy, myocardial fibrosis, altered electrophysiological properties, and ischemia create the arrhythmogenic substrate. Risk factors include the male sex, concomitant aortic regurgitation, elevated filling pressures, and syncope. Diagnostic approaches range from standard electrocardiography to continuous monitoring and advanced imaging. Management centers on timely valve intervention, with medical therapy serving primarily as a bridge to AVR. Conclusions: Ventricular arrhythmias represent a consequence of valvular pathology in severe AS rather than an independent entity. Their presence signals advanced disease and a heightened risk for adverse outcomes. Multidisciplinary management with vigilant monitoring and prompt surgical referral is essential. Understanding this relationship enables clinicians to better identify high-risk patients requiring urgent intervention before life-threatening arrhythmic events occur.

• Klíčová slova
• aortic stenosis, aortic valve disease, aortic valve replacement, sudden death, surgery, ventricular arrhythmia,
• MeSH
• aortální stenóza * komplikace   chirurgie   patofyziologie   MeSH
• chirurgická náhrada chlopně * metody   MeSH
• komorová tachykardie etiologie   MeSH
• lidé MeSH
• náhlá srdeční smrt etiologie   MeSH
• rizikové faktory MeSH
• srdeční arytmie * etiologie   patofyziologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

BACKGROUND AND AIMS: Risk stratification of sudden cardiac death after myocardial infarction and prevention by defibrillator rely on left ventricular ejection fraction (LVEF). Improved risk stratification across the whole LVEF range is required for decision-making on defibrillator implantation. METHODS: The analysis pooled 20 data sets with 140 204 post-myocardial infarction patients containing information on demographics, medical history, clinical characteristics, biomarkers, electrocardiography, echocardiography, and cardiac magnetic resonance imaging. Separate analyses were performed in patients (i) carrying a primary prevention cardioverter-defibrillator with LVEF ≤ 35% [implantable cardioverter-defibrillator (ICD) patients], (ii) without cardioverter-defibrillator with LVEF ≤ 35% (non-ICD patients ≤ 35%), and (iii) without cardioverter-defibrillator with LVEF > 35% (non-ICD patients >35%). Primary outcome was sudden cardiac death or, in defibrillator carriers, appropriate defibrillator therapy. Using a competing risk framework and systematic internal-external cross-validation, a model using LVEF only, a multivariable flexible parametric survival model, and a multivariable random forest survival model were developed and externally validated. Predictive performance was assessed by random effect meta-analysis. RESULTS: There were 1326 primary outcomes in 7543 ICD patients, 1193 in 25 058 non-ICD patients ≤35%, and 1567 in 107 603 non-ICD patients >35% during mean follow-up of 30.0, 46.5, and 57.6 months, respectively. In these three subgroups, LVEF poorly predicted sudden cardiac death (c-statistics between 0.50 and 0.56). Considering additional parameters did not improve calibration and discrimination, and model generalizability was poor. CONCLUSIONS: More accurate risk stratification for sudden cardiac death and identification of low-risk individuals with severely reduced LVEF or of high-risk individuals with preserved LVEF was not feasible, neither using LVEF nor using other predictors.

• Klíčová slova
• Implantable cardioverter-defibrillator, Myocardial infarction, Primary prevention, Sudden cardiac death,
• MeSH
• defibrilátory implantabilní * MeSH
• elektrokardiografie MeSH
• hodnocení rizik metody   MeSH
• infarkt myokardu * mortalita   komplikace   MeSH
• lidé MeSH
• náhlá srdeční smrt * prevence a kontrola   epidemiologie   etiologie   MeSH
• tepový objem * fyziologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• metaanalýza MeSH

BACKGROUND: The subcutaneous implantable cardioverter-defibrillator (S-ICD) was developed to overcome the risk of lead-related complications associated with the transvenous implantable cardioverter-defibrillator (TV-ICD). In contrast to the TV-ICD, the S-ICD is a completely extrathoracic device. Subsequently, complications differ between these 2 implantable cardioverter-defibrillators, which might impact patient perceptions of the therapies. This prespecified secondary analysis of the PRAETORIAN trial evaluates differences in quality of life. METHODS: The PRAETORIAN trial (A Prospective, Randomized Comparison of Subcutaneous and Transvenous Implantable Cardioverter Defibrillator Therapy) randomized patients with an implantable cardioverter-defibrillator indication, without the need for pacing to S-ICD or TV-ICD therapy. Two questionnaires were collected at baseline, discharge, 12 months, and 30 months. The Duke Activity Status Index measures cardiac-specific physical functioning, and the 36-Item Short Form Health Survey measures physical and mental well-being, with the subscales bodily pain and mental health being of interest in this analysis. Mann-Whitney U tests were used to compare study arms, and a mixed model was used to describe the questionnaire outcomes over time. RESULTS: Patients were randomized to S-ICD (n=426) and TV-ICD (n=423). In the S-ICD group, 20% were women versus 19% in the TV-ICD group. The median age was 63 (interquartile range, 54-69) years in the S-ICD group versus 64 (interquartile range, 56-69) years in the TV-ICD group. There were no significant differences in the Duke Activity Status Index and 36-Item Short Form Health Survey subscales for bodily pain and mental health between the groups at any time point. Patients with a shock in the last 90 days had significantly lower scores for social functioning (P=0.008) and role limitations due to emotional problems (P=0.001) than patients without a shock, but this effect did not differ between treatment arms. CONCLUSIONS: In a large randomized cohort of patients with an S-ICD or TV-ICD, no difference in overall quality of life was observed. However, implantable cardioverter-defibrillator shocks resulted in a reduction in quality of life, regardless of the device type or appropriateness. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT01296022.

• Klíčová slova
• arrhythmias, cardiac, defibrillators, implantable, mental health, quality of life,
• MeSH
• časové faktory MeSH
• defibrilátory implantabilní * MeSH
• duševní zdraví * MeSH
• elektrická defibrilace * přístrojové vybavení   škodlivé účinky   MeSH
• funkční status MeSH
• kvalita života * MeSH
• lidé středního věku MeSH
• lidé MeSH
• náhlá srdeční smrt prevence a kontrola   MeSH
• prospektivní studie MeSH
• protézy - design MeSH
• průzkumy a dotazníky MeSH
• rizikové faktory MeSH
• senioři MeSH
• srdeční arytmie terapie   diagnóza   patofyziologie   MeSH
• výsledek terapie MeSH
• zdravotní stav MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• randomizované kontrolované studie MeSH
• srovnávací studie MeSH

BACKGROUND: The subcutaneous implantable cardioverter-defibrillator (ICD) is associated with fewer lead-related complications than a transvenous ICD; however, the subcutaneous ICD cannot provide bradycardia and antitachycardia pacing. Whether a modular pacing-defibrillator system comprising a leadless pacemaker in wireless communication with a subcutaneous ICD to provide antitachycardia and bradycardia pacing is safe remains unknown. METHODS: We conducted a multinational, single-group study that enrolled patients at risk for sudden death from ventricular arrhythmias and followed them for 6 months after implantation of a modular pacemaker-defibrillator system. The safety end point was freedom from leadless pacemaker-related major complications, evaluated against a performance goal of 86%. The two primary performance end points were successful communication between the pacemaker and the ICD (performance goal, 88%) and a pacing threshold of up to 2.0 V at a 0.4-msec pulse width (performance goal, 80%). RESULTS: We enrolled 293 patients, 162 of whom were in the 6-month end-point cohort and 151 of whom completed the 6-month follow-up period. The mean age of the patients was 60 years, 16.7% were women, and the mean (±SD) left ventricular ejection fraction was 33.1±12.6%. The percentage of patients who were free from leadless pacemaker-related major complications was 97.5%, which exceeded the prespecified performance goal. Wireless-device communication was successful in 98.8% of communication tests, which exceeded the prespecified goal. Of 151 patients, 147 (97.4%) had pacing thresholds of 2.0 V or less, which exceeded the prespecified goal. The percentage of episodes of arrhythmia that were successfully terminated by antitachycardia pacing was 61.3%, and there were no episodes for which antitachycardia pacing was not delivered owing to communication failure. Of 162 patients, 8 died (4.9%); none of the deaths were deemed to be related to arrhythmias or the implantation procedure. CONCLUSIONS: The leadless pacemaker in wireless communication with a subcutaneous ICD exceeded performance goals for freedom from major complications related to the leadless pacemaker, for communication between the leadless pacemaker and subcutaneous ICD, and for the percentage of patients with a pacing threshold up to 2.0 V at a 0.4-msec pulse width at 6 months. (Funded by Boston Scientific; MODULAR ATP ClinicalTrials.gov NCT04798768.).

• MeSH
• bezdrátová technologie MeSH
• bradykardie * terapie   MeSH
• defibrilátory implantabilní * škodlivé účinky   MeSH
• design vybavení MeSH
• kardiostimulace umělá škodlivé účinky   metody   MeSH
• kardiostimulátor * škodlivé účinky   MeSH
• lidé středního věku MeSH
• lidé MeSH
• náhlá srdeční smrt * prevence a kontrola   etiologie   MeSH
• následné studie MeSH
• senioři MeSH
• srdeční arytmie * komplikace   terapie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• klinické zkoušky MeSH
• multicentrická studie MeSH

BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause sudden cardiac death (SCD) despite medical therapy. Therefore, implantable cardioverter-defibrillators (ICDs) are commonly advised. However, there is limited data on the outcomes of ICD use in children. OBJECTIVE: The purpose of this study was to compare the risk of arrhythmic events in pediatric patients with CPVT with and without an ICD. METHODS: We compared the risk of SCD in patients with RYR2 (ryanodine receptor 2) variants and phenotype-positive symptomatic CPVT patients with and without an ICD who were younger than 19 years and had no history of sudden cardiac arrest at phenotype diagnosis. The primary outcome was SCD; secondary outcomes were composite end points of SCD, sudden cardiac arrest, or appropriate ICD shocks with or without arrhythmic syncope. RESULTS: The study included 235 patients, 73 with an ICD (31.1%) and 162 without an ICD (68.9%). Over a median follow-up of 8.0 years (interquartile range 4.3-13.4 years), SCD occurred in 7 patients (3.0%), of whom 4 (57.1%) were noncompliant with medications and none had an ICD. Patients with ICD had a higher risk of both secondary composite outcomes (without syncope: hazard ratio 5.85; 95% confidence interval 3.40-10.09; P < .0001; with syncope: hazard ratio 2.55; 95% confidence interval 1.50-4.34; P = .0005). Thirty-one patients with ICD (42.5%) experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications. CONCLUSION: SCD events occurred only in patients without an ICD and mostly in those not on optimal medical therapy. Patients with an ICD had a high risk of appropriate and inappropriate shocks, which may be reduced with appropriate device programming. Severe ICD complications were common, and risks vs benefits of ICDs need to be considered.

• Klíčová slova
• Catecholaminergic polymorphic ventricular tachycardia, Implantable cardioverter-defibrillator, Inherited arrhythmia, Ryanodine receptor, Sudden cardiac death, Ventricular tachycardia,
• MeSH
• defibrilátory implantabilní * MeSH
• dítě MeSH
• katecholaminergní polymorfní komorová tachykardie MeSH
• komorová tachykardie * terapie   patofyziologie   MeSH
• lidé MeSH
• mladiství MeSH
• náhlá srdeční smrt * prevence a kontrola   etiologie   MeSH
• následné studie MeSH
• předškolní dítě MeSH
• retrospektivní studie MeSH
• ryanodinový receptor vápníkového kanálu genetika   MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Názvy látek
• ryanodinový receptor vápníkového kanálu MeSH

• MeSH
• defibrilátory implantabilní * MeSH
• dítě MeSH
• elektrická defibrilace přístrojové vybavení   MeSH
• lidé MeSH
• mladiství MeSH
• náhlá srdeční smrt prevence a kontrola   MeSH
• věkové faktory MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• Publikační typ
• úvodníky MeSH

Cardiac sarcoidosis (CS) is a form of inflammatory cardiomyopathy associated with significant clinical complications such as high-degree atrioventricular block, ventricular tachycardia, and heart failure as well as sudden cardiac death. It is therefore important to provide an expert consensus statement summarizing the role of different available diagnostic tools and emphasizing the importance of a multidisciplinary approach. By integrating clinical information and the results of diagnostic tests, an accurate, validated, and timely diagnosis can be made, while alternative diagnoses can be reasonably excluded. This clinical expert consensus statement reviews the evidence on the management of different CS manifestations and provides advice to practicing clinicians in the field on the role of immunosuppression and the treatment of cardiac complications based on limited published data and the experience of international CS experts. The monitoring and risk stratification of patients with CS is also covered, while controversies and future research needs are explored.

• Klíčová slova
• Cardiac sarcoidosis, Diagnosis, Epidemiology, Immunosuppression, Management, Multidisciplinary, Screening,
• MeSH
• imunosupresiva terapeutické užití   MeSH
• kardiomyopatie * diagnóza   terapie   MeSH
• lidé MeSH
• náhlá srdeční smrt prevence a kontrola   etiologie   MeSH
• sarkoidóza * diagnóza   terapie   komplikace   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH
• Názvy látek
• imunosupresiva MeSH

AIMS: Wearable cardioverter-defibrillators (WCDs) are indicated in patients at risk of sudden cardiac arrest who are not immediate candidates for implantable defibrillator therapy. Limitations of existing WCDs include poor compliance and high false alarm rates. The Jewel is a novel patch-WCD (P-WCD) that addresses these limitations with an adhesive-based design for near-continuous wear and a machine learning algorithm designed to minimize inappropriate detections. This was a first-in-human study of the Jewel P-WCD conducted in an electrophysiology (EP) lab to determine the safety and effectiveness of the device in terminating ventricular tachycardia/ventricular fibrillation (VT/VF) with a single shock. The aim was to evaluate the safety and effectiveness of terminating VT/VF with a single shock using the Jewel P-WCD. METHODS AND RESULTS: This was a first-in-human, prospective, single-arm, single-centre study in patients scheduled for an EP procedure in which VT/VF was expected to either spontaneously occur or be induced. The Jewel P-WCD was placed on consented patients; upon confirmation of VT/VF, a single shock (150 J) was delivered via the device. A group sequential design and Pocock alpha spending function was used to measure the observed proportion of successful VT/VF single-shock terminations. The endpoint was achieved if the lower confidence limit exceeded the performance goal of 62%, using a one-sided lower 97.4% exact confidence bound. Of 18 eligible subjects, 16 (88.9%, 97.4% confidence bound: 65.4%) were successfully defibrillated with a single shock, exceeding the primary endpoint performance goal with no adverse events. CONCLUSION: This first-in-human evaluation of the Jewel P-WCD demonstrated the safety and effectiveness of terminating VT/VF. CLINICAL TRIAL REGISTRATION: URL: https://clinicaltrials.gov/; Unique identifier: NCT05490459.

• Klíčová slova
• Sudden cardiac arrest, Ventricular arrhythmia, Wearable cardioverter-defibrillator,
• MeSH
• defibrilátory * MeSH
• design vybavení MeSH
• dospělí MeSH
• elektrická defibrilace * přístrojové vybavení   škodlivé účinky   MeSH
• fibrilace komor * terapie   diagnóza   MeSH
• komorová tachykardie * terapie   diagnóza   patofyziologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• náhlá srdeční smrt prevence a kontrola   MeSH
• nositelná elektronika * MeSH
• prospektivní studie MeSH
• senioři MeSH
• výsledek terapie MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• klinická studie MeSH

BACKGROUND: New methods to identify patients who benefit from a primary prophylactic implantable cardioverter-defibrillator (ICD) are needed. T-wave alternans (TWA) has been shown to associate with arrhythmogenesis of the heart and sudden cardiac death. We hypothesized that TWA might be associated with benefit from ICD implantation in primary prevention. METHODS AND RESULTS: In the EU-CERT-ICD (European Comparative Effectiveness Research to Assess the Use of Primary Prophylactic Implantable Cardioverter-Defibrillators) study, we prospectively enrolled 2327 candidates for primary prophylactic ICD. A 24-hour Holter monitor reading was taken from all recruited patients at enrollment. TWA was assessed from Holter monitoring using the modified moving average method. Study outcomes were all-cause death, appropriate shock, and survival benefit. TWA was assessed both as a contiguous variable and as a dichotomized variable with cutoff points <47 μV and <60 μV. The final cohort included 1734 valid T-wave alternans samples, 1211 patients with ICD, and 523 control patients with conservative treatment, with a mean follow-up time of 2.3 years. TWA ≥60 μV was a predicter for a higher all-cause death in patients with an ICD on the basis of a univariate Cox regression model (hazard ratio, 1.484 [95% CI, 1.024-2.151]; P=0.0374; concordance statistic, 0.51). In multivariable models, TWA was not prognostic of death or appropriate shocks in patients with an ICD. In addition, TWA was not prognostic of death in control patients. In a propensity score-adjusted Cox regression model, TWA was not a predictor of ICD benefit. CONCLUSIONS: T-wave alternans is poorly prognostic in patients with a primary prophylactic ICD. Although it may be prognostic of life-threatening arrhythmias and sudden cardiac death in several patient populations, it does not seem to be useful in assessing benefit from ICD therapy in primary prevention among patients with an ejection fraction of ≤35%.

• Klíčová slova
• T‐wave alternans, appropriate shock, death, heart failure, implantable cardioverter‐defibrillator,
• MeSH
• časové faktory MeSH
• defibrilátory implantabilní * MeSH
• elektrická defibrilace přístrojové vybavení   škodlivé účinky   MeSH
• elektrokardiografie ambulantní * metody   MeSH
• hodnocení rizik metody   MeSH
• lidé středního věku MeSH
• lidé MeSH
• náhlá srdeční smrt * prevence a kontrola   etiologie   MeSH
• prediktivní hodnota testů MeSH
• primární prevence * metody   MeSH
• prognóza MeSH
• prospektivní studie MeSH
• rizikové faktory MeSH
• senioři MeSH
• srdeční arytmie terapie   patofyziologie   diagnóza   prevence a kontrola   mortalita   MeSH
• srdeční frekvence fyziologie   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Geografické názvy
• Evropa epidemiologie   MeSH

BACKGROUND: The genetic basis of hypertrophic cardiomyopathy (HCM) is complex, and the relationship between genotype status and clinical outcome is incompletely resolved. METHODS AND RESULTS: We assessed a large international HCM cohort to define in contemporary terms natural history and clinical consequences of genotype. Consecutive patients (n=1468) with established HCM diagnosis underwent genetic testing. Patients with pathogenic (or likely pathogenic) variants were considered genotype positive (G+; n=312; 21%); those without definite disease-causing mutations (n=651; 44%) or variants of uncertain significance (n=505; 35%) were considered genotype negative (G-). Patients were followed up for a median of 7.8 years (interquartile range, 3.5-13.4 years); HCM end points were examined by cumulative event incidence. Over follow-up, 135 (9%) patients died, 33 from a variety of HCM-related causes. After adjusting for age, all-cause and HCM-related mortality did not differ between G- versus G+ patients (hazard ratio [HR], 0.78 [95% CI, 0.46-1.31]; P=0.37; HR, 0.93 [95% CI, 0.38-2.30]; P=0.87, respectively). Adverse event rates, including heart failure progression to class III/IV, heart transplant, or heart failure death, did not differ (G- versus G+) when adjusted for age (HR, 1.20 [95% CI, 0.63-2.26]; P=0.58), nor was genotype independently associated with sudden death event risk (HR, 1.39 [95% CI, 0.88-2.21]; P=0.16). In multivariable analysis, age was the only independent predictor of all-cause and HCM-related mortality, heart failure progression, and sudden death events. CONCLUSIONS: In this large consecutive cohort of patients with HCM, genotype (G+ or G-) was not a predictor of clinical course, including all-cause and HCM-related mortality and risk for heart failure progression or sudden death. G+ status should not be used to dictate clinical management or predict outcome in HCM.

• Klíčová slova
• genotype, hypertrophic cardiomyopathy, mortality, outcomes, sudden death,
• MeSH
• časové faktory MeSH
• dospělí MeSH
• fenotyp MeSH
• genetická predispozice k nemoci MeSH
• genetické testování metody   MeSH
• genotyp * MeSH
• hypertrofická kardiomyopatie * genetika   mortalita   diagnóza   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mutace MeSH
• náhlá srdeční smrt etiologie   epidemiologie   MeSH
• prognóza MeSH
• progrese nemoci MeSH
• rizikové faktory MeSH
• senioři MeSH
• srdeční selhání genetika   mortalita   MeSH
• transplantace srdce MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH