BACKGROUND: Among inherited cardiomyopathies involving the left ventricle, whether dilated or not, certain genotypes carry a well-established arrhythmic risk, notably manifested as sustained monomorphic ventricular tachycardia (SMVT). Nonetheless, the precise localization and electrophysiological profile of this substrate remain undisclosed across different genotypes. METHODS: Patients diagnosed with cardiomyopathy and left ventricle involvement due to high-risk genetic variants and SMVT treated by electrophysiological study were recruited from 18 European/US centers. Electrophysiological study, imaging, and outcomes data after ablation were assessed in relation to genotype. RESULTS: Seventy-one patients were included (49.6 Q1-Q3 [40-60] years, 76% men). They were divided into 4 groups according to the affected protein: desmosomal (DSP, PKP2, DSG2, and DSC2), nuclear membrane (LMNA and TMEM43), cytoskeleton (FLNC and DES), and sarcoplasmic reticulum (PLN). Desmosomal genes, TMEM43, and PLN were associated with biventricular disease, while variants in LMNA and cytoskeleton genes had predominant left ventricle involvement (P=0.001). The location of the clinical-SMVT substrate was significantly different based on genotype (P=0.005). DSP and cytoskeleton genes presented SMVTs with right bundle branch block morphology, which origin was identified in the inferolateral segments of the left ventricle. The other desmosomal genes (PKP2 and DSG2), along with TMEM43, showed SMVTs with left bundle branch block morphology and predominantly right ventricular substrate. In contrast, LMNA substrate was mainly observed in the interventricular septum. During a median of 26 Q1-Q3 (10.6-65) months, 27% of patients experienced recurrences of clinical SMVT with differences between genotypes (log-rank 0.016). Nuclear membrane genes demonstrated the highest recurrence rate compared with desmosomal genes (hazard ratio, 4.56 [95% CI, 1.5-13.8]). CONCLUSIONS: The anatomic substrate of SMVTs shows a strong correlation with the underlying genotype, electrocardiographic morphology, and recurrence rate. Particularly, patients with nuclear membrane gene variants have a significantly higher recurrence rate compared with those with desmosomal gene variants.

• MeSH
• akční potenciály MeSH
• dospělí MeSH
• elektrofyziologické techniky kardiologické MeSH
• fenotyp * MeSH
• genetická predispozice k nemoci * MeSH
• genetické asociační studie MeSH
• genotyp MeSH
• hodnocení rizik MeSH
• kardiomyopatie genetika   patofyziologie   diagnóza   MeSH
• katetrizační ablace MeSH
• komorová tachykardie * genetika   patofyziologie   diagnóza   MeSH
• lidé středního věku MeSH
• lidé MeSH
• rizikové faktory MeSH
• výsledek terapie MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• pozorovací studie MeSH
• Geografické názvy
• Evropa MeSH

AIMS: Catheter ablation is an effective treatment method for recurrent ventricular tachycardias (VTs). However, at least in part, procedural and clinical outcomes are limited by challenges in generating an adequate lesion size in the ventricular myocardium. We investigated procedural and clinical outcomes of VT ablation using a novel 'large-footprint' catheter that allows the creation of larger lesions either by radiofrequency (RF) or by pulsed field (PF) energy. METHODS AND RESULTS: In prospectively collected case series, we describe our initial experience with VT ablation using a lattice-tip, dual-energy catheter (Sphere-9, Medtronic), and a compatible proprietary electroanatomical mapping system (Affera, Medtronic). The study population consisted of 18 patients (aged 55 ± 15 years, one woman, structural heart disease: 94%, ischaemic heart disease: 56%, left ventricular ejection fraction: 34 ± 10%, electrical storm: 22%) with recurrent sustained VTs and ≥1 previously failed endocardial RF ablation with conventional irrigated-tip catheter in 66% of patients. On average, 12 ± 7 RF and 8 ± 9 PF applications were delivered per patient. In three-fourths of patients undergoing percutaneous epicardial ablation, spasms in coronary angiography were observed after PF applications. All resolved after intracoronary administration of nitrates. No acute phrenic nerve palsy was noted. One patient suffered from a stroke that resolved without sequelae. Post-ablation non-inducibility of VT was achieved in 89% of patients. Ventricular-arrhythmia-free survival at three months was 78%. CONCLUSION: VT ablation using a dual-energy lattice-tip catheter and a novel electroanatomical mapping system is feasible. It allows rapid mapping and effective substrate modification with good outcomes during short-term follow-up.

• MeSH
• akční potenciály MeSH
• časové faktory MeSH
• design vybavení MeSH
• doba přežití bez progrese choroby MeSH
• dospělí MeSH
• elektrofyziologické techniky kardiologické * MeSH
• katetrizační ablace * metody   přístrojové vybavení   škodlivé účinky   MeSH
• komorová tachykardie * chirurgie   patofyziologie   diagnóza   MeSH
• lidé středního věku MeSH
• lidé MeSH
• prospektivní studie MeSH
• recidiva MeSH
• senioři MeSH
• srdeční katétry * MeSH
• studie proveditelnosti * 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

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.

• 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

AIMS: Patients with structural heart disease (SHD) undergoing catheter ablation (CA) for ventricular tachycardia (VT) are at considerable risk of periprocedural complications, including acute haemodynamic decompensation (AHD). The PAINESD score was proposed to predict the risk of AHD. The goal of this study was to validate the PAINESD score using the retrospective analysis of data from a large-volume heart centre. METHODS AND RESULTS: Patients who had their first radiofrequency CA for SHD-related VT between August 2006 and December 2020 were included in the study. Procedures were mainly performed under conscious sedation. Substrate mapping/ablation was performed primarily during spontaneous rhythm or right ventricular pacing. A purposely established institutional registry for complications of invasive procedures was used to collect all periprocedural complications that were subsequently adjudicated using the source medical records. Acute haemodynamic decompensation triggered by CA procedure was defined as intraprocedural or early post-procedural (<12 h) development of acute pulmonary oedema or refractory hypotension requiring urgent intervention. The study cohort consisted of 1124 patients (age, 63 ± 13 years; males, 87%; ischaemic cardiomyopathy, 67%; electrical storm, 25%; New York Heart Association Class, 2.0 ± 1.0; left ventricular ejection fraction, 34 ± 12%; diabetes mellitus, 31%; chronic obstructive pulmonary disease, 12%). Their PAINESD score was 11.4 ± 6.6 (median, 12; interquartile range, 6-17). Acute haemodynamic decompensation complicated the CA procedure in 13/1124 = 1.2% patients and was not predicted by PAINESD score with AHD rates of 0.3, 1.8, and 1.1% in subgroups by previously published PAINESD terciles (<9, 9-14, and >14). However, the PAINESD score strongly predicted mortality during the follow-up. CONCLUSION: Primarily substrate-based CA of SHD-related VT performed under conscious sedation is associated with a substantially lower rate of AHD than previously reported. The PAINESD score did not predict these events. The application of the PAINESD score to the selection of patients for pre-emptive mechanical circulatory support should be reconsidered.

• MeSH
• hemodynamika * MeSH
• hypotenze etiologie   patofyziologie   diagnóza   MeSH
• jizva patofyziologie   MeSH
• katetrizační ablace * škodlivé účinky   MeSH
• komorová tachykardie * chirurgie   patofyziologie   etiologie   diagnóza   MeSH
• lidé středního věku MeSH
• lidé MeSH
• plicní edém etiologie   diagnóza   patofyziologie   MeSH
• pooperační komplikace etiologie   diagnóza   MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• senioři MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

AIMS: The ultra-low-temperature cryoablation (ULTC) ablation system using -196°C N2 cryogen has been reported to create lesions with freeze duration-dependent depth titratable to over 10 mm with minimum attenuation by scar. Cryocure-VT (NCT04893317) was a first-in-human clinical trial evaluating the safety and efficacy of a novel, purpose-built ULTC catheter in endocardial ablation of scar-dependent ventricular tachycardias (VTs). METHODS AND RESULTS: This prospective, multi-centre study enrolled patients referred for de novo or second ablations of recurrent monomorphic VT of both ischaemic and non-ischaemic aetiologies. Primary safety and efficacy endpoints of the study were freedom from device- or procedure-related major adverse events (MAEs) up to 30 days post-ablation, acute non-inducibility of clinical VTs at the end of the procedure, and freedom from sustained VT or implantable defibrillator intervention at 6 months. Ultra-low-temperature cryoablation was performed in 64 patients (age 67 ± 11 years, 78% ischaemic, ejection fraction = 35 ± 10%) at 9 centres. The primary acute effectiveness endpoint was achieved in 94% (51/54) of patients in whom post-ablation induction was attempted. There were no protocol-defined MAEs; four procedure-related serious adverse events resolved without clinical sequelae. At 6-month follow-up, 38 patients (60.3%) remained VT-free, and freedom from defibrillator shock was 81.0%, with no significant difference between ischaemic and non-ischaemic cohorts. In 47 patients with defibrillator for at least 6 months prior to the ablation, the VT burden was reduced from median of 4, inter-quartile range (IQR, 1-9) to 0, IQR (0-2). CONCLUSION: In this first-in-human multi-centre experience, endocardial ULTC ablation of monomorphic VT appears safe and effective in patients with both ischaemic-cardiomyopathy and non-ischaemic-cardiomyopathy. CLINICAL TRIAL REGISTRATION: NCT04893317.

• MeSH
• jizva komplikace   MeSH
• kardiomyopatie * komplikace   diagnóza   chirurgie   MeSH
• katetrizační ablace * škodlivé účinky   MeSH
• komorová tachykardie * diagnóza   etiologie   chirurgie   MeSH
• kryochirurgie * škodlivé účinky   MeSH
• lidé středního věku MeSH
• lidé MeSH
• prospektivní studie MeSH
• senioři MeSH
• teplota MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• senioři MeSH
• Publikační typ
• časopisecké články MeSH
• klinické zkoušky MeSH
• multicentrická studie MeSH

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.

• MeSH
• defibrilátory implantabilní * MeSH
• incidence MeSH
• komorová tachykardie * diagnóza   terapie   komplikace   MeSH
• lidé MeSH
• rizikové faktory MeSH
• srdeční arytmie diagnóza   terapie   MeSH
• srdeční selhání * komplikace   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Asie MeSH

AIMS: To predict worsening heart failure hospitalizations (WHFHs) in patients with implantable defibrillators and remote monitoring, the HeartInsight algorithm (Biotronik, Berlin, Germany) calculates a heart failure (HF) score combining seven physiologic parameters: 24 h heart rate (HR), nocturnal HR, HR variability, atrial tachyarrhythmia, ventricular extrasystoles, patient activity, and thoracic impedance. We compared temporal trends of the HF score and its components 12 weeks before a WHFH with 12-week trends in patients without WHFH, to assess whether trends indicate deteriorating HF regardless of alert status. METHODS AND RESULTS: Data from nine clinical trials were pooled, including 2050 patients with a defibrillator capable of atrial sensing, ejection fraction ≤ 35%, NYHA class II/III, no long-standing atrial fibrillation, and 369 WHFH from 259 patients. The mean HF score was higher in the WHFH group than in the no WHFH group (42.3 ± 26.1 vs. 30.7 ± 20.6, P < 0.001) already at the beginning of 12 weeks. The mean HF score further increased to 51.6 ± 26.8 until WHFH (+22% vs. no WHFH group, P = 0.003). As compared to the no WHFH group, the algorithm components either were already higher 12 weeks before WHFH (24 h HR, HR variability, thoracic impedance) or significantly increased until WHFH (nocturnal HR, atrial tachyarrhythmia, ventricular extrasystoles, patient activity). CONCLUSION: The HF score was significantly higher at, and further increased during 12 weeks before WHFH, as compared to the no WHFH group, with seven components showing different behaviour and contribution. Temporal trends of HF score may serve as a quantitative estimate of HF condition and evolution prior to WHFH.

• MeSH
• defibrilátory implantabilní * MeSH
• extrasystoly MeSH
• hospitalizace MeSH
• komorová tachykardie * diagnóza   terapie   MeSH
• lidé MeSH
• srdeční selhání * diagnóza   terapie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• metaanalýza MeSH

BACKGROUND: In severely affected patients with catecholaminergic polymorphic ventricular tachycardia, beta-blockers are often insufficiently protective. The purpose of this study was to evaluate whether flecainide is associated with a lower incidence of arrhythmic events (AEs) when added to beta-blockers in a large cohort of patients with catecholaminergic polymorphic ventricular tachycardia. METHODS: From 2 international registries, this multicenter case cross-over study included patients with a clinical or genetic diagnosis of catecholaminergic polymorphic ventricular tachycardia in whom flecainide was added to beta-blocker therapy. The study period was defined as the period in which background therapy (ie, beta-blocker type [beta1-selective or nonselective]), left cardiac sympathetic denervation, and implantable cardioverter defibrillator treatment status, remained unchanged within individual patients and was divided into pre-flecainide and on-flecainide periods. The primary end point was AEs, defined as sudden cardiac death, sudden cardiac arrest, appropriate implantable cardioverter defibrillator shock, and arrhythmic syncope. The association of flecainide with AE rates was assessed using a generalized linear mixed model assuming negative binomial distribution and random effects for patients. RESULTS: A total of 247 patients (123 [50%] females; median age at start of flecainide, 18 years [interquartile range, 14-29]; median flecainide dose, 2.2 mg/kg per day [interquartile range, 1.7-3.1]) were included. At baseline, all patients used a beta-blocker, 70 (28%) had an implantable cardioverter defibrillator, and 21 (9%) had a left cardiac sympathetic denervation. During a median pre-flecainide follow-up of 2.1 years (interquartile range, 0.4-7.2), 41 patients (17%) experienced 58 AEs (annual event rate, 5.6%). During a median on-flecainide follow-up of 2.9 years (interquartile range, 1.0-6.0), 23 patients (9%) experienced 38 AEs (annual event rate, 4.0%). There were significantly fewer AEs after initiation of flecainide (incidence rate ratio, 0.55 [95% CI, 0.38-0.83]; P=0.007). Among patients who were symptomatic before diagnosis or during the pre-flecainide period (n=167), flecainide was associated with significantly fewer AEs (incidence rate ratio, 0.49 [95% CI, 0.31-0.77]; P=0.002). Among patients with ≥1 AE on beta-blocker therapy (n=41), adding flecainide was also associated with significantly fewer AEs (incidence rate ratio, 0.25 [95% CI, 0.14-0.45]; P<0.001). CONCLUSIONS: For patients with catecholaminergic polymorphic ventricular tachycardia, adding flecainide to beta-blocker therapy was associated with a lower incidence of AEs in the overall cohort, in symptomatic patients, and particularly in patients with breakthrough AEs while on beta-blocker therapy.

• MeSH
• beta blokátory škodlivé účinky   MeSH
• defibrilátory implantabilní * MeSH
• flekainid škodlivé účinky   MeSH
• incidence MeSH
• klinické křížové studie MeSH
• komorová tachykardie * diagnóza   farmakoterapie   epidemiologie   MeSH
• lidé MeSH
• mladiství MeSH
• náhlá srdeční smrt epidemiologie   etiologie   prevence a kontrola   MeSH
• Check Tag
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

Ozařováním vyvolaná ischemická choroba srdeční je v současnosti jednou ze součástí oboru kardioonkologie, která na sebe poutá největší pozornost; případy akutního infarktu myokardu u mladých pacientů po radioterapii totiž nejsou nijak vzácné. Popisujeme klinický případ synkopy po infarktu myokardu s elevacemi úseku ST (STEMI) zadní stěny vyvolaném komorovou tachykardií u mladého pacienta, který před deseti lety absolvoval radioterapii a chemoterapii při léčbě non-Hodgkinova lymfomu.

Radiation induced coronary heart disease is among the most actively studied areas in cardio-oncology today. In fact, cases of acute myocardial infarction in young patients previously subjected to radiotherapy are not rare. We describe a clinical case of a syncope following an inferior STEMI started with ventricular tachycardia, in a young patient who underwent radio- and chemotherapy treatment 10 years ago for the treatment of non-Hodgkin's lymphoma.

• MeSH
• dospělí MeSH
• farmakoterapie metody   MeSH
• infarkt myokardu s elevacemi ST úseků etiologie   MeSH
• ischemická choroba srdeční diagnóza   etiologie   MeSH
• komorová tachykardie diagnóza   etiologie   MeSH
• lidé MeSH
• nehodgkinský lymfom komplikace   patologie   radioterapie   MeSH
• radioterapie metody   škodlivé účinky   MeSH
• synkopa * etiologie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH

• MeSH
• komorová tachykardie * diagnóza   etiologie   chirurgie   MeSH
• lidé MeSH
• píštěle * etiologie   chirurgie   MeSH
• radiochirurgie * škodlivé účinky   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH