Review
Medicine, General & Internal
Nicolo Martini, Martina Testolina, Gian Luca Toffanin, Rocco Arancio, Luca De Mattia, Sergio Cannas, Giovanni Morani, Bortolo Martini
Summary: Brugada syndrome is characterized by a unique electrocardiographic pattern and major ventricular arrhythmic events, with ajmaline challenge being a popular test despite controversial results. Additionally, the characteristic pattern of this syndrome may also be induced in other cardiac or systemic diseases.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Cardiac & Cardiovascular Systems
Peter J. Schwartz, Cristina Moreno, Maria-Christina Kotta, Matteo Pedrazzini, Lia Crotti, Federica Dagradi, Silvia Castelletti, Kristina H. Haugaa, Isabelle Denjoy, Maria A. Shkolnikova, Paul A. Brink, Marshall J. Heradien, Sandrine R. M. Seyen, Roel L. H. M. G. Spatjens, Carla Spazzolini, Paul G. A. Volders
Summary: Mutations surrounding the A341V mutation in the KCNE1 gene increase the risk of arrhythmias, and the specific loss of PKA-dependent I-Ks enhancement in A341V correlates with its phenotypic severity.
EUROPEAN HEART JOURNAL
(2021)
Article
Cardiac & Cardiovascular Systems
Meng Wang, Derick R. R. Peterson, Eleonora Pagan, Vincenzo Bagnardi, Andrea Mazzanti, Scott McNitt, David Q. Q. Rich, Christopher L. L. Seplaki, Valentina Kutyifa, Bronislava Polonsky, Alon Barsheshet, Deni Kukavica, Spencer Rosero, Ilan Goldenberg, Silvia Priori, Wojciech Zareba
Summary: This study developed two risk prediction algorithms for assessing the 5-year absolute risk of life-threatening arrhythmic events in LQTS patients, aiding in clinical decision making.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
Article
Cardiac & Cardiovascular Systems
Haoyang Lu, Wen Ding, Hui Xiao, Manyu Dai, Yangcheng Xue, Zhuoran Jia, Jie Guo, Mengzuo Wu, Bing Shen, Ren Zhao
Summary: In this study, we report a missense mutation P441L in the C-terminus of KCNQ1 gene, which leads to a severe LQT1 phenotype in a 37-year-old female patient. Variant P441L shows reduced membrane trafficking and interaction with KCNQ1-KCNE1, resulting in potassium efflux disorder and eventually causing LQT1.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
Article
Cardiac & Cardiovascular Systems
Kathryn E. Waddell-Smith, Alexandra A. Chaptynova, Jian Li, Jackie R. Crawford, Halina Hinds, Jonathan R. Skinner
Summary: This study found that the average repolarization length recorded by Holter, particularly mRTPc, better reflects the status and clinical risk of LQTS than the initial 12-lead QTc. mRTPc showed higher sensitivity and specificity for genotype positive status and the risk of cardiac arrest compared to 12-lead QTc.
JOURNAL OF CARDIOVASCULAR DEVELOPMENT AND DISEASE
(2022)
Article
Chemistry, Analytical
Eros Pasero, Fiorenzo Gaita, Vincenzo Randazzo, Pierre Meynet, Sergio Cannata, Philippe Maury, Carla Giustetto
Summary: This study aimed to use artificial intelligence algorithms to analyze ECGs of SQTS patients and assist in distinguishing between those with and without life-threatening arrhythmic events. The results showed that artificial intelligence can help clinicians better assess the arrhythmia risk in SQTS patients, with shallow neural networks' processing features performing the best in identifying patients who will not experience potentially lethal events. This could potentially improve ECG-based risk stratification and save the lives of young and healthy individuals.
Review
Cardiac & Cardiovascular Systems
Chiara Scrocco, Connie R. Bezzina, Michael J. Ackerman, Elijah R. Behr
Summary: Epidemiological studies have shown a genetic component to the risk of sudden cardiac death (SCD), especially in young individuals with rare, monogenic forms of heart disease. Despite comprehensive pathology and genetic evaluations, a proportion of SCD cases in young individuals remain unexplained, posing challenges in identifying family members at risk of SCD.
NATURE REVIEWS CARDIOLOGY
(2021)
Article
Physiology
Dafni Charisopoulou, George Koulaouzidis, Annika Rydberg, Michael Y. Henein
Summary: Patients with inherited long QT syndrome (LQTS) exhibit a reversed longitudinal relaxation sequence, which worsens during exercise, particularly in those with previous cardiac events.
FRONTIERS IN PHYSIOLOGY
(2022)
Review
Biology
J. C. Hancox, C. Y. Du, A. Butler, Y. Zhang, C. E. Dempsey, S. C. Harmer, H. Zhang
Summary: Congenital short QT syndrome (SQTS) is a rare condition characterized by shortened QTc intervals on the electrocardiogram and increased susceptibility to arrhythmias and sudden death. Mutations in K+ channel subunit genes (SQT1-3) have a strong causal relationship with SQTS and impact the electrophysiological properties of cardiac potassium currents. This article reviews the evidence for these mutations and their link to arrhythmia susceptibility, and discusses future research directions.
PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES
(2023)
Article
Multidisciplinary Sciences
Ashish Kumar Sharma, Shivam Singh, Mehvish Bhat, Kartik Gill, Mohammad Zaid, Sachin Kumar, Anjali Shakya, Junaid Tantray, Divyamol Jose, Rashmi Gupta, Tsering Yangzom, Rajesh Kumar Sharma, Sanjay Kumar Sahu, Gulshan Rathore, Priyanka Chandolia, Mithilesh Singh, Anurag Mishra, Shobhit Raj, Archita Gupta, Mohit Agarwal, Sumaiya Kifayat, Anamika Gupta, Prashant Gupta, Ankit Vashist, Parth Vaibhav, Nancy Kathuria, Vipin Yadav, Ravindra Pal Singh, Arun Garg
Summary: Cardiac rhythm is regulated by micro-macroscopic structures of the heart. Both traditional and genetic animal models are important for studying the characteristics and significance of cardiac arrhythmias.
SCIENTIFIC REPORTS
(2023)
Review
Pharmacology & Pharmacy
Tibor Hornyik, Marina Rieder, Alessandro Castiglione, Peter Major, Istvan Baczko, Michael Brunner, Gideon Koren, Katja E. Odening
Summary: To study human cardiac diseases and develop treatment strategies, complex interactions of cardiac cells on multiple levels need to be considered. While mice are commonly used in cardiac research, differences in function and composition limit their translatability; rabbits, with closer resemblance to human hearts, are suitable as models. Different methodological approaches for generating transgenic rabbits and their utility in understanding disease mechanisms and treatment options are discussed.
BRITISH JOURNAL OF PHARMACOLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Roddy Walsh, Arnon Adler, Ahmad S. Amin, Emanuela Abiusi, Melanie Care, Hennie Bikker, Simona Amenta, Harriet Feilotter, Eline A. Nannenberg, Francesco Mazzarotto, Valentina Trevisan, John Garcia, Ray E. Hershberger, Marco Perez, Amy C. Sturm, James S. Ware, Wojciech Zareba, Valeria Novelli, Arthur A. M. Wilde, Michael H. Gollob
Summary: Catecholaminergic polymorphic ventricular tachycardia (CPVT) and short QT syndrome (SQTS) are inherited arrhythmogenic disorders. Seven CPVT and four SQTS genes have valid evidence for disease causation and should be included in genetic testing panels. Additional genes associated with conditions that may mimic clinical features of CPVT/SQTS have potential utility for differential diagnosis.
EUROPEAN HEART JOURNAL
(2022)
Article
Cardiac & Cardiovascular Systems
Sami Viskin, Ehud Chorin, Arie Lorin Schwartz, Piotr Kukla, Raphael Rosso
Summary: Cardiac memory is an interesting electrocardiographic phenomenon where T waves become abnormal in the opposite direction of wide QRS complex, and normalize at a later stage. Reduction of repolarizing potassium currents during cardiac memory may lead to prolonged QT syndrome and ventricular arrhythmias.
Article
Biochemistry & Molecular Biology
William A. Agudelo, Sebastian Ramiro Gil-Quinones, Alejandra Fonseca, Alvaro Arenas, Laura Castro, Diana Carolina Sierra-Diaz, Manuel A. Patarroyo, Paul Laissue, Carlos F. Suarez, Rodrigo Cabrera
Summary: Congenital long QT syndrome (LQTS) is a cardiac channelopathy characterized by prolonged QT interval and T-wave abnormalities, often caused by mutations in genes such as KCNQ1, KCNH2, and SCN5A. This study aimed to investigate the structural impact of compound mutations on potassium channels in severe LQTS phenotypes using molecular dynamic simulations. The findings suggest that mutant channels show moderate changes in energy and mobility, potentially influencing K+ ion flow and differentiating from wild-type channels.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Audrey Farrugia, Kevin Rollet, Jerome Sinniger, Susana Brun, Caroline Spenle, Bertrand Ludes, Omar Taleb, Ayikoe Guy Mensah-Nyagan
Summary: The KCNH2 mutation identified in this study affects the electrophysiological properties of cells, leading to Long QT syndrome.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Cardiac & Cardiovascular Systems
Jean-Baptiste Guichard, Feng Xiong, Xiao-Yan Qi, Nathalie L'Heureux, Roddy Hiram, Jiening Xiao, Patrice Naud, Jean-Claude Tardif, Antoine Da Costa, Stanley Nattel
Summary: This study assessed the specific contributions of atrial fibrillation-related atrial arrhythmia vs. associated ventricular arrhythmia to remodeling. The results showed that both atrial tachyarrhythmia and rapid ventricular response during AF produce distinct atrial remodelling, providing new insights into AF-related remodeling and ventricular rate-control considerations.
CARDIOVASCULAR RESEARCH
(2021)
Article
Cardiac & Cardiovascular Systems
Roddy Hiram, Feng Xiong, Patrice Naud, Jiening Xiao, Martin Sirois, Jean-Francois Tanguay, Jean-Claude Tardif, Stanley Nattel
Summary: The study found that the inflammation-resolution enhancing molecule RvD1 can prevent atrial arrhythmogenic remodeling in experimental RHD by suppressing inflammatory signaling and fibrotic/electrical remodeling.
CARDIOVASCULAR RESEARCH
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Martin Aguilar, Jordi Heijman, Dobromir Dobrev, Stanley Nattel
CANADIAN JOURNAL OF CARDIOLOGY
(2021)
Review
Cardiac & Cardiovascular Systems
Sami O. Simons, Adrian Elliott, Manuel Sastry, Jeroen M. Hendriks, Michael Arzt, Michiel Rienstra, Jonathan M. Kalman, Hein Heidbuchel, Stanley Nattel, Geertjan Wesseling, Ulrich Schotten, Isabelle C. van Gelder, Frits M. E. Franssen, Prashanthan Sanders, Harry J. G. M. Crijns, Dominik Linz
Summary: COPD is highly prevalent in AF patients and may promote AF progression and reduce treatment efficacy. Close interdisciplinary collaboration between electrophysiologists/cardiologists and pulmonologists is required for the diagnosis and treatment of COPD in AF patients. Acute exacerbation of COPD may transiently increase AF risk.
EUROPEAN HEART JOURNAL
(2021)
Review
Cardiac & Cardiovascular Systems
Xixiao Li, Anna Garcia-Elias, Begona Benito, Stanley Nattel
Summary: This article reviews the direct effects of stretch on cardiac fibroblasts, discussing the similarities and differences among studies in observed effects, the signaling pathways involved, and the factors affecting stretch-related phenotypes. The evidence regarding direct fibroblast activation by stretch is deemed weak and inconsistent, and further research is needed to clarify this issue.
CARDIOVASCULAR RESEARCH
(2022)
Review
Cardiac & Cardiovascular Systems
Stanley Nattel, Philip T. Sager, Joerg Hueser, Jordi Heijman, Dobromir Dobrev
Summary: Atrial fibrillation is the most common sustained clinical arrhythmia, contributing significantly to population morbidity and mortality. Current therapies for rhythm control are not optimally effective or safe, requiring the use of preclinical models for development of improved treatment options. Despite insights gained from extensive research, translating mechanistic discoveries into clinically applicable safe and effective therapies has proven challenging.
CARDIOVASCULAR RESEARCH
(2021)
Review
Cardiac & Cardiovascular Systems
Jordi Heijman, Henry Sutanto, Harry J. G. M. Crijns, Stanley Nattel, Natalia A. Trayanova
Summary: This review discusses the emerging and future applications of computational modeling in atrial fibrillation (AF) management. Computational modeling enables understanding of AF mechanisms, risk prediction, and personalized therapy, but faces challenges and limitations in routine clinical application. Future perspectives include the increasing prominence of computational modeling in clinical trials with rapid advancements in electronic technologies.
CARDIOVASCULAR RESEARCH
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Donald M. Bers, Stanley Nattel
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2021)
Review
Cardiac & Cardiovascular Systems
Ihab Elsokkari, Yukiomi Tsuji, John L. Sapp, Stanley Nattel
Summary: Electrical storm, a condition characterized by frequent ventricular tachycardia/ventricular fibrillation within a short period, is becoming more common with the widespread use of implantable cardioverter defibrillator (ICD) therapy. It is considered a medical emergency that requires hospitalization and expert management, significantly impacting short- and long-term outcomes. This condition typically occurs in patients with structural heart disease or inherited channelopathies. The management involves maintaining hemodynamic stability, ICD interrogation and reprogramming, identification and management of triggers, and the use of antiarrhythmic drugs or catheter ablation.
CANADIAN JOURNAL OF CARDIOLOGY
(2022)
Editorial Material
Cardiac & Cardiovascular Systems
Stanley Nattel
CANADIAN JOURNAL OF CARDIOLOGY
(2022)
Letter
Cardiac & Cardiovascular Systems
Amanda K. Morrow, T. Andrew Zabel, Jennifer Dodson, Sarah A. Korth
CIRCULATION-ARRHYTHMIA AND ELECTROPHYSIOLOGY
(2022)
Review
Cardiac & Cardiovascular Systems
Mozhdeh Mehdizadeh, Martin Aguilar, Eric Thorin, Gerardo Ferbeyre, Stanley Nattel
Summary: This review discusses the role of cellular senescence in cardiac disease, outlines therapeutic strategies for targeting senescence, and considers potential implications for improving the management of patients with heart disease. The complex senescence-associated secretory phenotype (SASP) of senescent cells has important effects on cell and tissue biology. Future research is needed to better understand the precise role of senescent cells in various cardiac pathologies and develop strategies for preventing their accumulation.
NATURE REVIEWS CARDIOLOGY
(2022)
Review
Cardiac & Cardiovascular Systems
Monika Gawalko, Arnela Saljic, Na Li, Issam Abu-Taha, Thomas Jespersen, Dominik Linz, Stanley Nattel, Jordi Heijman, Anke Fender, Dobromir Dobrev
Summary: Obesity is a significant contributing factor to atrial fibrillation (AF) by causing systemic changes and affecting different adipose tissue depots. Adipose tissue secretes various mediators that promote AF and cardiac remodeling. This review discusses the role of obesity-related factors, specific adipose tissue depots, and molecular mechanisms in AF, highlighting gaps in current knowledge and implications for clinical management.
CARDIOVASCULAR RESEARCH
(2023)
Review
Cardiac & Cardiovascular Systems
Dobromir Dobrev, Jordi Heijman, Roddy Hiram, Na Li, Stanley Nattel
Summary: This Review discusses the role of cardiomyocyte inflammatory signaling in atrial fibrillation (AF), evaluates the therapeutic potential associated with these signaling pathways, and highlights crucial questions for future research. The authors summarize the evidence for the involvement of inflammation in AF and focus on the NLRP3 inflammasome in atrial cardiomyocytes as a potential key player. They also discuss the possibility of similar signaling in non-myocyte cardiac cells and the importance of active resolution of inflammation in suppressing AF-related inflammatory signaling. The review concludes by considering the therapeutic potential and broader implications of this knowledge.
NATURE REVIEWS CARDIOLOGY
(2023)
Editorial Material
Cardiac & Cardiovascular Systems
Sandro Ninni, Stanley Nattel
CARDIOVASCULAR RESEARCH
(2023)
