Editorial

3 pages, 182 KiB

Open AccessEditorial

Cardiac Implantable Electronic Devices Breakthrough: Are We Ready to Face the Future?

by Gianfranco Mitacchione

J. Clin. Med. 2022, 11(21), 6321; https://doi.org/10.3390/jcm11216321 - 26 Oct 2022

Viewed by 1107

Since its inception cardiac electrical therapy has evolved, with transvenous pacemakers (PMs) and implantable cardiac defibrillators (ICDs) providing significant benefits in terms of improved quality of life and reducing mortality in patients with cardiac conduction disturbances and/or requiring protection against ventricular arrhythmias [...] [...] Read more.

Since its inception cardiac electrical therapy has evolved, with transvenous pacemakers (PMs) and implantable cardiac defibrillators (ICDs) providing significant benefits in terms of improved quality of life and reducing mortality in patients with cardiac conduction disturbances and/or requiring protection against ventricular arrhythmias [...] Full article

(This article belongs to the Special Issue Advances in Cardiac Electrophysiology and Pacing)

Research

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11 pages, 2048 KiB

Open AccessArticle

Antiplatelet and Anti-Coagulation Therapy for Left-Sided Catheter Ablations: What Is beyond Atrial Fibrillation?

by Martina Nesti, Fabiana Lucà, David Duncker, Francesco De Sensi, Katarzyna Malaczynska-Rajpold, Jonathan M. Behar, Victor Waldmann, Ahmed Ammar, Gianluca Mirizzi, Rodrigue Garcia, Ahran Arnold, Evgeny N. Mikhaylov, Jedrzej Kosiuk and Luigi Sciarra

J. Clin. Med. 2023, 12(19), 6183; https://doi.org/10.3390/jcm12196183 - 25 Sep 2023

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Abstract

Aim: International guidelines on the use of anti-thrombotic therapies in left-sided ablations other than atrial fibrillation (AF) are lacking. The data regarding antiplatelet or anticoagulation strategies after catheter ablation (CA) procedures mainly derive from AF, whereas for the other arrhythmic substrates, the anti-thrombotic [...] Read more.

Aim: International guidelines on the use of anti-thrombotic therapies in left-sided ablations other than atrial fibrillation (AF) are lacking. The data regarding antiplatelet or anticoagulation strategies after catheter ablation (CA) procedures mainly derive from AF, whereas for the other arrhythmic substrates, the anti-thrombotic approach remains unclear. This survey aims to explore the current practices regarding antithrombotic management before, during, and after left-sided endocardial ablation, not including atrial fibrillation (AF), in patients without other indications for anti-thrombotic therapy. Material and Methods: Electrophysiologists were asked to answer a questionnaire containing questions on antiplatelet (APT) and anticoagulation therapy for the following left-sided procedures: accessory pathway (AP), atrial (AT), and ventricular tachycardia (VT) with and without structural heart disease (SHD). Results: We obtained 41 answers from 41 centers in 15 countries. For AP, before ablation, only four respondents (9.7%) used antiplatelets and two (4.9%) used anticoagulants. At discharge, APT therapy was prescribed by 22 respondents (53.7%), and oral anticoagulant therapy (OAC) only by one (2.4%). In patients with atrial tachycardia (AT), before ablation, APT prophylaxis was prescribed by only four respondents (9.7%) and OAC by eleven (26.8%). At discharge, APT was recommended by 12 respondents (29.3%) and OAC by 24 (58.5%). For VT without SHD, before CA, only six respondents (14.6%) suggested APT and three (7.3%) suggested OAC prophylaxis. At discharge, APT was recommended by fifteen respondents (36.6%) and OAC by five (12.2%). Regarding VT in SHD, before the procedure, eight respondents (19.5%) prescribed APT and five (12.2%) prescribed OAC prophylaxis. At discharge, the administration of anti-thrombotic therapy depended on the LV ejection fraction for eleven respondents (26.8%), on the procedure time for ten (24.4%), and on the radiofrequency time for four (9.8%), with a cut-off value from 1 to 30 min. Conclusions: Our survey indicates that the management of anti-thrombotic therapy surrounding left-sided endocardial ablation of patients without other indications for anti-thrombotic therapy is highly variable. Further studies are necessary to evaluate the safest approach to these procedures. Full article

(This article belongs to the Special Issue Advances in Cardiac Electrophysiology and Pacing)

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11 pages, 2180 KiB

Open AccessArticle

Electroanatomical Conduction Characteristics of Pig Myocardial Tissue Derived from High-Density Mapping

by Theresa Isabelle Wilhelm, Thorsten Lewalter, Johannes Fischer, Judith Reiser, Julia Werner, Christine Baumgartner, Lukas Gleirscher, Petra Hoppmann, Christian Kupatt, Klaus Tiemann and Clemens Jilek

J. Clin. Med. 2023, 12(17), 5598; https://doi.org/10.3390/jcm12175598 - 28 Aug 2023

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Abstract

Background: Ultra-high-density mapping systems allow more precise measurement of the heart chambers at corresponding conduction velocities (CVs) and voltage amplitudes (VAs). Our aim for this study was to define and compare a basic value set for unipolar CV and VA in all four [...] Read more.

Background: Ultra-high-density mapping systems allow more precise measurement of the heart chambers at corresponding conduction velocities (CVs) and voltage amplitudes (VAs). Our aim for this study was to define and compare a basic value set for unipolar CV and VA in all four heart chambers and their separate walls in healthy, juvenile porcine hearts using ultra-high-density mapping. Methods: We used the Rhythmia Mapping System to create electroanatomical maps of four pig hearts in sinus rhythm. CVs and VAs were calculated for chambers and wall segments with overlapping circular areas (radius of 5 mm). Results: We analysed 21 maps with a resolution of 1.4 points/mm². CVs were highest in the left atrium (LA), followed by the left ventricle (LV), right ventricle (RV), and right atrium (RA). As for VA, LV was highest, followed by RV, LA, and RA. The left chambers had a higher overall CV and VA than the right. Within the chambers, CV varied more in the right than in the left chambers, and VA varied in the ventricles but not in the atria. There was a slightly positive correlation between CVs and VAs at velocity values of <1.5 m/s. Conclusions: In healthy porcine hearts, the left chambers showed higher VAs and CVs than the right. CV differs mainly within the right chambers and VA differs only within the ventricles. A slightly positive linear correlation was found between slow CVs and low VAs. Full article

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11 pages, 681 KiB

Open AccessArticle

Long-Term Outcomes of Transvenous Lead Extraction: A Comparison in Patients with or without Infection from the Italian Region with the Oldest Population

by Luca Barca, Giuseppe Mascia, Paolo Di Donna, Paolo Sartori, Daniele Bianco, Roberta Della Bona, Stefano Benenati, Andrea Carlo Merlo, Antonia Luisa Buongiorno, Niki Kaufman, Antonio Vena, Matteo Bassetti and Italo Porto

J. Clin. Med. 2023, 12(13), 4543; https://doi.org/10.3390/jcm12134543 - 07 Jul 2023

Viewed by 683

Abstract

Background: The gold standard for the treatment of cardiac implantable electronic devices (CIEDs)-related infection and lead malfunction is transvenous lead extraction (TLE). To date, the risk of mortality directly related to TLE procedures is relatively low, but data on post-procedural and long-term mortality [...] Read more.

Background: The gold standard for the treatment of cardiac implantable electronic devices (CIEDs)-related infection and lead malfunction is transvenous lead extraction (TLE). To date, the risk of mortality directly related to TLE procedures is relatively low, but data on post-procedural and long-term mortality are limited, even more in the aging population. Methods: Consecutive patients with CIEDs who underwent TLE were retrospectively studied. The primary outcome was the endpoint of death, considering independent predictors of long-term clinical outcomes in the TLE aging population comparing patients with and without infection. Results: One hundred nineteen patients (male 77%; median age 76 years) were included in the analysis. Eighty-two patients (69%) documented infection, and thirty-seven (31%) were extracted for a different reason. Infected patients were older (80 vs. 68 years, p-value > 0.001) with more implanted catheters (p-value < 0.001). At the last follow-up (FU) available (median FU 4.1 years), mortality reached 37% of the patient population, showing a statistically significant difference between infected versus non-infected groups. At univariable analysis, age at TLE, atrial fibrillation, and anemia remained significant correlates of mortality; at multivariable analysis, only patients with anemia and atrial fibrillation have a 2.3-fold (HR 2.34; CI 1.16–4.75) and a 2.5-fold (HR 2.46; CI 1.33–4.54) increased rate of death, respectively. Conclusion: Our long-term data showed that aging patients who underwent TLE for CIED-related infection exhibit a high mortality risk during a long-term follow-up, potentially leading to a rapid and effective procedural approach in this patient population. Full article

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15 pages, 787 KiB

Open AccessArticle

The Value of Left Ventricular Mechanical Dyssynchrony and Scar Burden in the Combined Assessment of Factors Associated with Cardiac Resynchronization Therapy Response in Patients with CRT-D

by Tariel A. Atabekov, Mikhail S. Khlynin, Anna I. Mishkina, Roman E. Batalov, Svetlana I. Sazonova, Sergey N. Krivolapov, Victor V. Saushkin, Yuliya V. Varlamova, Konstantin V. Zavadovsky and Sergey V. Popov

J. Clin. Med. 2023, 12(6), 2120; https://doi.org/10.3390/jcm12062120 - 08 Mar 2023

Viewed by 1323

Abstract

Background: Cardiac resynchronization therapy (CRT) improves the outcome in patients with heart failure (HF). However, approximately 30% of patients are nonresponsive to CRT. The aim of this study was to determine the role of the left ventricular (LV) mechanical dyssynchrony (MD) and scar [...] Read more.

Background: Cardiac resynchronization therapy (CRT) improves the outcome in patients with heart failure (HF). However, approximately 30% of patients are nonresponsive to CRT. The aim of this study was to determine the role of the left ventricular (LV) mechanical dyssynchrony (MD) and scar burden as predictors of CRT response. Methods: In this study, we included 56 patients with HF and the left bundle-branch block with QRS duration ≥ 150 ms who underwent CRT-D implantation. In addition to a full examination, myocardial perfusion imaging and gated blood-pool single-photon emission computed tomography were performed. Patients were grouped based on the response to CRT assessed via echocardiography (decrease in LV end-systolic volume ≥15% or/and improvement in the LV ejection fraction ≥5%). Results: In total, 45 patients (80.3%) were responders and 11 (19.7%) were nonresponders to CRT. In multivariate logistic regression, LV anterior-wall standard deviation (adjusted odds ratio (OR) 1.5275; 95% confidence interval (CI) 1.1472–2.0340; p = 0.0037), summed rest score (OR 0.7299; 95% CI 0.5627–0.9469; p = 0.0178), and HF nonischemic etiology (OR 20.1425; 95% CI 1.2719–318.9961; p = 0.0331) were the independent predictors of CRT response. Conclusion: Scar burden and MD assessed using cardiac scintigraphy are associated with response to CRT. Full article

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12 pages, 4490 KiB

Open AccessArticle

Isolated Atrial Fibrillation, Inflammation and Efficacy of Radiofrequency Ablation: Preliminary Insights Based on a Single-Center Endomyocardial Biopsy Study

by Roman E. Batalov, Mikhail S. Khlynin, Yulia V. Rogovskaya, Svetlana I. Sazonova, Roman B. Tatarskiy, Nina D. Anfinogenova and Sergey V. Popov

J. Clin. Med. 2023, 12(4), 1254; https://doi.org/10.3390/jcm12041254 - 04 Feb 2023

Cited by 2 | Viewed by 1059

Abstract

The aim of the study was to evaluate the inflammatory changes in the myocardium, based on endomyocardial biopsy (EMB) data in patients undergoing radiofrequency ablation (RFA) for idiopathic atrial fibrillation (AF). A total of 67 patients with idiopathic AF were enrolled in the [...] Read more.

The aim of the study was to evaluate the inflammatory changes in the myocardium, based on endomyocardial biopsy (EMB) data in patients undergoing radiofrequency ablation (RFA) for idiopathic atrial fibrillation (AF). A total of 67 patients with idiopathic AF were enrolled in the study. Patients underwent the intracardiac examination, RFA of AF, and EMB with histological and immunohistochemical studies. The catheter-treatment effectiveness, and occurrence of early and late recurrences of atrial tachyarrhythmias, were assessed depending on the identified histological changes. Nine patients (13.4%) did not have any histological changes in the myocardium according to EMB. Fibrotic changes were detected in 26 cases (38.8%). Inflammatory changes according to the Dallas criteria were observed in 32 patients (47.8%). The follow-up period for patients averaged 19.3 ± 3.7 months. The effectiveness rates of primary RFA were 88.9% in patients with the intact myocardium, 46.2% in patients with fibrotic changes of varying severity, and 34.4% in patients with the presence of criteria for myocarditis. No early recurrence of arrhythmias was observed in patients with unchanged myocardia. The presence of inflammatory and fibrotic changes in the myocardium increased the rates of early and late arrhythmia recurrences and accordingly halved the effectiveness RFA of AF. Full article

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12 pages, 652 KiB

Open AccessArticle

Long-Term Follow-Up of Catheter Ablation for Premature Ventricular Complexes in the Modern Era: The Importance of Localization and Substrate

by Simone Gulletta, Alessio Gasperetti, Marco Schiavone, Gabriele Paglino, Pasquale Vergara, Paolo Compagnucci, Caterina Bisceglia, Manuela Cireddu, Nicolai Fierro, Giuseppe D’Angelo, Simone Sala, Lorenzo Rampa, Michela Casella, Patrizio Mazzone, Antonio Dello Russo, Giovanni Battista Forleo and Paolo Della Bella

J. Clin. Med. 2022, 11(21), 6583; https://doi.org/10.3390/jcm11216583 - 06 Nov 2022

Cited by 2 | Viewed by 2160

Abstract

Background: Large-scale studies evaluating long-term recurrence rates in both idiopathic and non-idiopathic PVC catheter ablation (CA) patients have not been reported. Objective: To evaluate the efficacy and safety of idiopathic and non-idiopathic PVC CA, investigating the predictors of acute and long-term efficacy. Methods: [...] Read more.

Background: Large-scale studies evaluating long-term recurrence rates in both idiopathic and non-idiopathic PVC catheter ablation (CA) patients have not been reported. Objective: To evaluate the efficacy and safety of idiopathic and non-idiopathic PVC CA, investigating the predictors of acute and long-term efficacy. Methods: This retrospective multicentric study included 439 patients who underwent PVC CA at three institutions from April-2015 to December-2021. Clinical success at 6 months’ follow-up, defined as a reduction of at least 80% of the pre-procedural PVC burden, was deemed the primary outcome. The secondary aims of the study were: clinical success at the last available follow-up, predictors of arrhythmic recurrences at long-term follow-up, and safety outcomes. Results: The median age was 51 years, with 24.9% patients being affected suffering from structural heart disease. The median pre-procedural PVC burden was 20.1%. PVCs originating from the RVOT were the most common index PVC observed (29.1%), followed by coronary cusp (CC) and non-outflow tract (OT) LV PVCs (23.1% and 19.0%). The primary outcome at 6 months was reached in 85.1% cases, with a significant reduction in the 24 h% PVC burden (−91.4% [−83.4; −96.7], p < 0.001); long-term efficacy was observed in 82.1% of cases at almost 3-year follow-up. The presence of underlying structural heart disease and non-OT LV region origin (aHR 1.77 [1.07–2.93], p = 0.027 and aHR = 1.96 [1.22–3.14], p = 0.005) was independently associated with recurrences. Conclusion: CA of both idiopathic and non-idiopathic PVCs showed a very good acute and long-term procedural success rate, with an overall low complication. Predictors of arrhythmic recurrence at follow-up were underlying structural heart disease and non-OT LV origin. Full article

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10 pages, 821 KiB

Open AccessArticle

Clinical Management of New-Onset Atrial Fibrillation in COVID-19 Patients Referred to a Tertiary Cardiac Arrhythmia Center after Hospital Discharge

by Marco Schiavone, Fabiola B. Sozzi, Alessio Gasperetti, Cecilia Gobbi, Elisa Gherbesi, Lucia Barbieri, Roberto Arosio, Gianfranco Mitacchione, Filippo Toriello, Andrea Faggiano, Maurizio Viecca, Giovanni B. Forleo and Stefano Carugo

J. Clin. Med. 2022, 11(19), 5661; https://doi.org/10.3390/jcm11195661 - 26 Sep 2022

Cited by 6 | Viewed by 1837

Abstract

Background: Available reports on the post-discharge management of atrial fibrillation (AF) in COVID-19 patients are scarce. The aim of this case series was to describe the clinical outcomes of new-onset AF in COVID-19 patients referred to a tertiary cardiac arrhythmia center after hospital [...] Read more.

Background: Available reports on the post-discharge management of atrial fibrillation (AF) in COVID-19 patients are scarce. The aim of this case series was to describe the clinical outcomes of new-onset AF in COVID-19 patients referred to a tertiary cardiac arrhythmia center after hospital discharge. Methods: All consecutive patients referred to our center for an ambulatory evaluation from 18 May 2020 to 15 March 2022 were retrospectively screened. Patients were included in the current analysis if new-onset AF was diagnosed during hospitalization for COVID-19 and then referred to our clinic. Results: Among 946 patients, 23 (2.4%) were evaluated for new-onset AF during COVID-19. The mean age of the study cohort was 71.5 ± 8.1 years; 87.0% were male. Median time from COVID-19 discharge and the first ambulatory evaluation was 53 (41.5–127) days; median follow-up time was 175 (83–336) days. At the in-office evaluation, 14 (60.9%) patients were in sinus rhythm, and nine patients were in AF. In 13.0% of cases, oral anticoagulation was stopped according to CHADS-VASc. Eight patients in AF were scheduled for electrical cardioversion; one patient was rate-controlled. Four patients were treated with catheter ablation (CA) during follow-up. Two post-cardioversion AF recurrences were detected during follow-up, while no recurrences were diagnosed among patients who underwent CA. Conclusion: Our data suggest that AF may not be considered as a simple bystander of the in-hospital COVID-19 course. Management of new-onset AF in post-COVID-19 patients referred to our clinic did not significantly differ from our usual practice, both in terms of long-term oral anticoagulation and in terms of rhythm control strategy. Full article

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13 pages, 2959 KiB

Open AccessArticle

Vacuum-Implemented Removal of Lead Vegetations in Cardiac Device-Related Infective Endocarditis

by Vincenzo Tarzia, Matteo Ponzoni, Giuseppe Evangelista, Chiara Tessari, Emanuele Bertaglia, Manuel De Lazzari, Fabio Zanella, Demetrio Pittarello, Federico Migliore and Gino Gerosa

J. Clin. Med. 2022, 11(15), 4600; https://doi.org/10.3390/jcm11154600 - 07 Aug 2022

Cited by 7 | Viewed by 1799

Abstract

When approaching infected lead removal in cardiac device-related infective endocarditis (CDRIE), a surgical consideration for large (>20 mm) vegetations is recommended. We report our experience with the removal of large CDRIE vegetations using the AngioVac system, as an alternative to conventional surgery. We [...] Read more.

When approaching infected lead removal in cardiac device-related infective endocarditis (CDRIE), a surgical consideration for large (>20 mm) vegetations is recommended. We report our experience with the removal of large CDRIE vegetations using the AngioVac system, as an alternative to conventional surgery. We retrospectively reviewed all infected lead extractions performed with a prior debulking using the AngioVac system, between October 2016 and April 2022 at our institution. A total of 13 patients presented a mean of 2(1) infected leads after a mean of 5.7(5.7) years from implantation (seven implantable cardioverter-defibrillators, four cardiac resynchronization therapy-defibrillators, and two pacemakers). The AngioVac system was used as a venous–venous bypass in six cases (46.2%), venous–venous ECMO-like circuit (with an oxygenator) in five (38.5%), and venous–arterial ECMO-like circuit in two cases (15.4%). Successful (>70%) aspiration of the vegetations was achieved in 12 patients (92.3%) and an intraoperative complication (cardiac perforation) only occurred in 1 case (7.7%). Subsequent lead extraction was successful in all cases, either manually (38.5%) or using mechanical tools (61.5%). The AngioVac system is a promising effective and safe option for large vegetation debulking in CDRIE. Planning the extracorporeal circuit design may represent the optimal strategy to enhance the tolerability of the procedure and minimize adverse events. Full article

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Review

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12 pages, 297 KiB

Open AccessReview

Catheter Ablation for Atrial Fibrillation in Structural Heart Disease: A Review

by Francesco Maria Angelo Brasca, Roberto Menè and Giovanni Battista Perego

J. Clin. Med. 2023, 12(4), 1431; https://doi.org/10.3390/jcm12041431 - 10 Feb 2023

Viewed by 1639

Abstract

Atrial fibrillation (AF) is the most common arrhythmia encountered in clinical practice. Patients with structural heart disease (SHD) are at an increased risk of developing this arrhythmia and are particularly susceptible to the deleterious hemodynamic effects it carries. In the last two decades, [...] Read more.

Atrial fibrillation (AF) is the most common arrhythmia encountered in clinical practice. Patients with structural heart disease (SHD) are at an increased risk of developing this arrhythmia and are particularly susceptible to the deleterious hemodynamic effects it carries. In the last two decades, catheter ablation (CA) has emerged as a valuable strategy for rhythm control and is currently part of the standard care for symptomatic relief in patients with AF. Growing evidence suggests that CA of AF may have potential benefits that extend beyond symptoms. In this review, we summarize the current knowledge of this intervention on SHD patients. Full article

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Other

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10 pages, 664 KiB

Open AccessSystematic Review

Systematic Review on S-ICD Lead Extraction

by Riccardo Vio, Enrico Forlin, Viktor Čulić, Sakis Themistoclakis, Riccardo Proietti and Paolo China

J. Clin. Med. 2023, 12(11), 3710; https://doi.org/10.3390/jcm12113710 - 27 May 2023

Cited by 1 | Viewed by 1179

Abstract

Background and purpose: Subcutaneous implantable cardioverter defibrillators (S-ICDs) have emerged in recent years as a valid alternative to traditional transvenous ICDs (TV-ICDs). Therefore, the number of S-ICD implantations is rising, leading to a consequent increase in S-ICD-related complications sometimes requiring complete device removal. [...] Read more.

Background and purpose: Subcutaneous implantable cardioverter defibrillators (S-ICDs) have emerged in recent years as a valid alternative to traditional transvenous ICDs (TV-ICDs). Therefore, the number of S-ICD implantations is rising, leading to a consequent increase in S-ICD-related complications sometimes requiring complete device removal. Thus, the aim of this systematic review is to gather all the available literature on S-ICD lead extraction (SLE), with particular reference to the type of indication, techniques, complications and success rate. Methods: Studies were identified by searching electronic databases (Medline via PubMed, Scopus and Web of Science) from inception to 21 November 2022. The search strategy adopted was developed using the following key words: subcutaneous, S-ICD, defibrillator, ICD, extraction, explantation. Studies were included if they met both of the following criteria: (1) inclusion of patients with S-ICD; (2) inclusion of patients who underwent SLE. Results: Our literature search identified 238 references. Based on the abstract evaluation, 38 of these citations were considered potentially eligible for inclusion, and their full texts were analyzed. We excluded 8 of these studies because no SLE was performed. Eventually, 30 studies were included, with 207 patients who underwent SLE. Overall, the majority of SLEs were performed for non-infective causes (59.90%). Infection of the device (affecting either the lead or the pocket) was the cause of SLE in 38.65% of cases. Indication data were not available in 3/207 cases. The mean dwelling time was 14 months. SLEs were performed using manual traction or with the aid of a tool designed for transvenous lead extraction (TLE), including either a rotational or non-powered mechanical dilator sheath. Conclusions: SLE is performed mainly for non-infective causes. Techniques vary greatly across different studies. Dedicated tools for SLE might be developed in the future and standard approaches should be defined. In the meantime, authors are encouraged to share their experience and data to further refine the existing variegated approaches. Full article

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6 pages, 2046 KiB

Open AccessBrief Report

Biventricular Arrhythmogenic Cardiomyopathy Associated with a Novel Heterozygous Plakophilin-2 Early Truncating Variant

by Tolga Çimen, Verena C. Wilzeck, Giulia Montrasio, Nicole R. Bonetti, Argelia Medeiros-Domingo, Christian Grebmer, Christian M. Matter, Felix C. Tanner, Robert Manka, Corinna B. Brunckhorst, Firat Duru and Ardan M. Saguner

J. Clin. Med. 2022, 11(24), 7513; https://doi.org/10.3390/jcm11247513 - 19 Dec 2022

Viewed by 1193

Abstract

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a hereditary condition that can cause sudden cardiac death in young, frequently athletic individuals under the age of 35 due to malignant arrhythmias. Competitive and endurance exercise may hasten the onset and progression of ARVC, leading to [...] Read more.

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a hereditary condition that can cause sudden cardiac death in young, frequently athletic individuals under the age of 35 due to malignant arrhythmias. Competitive and endurance exercise may hasten the onset and progression of ARVC, leading to right ventricular dysfunction and potentially fatal ventricular arrhythmias earlier in life. In this article, we present a novel, pathogenic, early truncating heterozygous variant in the PKP2 gene that causes biventricular arrhythmogenic cardiomyopathy and affects a family, of which the only member with the positive phenotype is a competitive endurance athlete. Full article

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