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We report the case of a 36-year-old woman who presented to the emergency department complaining of palpitations and asthenia. Investigations showed frequent ventricular ectopy and severe left ventricular ejection fraction impairment. She was diagnosed with a peculiar condition defined multifocal ectopic premature Purkinje-related contractions syndrome, which in some cases can be associated with a dilated cardiomyopathy phenotype. Genetic testing showed a novel mutation in the SCN5A gene (c.673C > G). In the context of acute left ventricular dysfunction in a young patient, we discuss the clinical presentation of this rare condition and its clinical management, as well as its genetic substrate., (© 2024 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Background: Hybrid-convergent radiofrequency (RF) ablation targeting pulmonary veins (PVs) and left atrial posterior wall (LAPW) has shown better arrhythmic outcomes than an endocardial-only RF strategy, despite higher rates of complications. Comparisons with extensive pulsed field ablation (PFA) are currently lacking., Objectives: This study aimed to compare the efficacy and safety of the hybrid-convergent RF vs PFA of PVs and LAPW in long-standing persistent atrial fibrillation (LSPAF)., Methods: Ninety-three consecutive LSPAF patients, treated with 2-step hybrid-convergent RF ablation (hybrid group, n = 49) or with PFA of PVs and LAPW (PFA group, n = 44) were enrolled. Primary efficacy endpoint was defined as any atrial tachyarrhythmias (ATA) recurrence after the 3-month blanking period, over a follow-up time of 12 months. Periprocedural adverse events and late complications during follow-up were deemed primary safety outcomes., Results: The hybrid and PFA groups had similar baseline characteristics; mean age was hybrid 63.8 ± 10.6 years vs PFA 66.0 ± 7.4 years; P = 0.105. PV and LAPW ablation were acutely successful in all patients. Step 1 hybrid-epicardial procedures were longer than PFA (166 [Q1-Q3: 140-205] minutes vs 107.5 [Q1-Q3: 82.5-12] minutes; P < 0.01). At 12-month follow-up, there was no difference in ATA recurrences between groups (hybrid 36.7% vs PFA 40.9%; P = 0.680; log-rank at survival analysis P = 0.539). After adjusting for confounders, a larger left atrial volume and recurrences during the blanking-period were predictors of ATA recurrences after ablation, regardless of procedural technique employed. PFA showed a better safety profile with a lower rate of major periprocedural complications compared with hybrid ablation (12% vs 0%; P = 0.028)., Conclusions: Hybrid-convergent and PFA share comparable arrhythmic outcomes in LSPAF, but hybrid-convergent ablation carries higher periprocedural risks., Competing Interests: Funding Support and Author Disclosures Dr Tondo serves a member of scientific advisory boards for Medtronic Inc and Boston Scientific; and receives lecture/proctoring fees from Abbott Medical, AtriCure, Boston Scientific, and Medtronic Inc. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Conflict of interest: The authors do no report disclosures regarding this article.

Background: Arrhythmogenic cardiomyopathy (ACM) is an inherited heart disease characterized by fibro-fatty replacement of myocardium. Limited data is available concerning cardioembolic stroke. This study sought to determine the occurrence of cardioembolic ischemic events (CIEs) in ACM patients and to identify clinical and imaging predictors of CIEs., Methods: Every consecutive ACM patient was enrolled. ECG, Holter monitoring or implantable cardiac devices were used to detect atrial arrhythmias (AAs). CIEs were defined according to TOAST classification., Results: In our cohort of 111 patients, CIEs were observed in eleven (10%) over a 12.9-year median follow-up, with an incidence of 7.9 event/1000 patient-year (HR 4.12 compared to general population). Mean age at the event was 42 ± 9 years. Female sex (p = 0.03), T-wave inversion (p = 0.03), RVOT dilatation (p = 0.006) and lower LVEF (p = 0.006) were associated with CIEs. Among patients with AAs (23/111, 20.7%), 5 (21.7%) experienced CIEs. CHA 2 DS 2 -VASc did not prove useful to define patients at higher risk of CIEs (p = 0.098). 60% of stroke suffering patients had a pre-event score between 0 and 1 (if female)., Conclusions: In ACM patients, CIEs are much more common than in general population and present a high burden at younger age. AAs relate to less than half of these events. In AAs patients, CHA 2 DS 2 -VASc is not useful to stratify those requiring oral anticoagulation. As a hypothesis-generating study, our research proposes the role of atrial myopathy, irrespective of AAs, as a pivotal factor in thrombogenesis risk, pointing out a definite unmet need in ACM therapeutic algorithm., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

The identification of heart failure (HF) patients at risk for arrhythmic sudden cardiac arrest (SCA) is a major challenge in the cardiovascular field. In addition to optimal medical treatment for HF, implantable cardioverter defibrillator (ICD) is currently recommended to prevent SCA in patients with reduced left ventricular ejection fraction (LVEF). The indication for an ICD implantation, in addition to HF etiology, New York Health Association (NYHA) class and life expectancy, mainly depends on LVEF value at echocardiography. However, the actual role of LVEF in the prediction of SCA has recently been debated, while newer multimodality imaging techniques with increased prognostic accuracy have been developed. Speckle tracking imaging allows the quantification of mechanical dispersion, a marker of electrophysiological heterogeneity predisposing to malignant arrhythmias, while advanced cardiac magnetic resonance techniques such as myocardial T1-mapping and extracellular volume fraction assessment allow the evaluation of interstitial diffuse fibrosis. Nuclear imaging is helpful for the appraisal of sympathetic nervous system dysfunction, while newer computed tomography techniques assessing myocardial delayed enhancement allow the identification of focal myocardial scar. This review will focus on the most modern advances in the field of cardiovascular imaging along with its applications for the prediction of SCA in patients with HF. Modern artificial intelligence applications in cardiovascular imaging will also be discussed., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2023 The Authors. Published by Elsevier Ltd.)

Background: The adoption of Computed tomography (CT)-defined sarcopenia to risk stratify transcatheter aortic valve implantation (TAVI) candidates remains limited by a lack of both standardized definition and evidence of independent value over currently adopted mortality prediction tools., Methods: 391 consecutive TAVI patients with pre-procedural CT scan were included (81 ​± ​6 years, 57.5% male, STS-PROM score 4.4 ​± ​3.6%) and abdominal muscle retrospectively quantified. The two definitions of radiologic sarcopenia previously adopted in TAVI studies were compared (psoas muscle area [PMA] at the L4 vertebra level: "PMA-sarcopenia"; indexed skeletal muscle area at the L3 vertebra level: "SMI-sarcopenia"). The primary endpoint was longer available-term all-cause mortality. Secondary endpoints were Valve Academic Research Consortium-2-defined in-hospital and 30-day outcomes., Results: SMI- and PMA-sarcopenia were present in 192 (49.1%) and 117 (29.9%) patients, respectively. After a median of 24 (12-30) months follow-up, 83 (21.2%) patients died. PMA-(adj-HR 1.81, 95%CI 1.12-2.93, p ​= ​0.015), but not SMI-sarcopenia (adj-HR 1.23, 95%CI 0.76-2.00, p ​= ​0.391), was associated with all-cause mortality independently of age, sex and in-study outcome predictors (atrial fibrillation, hemoglobin, history of peripheral artery disease, cancer and subcutaneous adipose tissue). PMA-defined sarcopenia provided additive prognostic value over current post-TAVI mortality risk estimators including STS-PROM (p ​= ​0.001), Euroscore II (p ​= ​0.025), Charlson index (p ​= ​0.025) and TAVI2-score (p ​= ​0.020). Device success, early safety, clinical efficacy and 30-day all-cause death were unaffected by sarcopenia status regardless of definition., Conclusions: PMA-sarcopenia (but not SMI-sarcopenia) is predictive of 2 year mortality among TAVI patients. The prognostic information provided by PMA-sarcopenia is independent of the tools currently adopted to predict post-TAVI mortality in clinical practice., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to declare., (Copyright © 2021 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)