Article
Multidisciplinary Sciences
Andrew E. Radbill, Lucy Y. Lei, Sachin Y. Paranjape, Daniel J. Blackwell, Robert L. Abraham, Derek S. Chew, Satish R. Raj, Bjorn C. Knollmann
Summary: This study aimed to assess rate-dependence of clinical surrogates of contractility and repolarization in humans with hypertrophic cardiomyopathy. Findings show that rapid pacing trains triggered ventricular ectopy in hypertrophic cardiomyopathy patients, but not controls. However, there were no significant differences observed in surrogate measures for cardiac contractility, suggesting that altered length-dependent myofilament activation may not have significant clinical implications in these patients.
Article
Cardiac & Cardiovascular Systems
Malene Brohus, Todor Arsov, David A. Wallace, Helene Halkjaer Jensen, Mette Nyegaard, Lia Crotti, Marcin Adamski, Yafei Zhang, Matt A. Field, Vicki Athanasopoulos, Isabelle Baro, Barbara B. Ribeiro de Oliveira-Mendes, Richard Redon, Flavien Charpentier, Hariharan Raju, Deborah DiSilvestre, Jinhong Wei, Ruiwu Wang, Haloom Rafehi, Antony Kaspi, Melanie Bahlo, Ivy E. Dick, Sui Rong Wayne Chen, Matthew C. Cook, Carola G. Vinuesa, Michael Toft Overgaard, Peter J. Schwartz
Summary: This study investigated the case of an Australian mother convicted of killing her four children, revealing a novel CALM2 gene variant in two of the children. This variant impairs calmodulin function, potentially leading to fatal arrhythmias.
Article
Cardiac & Cardiovascular Systems
Juan P. Hernandez del Rincon, Mari C. Olmo Conesa, Ana Rodriguez Serrano, Helena Garcia Pulgar, David Lopez Cuenca, Carmen Munoz Esparza, Marina Navarro Penalver, Juan Jose Santos Mateo, Elisa Nicolas Rocamora, Cristina Gil Ortuno, Maria Sabater-Molina, Juan Ramon Gimeno Blanes, Francisco Pastor Quirante
Summary: Inherited cardiovascular diseases are a major cause of sudden cardiac death, but the prevention rate remains low at 35.6%. Among resuscitated cases, female sex and channelopathies were more prevalent, with 60.4% of patients achieving good neurological recovery.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2021)
Article
Radiology, Nuclear Medicine & Medical Imaging
David M. Raffel, Thomas C. Crawford, Yong-Woon Jung, Robert A. Koeppe, Guie Gu, Jill Rothley, Kirk A. Frey
Summary: The study evaluated the performance of F-18-4F-MHPG and F-18-3F-PHPG in cardiomyopathy patients, demonstrating their robustness in quantifying regional cardiac sympathetic nerve density. Both tracers provided comparable metrics of nerve density and left ventricular denervation, with F-18-4F-MHPG showing advantages of faster liver clearance and more consistent plasma metabolism.
EUROPEAN JOURNAL OF NUCLEAR MEDICINE AND MOLECULAR IMAGING
(2022)
Review
Medicine, General & Internal
Yun Gi Kim, Suk-Kyu Oh, Ha Young Choi, Jong-Il Choi
Summary: Inherited arrhythmia is a major cause of sudden cardiac death in young people, predominantly caused by mutations in genes encoding cardiac ion channels. While genetic testing has advanced risk stratification and precise diagnosis for IA, ongoing debates remain regarding its implications.
KOREAN JOURNAL OF INTERNAL MEDICINE
(2021)
Article
Cardiac & Cardiovascular Systems
Minna Koivunen, Juho Tynkkynen, Niku Oksala, Markku Eskola, Jussi Hernesniemi
Summary: This study aimed to provide comprehensive information on the epidemiology of sudden cardiac arrests (SCA) and sudden cardiac deaths (SCD) after acute coronary syndrome. Through a retrospective study of 10,316 patients with acute coronary syndrome, it was found that the incidence of SCA and SCD was relatively high, accounting for nearly one-third of deaths due to cardiovascular causes. Patients with ST-elevation myocardial infarction and non-ST-elevation myocardial infarction had a higher incidence of SCA.
AMERICAN HEART JOURNAL
(2023)
Review
Medicine, General & Internal
Natallia Laptseva, Valentina A. Rossi, Isabella Sudano, Rahel Schwotzer, Frank Ruschitzka, Andreas J. Flammer, Firat Duru
Summary: Amyloidosis is a systemic disease characterized by extracellular deposits of insoluble amyloid in various tissues and organs. Cardiac amyloidosis is a frequent feature of the disease, causing a progressive, restrictive type of cardiomyopathy, and is associated with adverse clinical outcomes and increased mortality. This review aims to provide insights into the arrhythmic features of cardiac amyloidosis by discussing the pathogenesis, challenges in risk stratification, and management strategies for these arrhythmias.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Cardiac & Cardiovascular Systems
Andrew D. Krahn, Jacob Tfelt-Hansen, Rafik Tadros, Christian Steinberg, Christopher Semsarian, Hui-Chen Han
Summary: Inherited arrhythmia syndromes, such as long QT syndrome and Brugada syndrome, are common causes of unexplained cardiac arrest. Less obvious conditions like catecholaminergic polymorphic ventricular tachycardia, short QT syndrome, and early repolarization syndrome may also lead to sudden cardiac death. In cases where extensive testing does not reveal an underlying cause, a diagnosis of idiopathic ventricular fibrillation or short-coupled ventricular fibrillation may be given.
JACC-CLINICAL ELECTROPHYSIOLOGY
(2021)
Review
Cardiac & Cardiovascular Systems
Christopher Balfe, Rory Durand, Derek Crinion, Deirdre Ward, Richard Sheahan
Summary: This article discusses the challenges of risk stratification for patients with inherited arrhythmia syndromes (IASs). Recent guidelines recognize the importance of using the implantable loop recorder (ILR) to rule out malignant arrhythmia as a cause of syncope in certain low-risk IAS patients. In this comprehensive literature review, the available evidence for the use of ILR in IASs and relatives of sudden arrhythmic death syndrome victims is evaluated.
Article
Cardiac & Cardiovascular Systems
Nour Raad, Philip Bittihn, Marine Cacheux, Dongtak Jeong, Zeki Ilkan, Delaine Ceholski, Erik Kohlbrenner, Lu Zhang, Chen-Leng Cai, Evangelia G. Kranias, Roger J. Hajjar, Francesca Stillitano, Fadi G. Akar
Summary: This study highlights the crucial role of primary electrical remodeling caused by the hPLN(R14del) mutation in early-stage arrhythmogenic phenotype. The preferential right ventricular action potential prolongation and steep repolarization gradient at the left ventricular/right ventricular interface are key mechanisms underlying the increased arrhythmia susceptibility in R14del hearts. The unique spatiotemporal dynamics of high-frequency rotors pinned to the right ventricle contribute to their increased stability in R14del compared with wildtype hearts.
Article
Genetics & Heredity
Fergus Stafford, Neesha Krishnan, Ebony Richardson, Alexandra Butters, Sophie Hespe, Charlotte Burns, Belinda Gray, Caroline Medi, Natalie Nowak, Julia C. Isbister, Hariharan Raju, David Richmond, Mark P. Ryan, Emma S. Singer, Raymond W. Sy, Laura Yeates, Richard D. Bagnall, Christopher Semsarian, Jodie Ingles
Summary: This study evaluated the role of genomics in diagnosis and management, and found that genetic testing can increase the diagnostic yield and have clinical utility in the final diagnosis. The study also highlighted the inequalities based on ancestry and proposed a framework for prioritizing likely monogenic cases.
Review
Veterinary Sciences
Celine Brugada-Terradellas, Arnaut Hellemans, Pedro Brugada, Pascale Smets
Summary: Sudden death is a common cause of death in Western countries, with 85% of cases being cardiac in origin. Sudden cardiac death is also common in dogs and cats, but there is limited data available. Research has shown that there are similar underlying mechanisms between humans and companion animals in certain heart diseases.
VETERINARY JOURNAL
(2021)
Article
Cardiac & Cardiovascular Systems
James P. Curtain, Carly Adamson, Toru Kondo, Jawad Butt, Akshay S. Desai, Faiez Zannad, Jean L. Rouleau, Luis E. Rohde, Lars Kober, Inder S. Anand, Dirk J. van Veldhuisen, Michael R. Zile, Martin P. Lefkowitz, Scott D. Solomon, Milton Packer, Mark C. Petrie, Pardeep S. Jhund, John J. McMurray
Summary: This study examined the incidence of ventricular tachycardia (VT) and ventricular fibrillation (VF) in patients with heart failure and their relationship with mortality. The results showed that VT/VF events were uncommon but strongly associated with mortality in heart failure patients. These events seem to be markers of disease severity rather than risk of sudden death.
EUROPEAN HEART JOURNAL
(2023)
Review
Cardiac & Cardiovascular Systems
Mehul Adhaduk, Bishow Paudel, Kan Liu, Mahi Ashwath, Michael Giudici
Summary: The study found that EPS is an effective risk stratification tool in patients with cardiac sarcoidosis (CS) across all subgroups with high sensitivity and specificity, making it valuable for predicting adverse clinical outcomes.
INTERNATIONAL JOURNAL OF CARDIOLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Vincent Portero, Thomas Nicol, Svitlana Podliesna, Gerard A. Marchal, Antonius Baartscheer, Simona Casini, Rafik Tadros, Jorien L. Treur, Michael W. T. Tanck, I. Jane Cox, Fay Probert, Tertius A. Hough, Sara Falcone, Leander Beekman, Martina Muller-Nurasyid, Gabi Kastenmuller, Christian Gieger, Annette Peters, Stefan Kaab, Moritz F. Sinner, Andrew Blease, Arie O. Verkerk, Connie R. Bezzina, Paul K. Potter, Carol Ann Remme
Summary: This study identified a mouse line with a high incidence of sudden death at a young age due to a nonsense mutation in the Bcat2 gene, leading to elevated levels of branch chain amino acids. The elevated BCAA levels were found to be positively correlated with arrhythmia-related indices and calcium dysregulation in both mouse models and human cardiomyocytes. The involvement of the mTOR pathway activation was demonstrated in preventing pro-arrhythmic events induced by high BCAA concentrations, suggesting a potential link between elevated BCAAs and arrhythmia in conditions like diabetes and heart failure.
CARDIOVASCULAR RESEARCH
(2022)