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Sudden arrhythmic death syndrome

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https://www.readbyqxmd.com/read/27996317/cardiac-arrest-with-clozapine-and-olanzapine-revealing-long-qt-syndrome
#1
Edward Woloszyn, Nishant Whig, Eileen Trigoboff, Jeffery J Grace
The authors describe a rare case of "concealed" congenital Long QT Syndrome (LQTS) Type 3 in a patient with treatment resistant schizophrenia and no known personal or family history of cardiac disease. The patient in this Case Report had a hidden genetic condition revealed only following the essential administration of antipsychotics. As a result, this patient experienced an aborted cardiac arrest and a total of five episodes of ventricular tachycardia (VT) requiring cardioversion. Successful control of the VT occurred with an Automatic Internal Defibrillator (AID), judicious use of antipsychotic medications, and anti-arrhythmic medications...
December 20, 2016: Clinical Schizophrenia & related Psychoses
https://www.readbyqxmd.com/read/27992820/gja1-gene-variations-in-sudden-unexplained-nocturnal-death-syndrome-in-the-chinese-han-population
#2
Qiuping Wu, Yeda Wu, Liyong Zhang, Jinxiang Zheng, Shuangbo Tang, Jianding Cheng
Sudden unexplained nocturnal death syndrome (SUNDS) is a conundrum to both forensic pathologists and physicians, more than 80% of which the molecular pathogenesis remains unclear. Reported studies on both clinical and genetic phenotypes suggest SUNDS is related to congenital and acquired arrhythmias. Recent researches have linked the mutations of gene gap junction alpha 1 (GJA1) with arrhythmogenic cardiac disorders. In the present study, we investigate the potential correlation between GJA1 gene variations and the occurrence of SUNDS...
December 9, 2016: Forensic Science International
https://www.readbyqxmd.com/read/27965319/high-prevalence-of-early-repolarization-in-the-paediatric-relatives-of-sudden-arrhythmic-death-syndrome-victims-and-in-normal-controls
#3
Anna McCorquodale, Rachel Poulton, Jennifer Hendry, Gabrielle Norrish, Ella Field, Sarah Mead-Regan, Martin Lowe, Juan Pablo Kaski
AIMS: Elevation of the ECG J-point in the inferior and lateral leads (early repolarization) has been described in survivors of ventricular fibrillation (VF) arrest and occurs in adult first-degree relatives of sudden cardiac death (SCD) probands at a frequency significantly greater than in controls, raising the possibility that this could represent an independent risk factor in the aetiology of SCD. However, data on early repolarization in the paediatric population are lacking. This study aimed to assess the prevalence of early repolarization in paediatric first-degree relatives of sudden arrhythmic death syndrome (SADS) victims...
December 13, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/27925329/fragmented-qrs-is-a-novel-risk-factor-for-ventricular-arrhythmic-events-after-receiving-cardiac-resynchronization-therapy-in-nonischemic-cardiomyopathy
#4
Miyako Igarashi, Hiroshi Tada, Hiro Yamasaki, Kenji Kuroki, Tomoko Ishizu, Yoshihiro Seo, Takeshi Machino, Nobuyuki Murakoshi, Yukio Sekiguchi, Yuichi Noguchi, Akihiko Nogami, Kazutaka Aonuma
INTRODUCTION: A fragmented QRS (fQRS) is reported to be associated with a poor prognosis or sudden cardiac death (SCD) in patients with Brugada syndrome or ischemic heart disease. However, no studies have clarified the impact of the presence of an fQRS on SCD or ventricular arrhythmic events in patients receiving cardiac resynchronization therapy (CRT). This study aimed to clarify this point in patients with nonischemic cardiomyopathy. METHODS AND RESULTS: This study included 137 heart failure patients with nonischemic cardiomyopathy who received CRT (NYHA functional class: II/III/IV = 25/84/28)...
December 7, 2016: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/27909531/brugada-syndrome-risk-stratification-and-management
#5
REVIEW
Konstantinos P Letsas Md Fesc, Stamatis Georgopoulos Md, Konstantinos Vlachos Md, Nikolaos Karamichalakis Md, Ioannis Liatakis Md, Panagiotis Korantzopoulos Md PhD, Tong Liu Md PhD, Michael Efremidis Md, Antonios Sideris Md
The Brugada syndrome (BrS) is an arrhythmogenic disease associated with an increased risk of ventricular fibrillation and sudden cardiac death. The risk stratification and management of BrS patients, particularly of asymptomatic ones, still remains challenging. A previous history of aborted sudden cardiac death or arrhythmic syncope in the presence of spontaneous type 1 ECG pattern of BrS phenotype appear to be the most reliable predictors of future arrhythmic events. Several other ECG parameters have been proposed for risk stratification...
August 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27803673/electrophysiological-mechanisms-of-brugada-syndrome-insights-from-pre-clinical-and-clinical-studies
#6
REVIEW
Gary Tse, Tong Liu, Ka H C Li, Victoria Laxton, Yin W F Chan, Wendy Keung, Ronald A Li, Bryan P Yan
Brugada syndrome (BrS), is a primary electrical disorder predisposing affected individuals to sudden cardiac death via the development of ventricular tachycardia and fibrillation (VT/VF). Originally, BrS was linked to mutations in the SCN5A, which encodes for the cardiac Na(+) channel. To date, variants in 19 genes have been implicated in this condition, with 11, 5, 3, and 1 genes affecting the Na(+), K(+), Ca(2+), and funny currents, respectively. Diagnosis of BrS is based on ECG criteria of coved- or saddle-shaped ST segment elevation and/or T-wave inversion with or without drug challenge...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27761159/molecular-autopsy-in-victims-of-inherited-arrhythmias
#7
REVIEW
Christopher Semsarian, Jodie Ingles
Sudden cardiac death (SCD) is a rare but devastating complication of a number of underlying cardiovascular diseases. While coronary artery disease and acute myocardial infarction are the most common causes of SCD in older populations, inherited cardiac disorders comprise a substantial proportion of SCD cases aged less than 40 years. Inherited cardiac disorders include primary inherited arrhythmogenic disorders such as familial long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and inherited cardiomyopathies, most commonly hypertrophic cardiomyopathy (HCM)...
October 2016: Journal of Arrhythmia
https://www.readbyqxmd.com/read/27759343/early-repolarisation-changes-in-ecg-are-they-benign-or-malignant
#8
Oruganti Sai Satish, K Sashikanth Srivastav
Till recently, ST segment elevation in the absence of conduction abnormalities or chest pain occurring particularly in young bradycardia individuals has been considered a normal variant called early repolarisation (ER). However, recent studies suggest a more worrisome picture as patients with history of idiopathic ventricular fibrillation showed increased prevalence of ER in ECG. ER is an ECG pattern characterised by elevation of the QRS-ST junction (J point) ≥ 2 mv from baseline in the inferior (II, III, aVF) or lateral (I, aVL, V4-V6) leads manifested as QRS slurring or notching...
July 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27738063/brugada-syndrome-in-the-young-an-assessment-of-risk-factors-predicting-future-events
#9
Maria Cecilia Gonzalez Corcia, Juan Sieira, Andrea Sarkozy, Carlo de Asmundis, Gian-Battista Chierchia, Jaime Hernandez Ojeda, Gudrun Pappaert, Pedro Brugada
AIMS: To investigate the clinical characteristics, prognoses, and presence of risk factors in young patients with Brugada syndrome (BS). METHODS AND RESULTS: A consecutive cohort of 128 young BS patients (≤25 years old at diagnosis) was analysed. Eighty-eight patients (69%) were asymptomatic, whereas 40 (31%) presented with clinical manifestations of BS. Markers of prognosis and risk were identified upon comparison of these two groups. A history of malignant syncope was strong predictors of ventricular arrhythmic events...
October 13, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/27692676/sudden-death-and-cardiac-arrest-without-phenotype-the-utility-of-genetic-testing
#10
Yanushi D Wijeyeratne, Elijah R Behr
Approximately 4% of sudden cardiac deaths are unexplained [the sudden arrhythmic death syndrome (SADS)], and up to 6-10% of survivors of cardiac arrest do not have an identifiable cardiac abnormality after comprehensive clinical evaluation [idiopathic ventricular fibrillation (IVF)]. Genetic testing may be able to play a role in diagnostics and can be targeted to an underlying phenotype present in family members following clinical evaluation. Alternatively, post-mortem genetic testing (the "molecular autopsy") may diagnose the underlying cause if a clearly pathogenic rare variant is found...
August 31, 2016: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/27641793/incidence-and-predictors-of-sudden-arrhythmic-death-or-ventricular-tachyarrhythmias-after-acute-coronary-syndrome-an-asian-perspective
#11
Jo-Jo Hai, Edward Tam, Pak-Hei Chan, Chu-Pak Lau, Chung-Wah Siu, Hung-Fat Tse
BACKGROUND: Current data on the risk of sudden arrhythmic death (SAD) and ventricular tachyarrhythmias (VTs) after acute coronary syndrome (ACS) in the Asian population are limited. OBJECTIVE: The purpose of this study was to investigate the incidence and predictors of SAD or VT after ACS in a contemporary cohort of Chinese patients in the era of early revascularization. METHODS: Consecutive patients admitted to our unit for ACS from 2010 to 2015 were retrospectively reviewed...
January 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27498076/inhibition-of-late-sodium-current-attenuates-ionic-arrhythmia-mechanism-in-ventricular-myocytes-expressing-lamina-n195k-mutation
#12
Yogananda S Markandeya, Tadashi Tsubouchi, Timothy A Hacker, Matthew R Wolff, Luiz Belardinelli, Ravi C Balijepalli
BACKGROUND: Lamin A and C are nuclear filament proteins encoded by the LMNA gene. Mutations in the LMNA gene cause many congenital diseases known as laminopathies, including Emery-Dreifuss muscular dystrophy, Hutchinson-Gilford progeria syndrome, and familial dilated cardiomyopathy (DCM) with conduction disease. A missense mutation (N195K) in the A-type lamins results in familial DCM and sudden arrhythmic death. OBJECTIVE: The purpose of this study was to investigate the ion current mechanism of arrhythmia and DCM caused by the LaminA-N195K variant...
November 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27437140/monogenec-arrhythmic-syndromes-from-molecular-and-genetic-aspects-to-bedside
#13
Golukhova E Z, Gromova O I, Shomahov R A, Bulaeva N I, Bockeria L A
The abrupt cessation of effective cardiac function that is generally due to heart rhythm disorders can cause sudden and unexpected death at any age and is referred to as a syndrome called "sudden cardiac death" (SCD). Annually, about 400,000 cases of SCD occur in the United States alone. Less than 5% of the resuscitation techniques are effective. The prevalence of SCD in a population rises with age according to the prevalence of coronary artery disease, which is the most common cause of sudden cardiac arrest...
April 2016: Acta Naturae
https://www.readbyqxmd.com/read/27378596/predictors-of-ventricular-arrhythmias-and-sudden-death-in-a-qu%C3%A3-bec-cohort-with-brugada-syndrome
#14
Lena Rivard, Antoine Roux, Isabelle Nault, Jean Champagne, Jean-François Roux, Rafik Tadros, Mario Talajic, Julia Cadrin-Tourigny, Azadeh Shohoudi, Blandine Mondésert, Denis Roy, Laurent Macle, Jason Andrade, Katia Dyrda, Marc Dubuc, Peter G Guerra, Jean-François Sarrazin, Bernard Thibault, Paul Khairy
BACKGROUND: Patients with Brugada syndrome (BrS) are at risk for ventricular arrhythmias (VAs) and sudden death. Identification of high-risk individuals beyond those with syncope or resuscitated sudden death remains a major challenge. METHODS: We assessed the value of clinical, electrophysiological, and electrocardiographic (ECG) features, including depolarization and repolarization metrics, in predicting arrhythmic events and sudden death in consecutive patients with BrS diagnosed between 2002 and 2013 in Quebec, Canada...
March 31, 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/27303672/next-generation-sequencing-in-post-mortem-genetic-testing-of-young-sudden-cardiac-death-cases
#15
REVIEW
Najim Lahrouchi, Elijah R Behr, Connie R Bezzina
Sudden cardiac death (SCD) in the young (<40 years) occurs in the setting of a variety of rare inherited cardiac disorders and is a disastrous event for family members. Establishing the cause of SCD is important as it permits the pre-symptomatic identification of relatives at risk of SCD. Sudden arrhythmic death syndrome (SADS) is defined as SCD in the setting of negative autopsy findings and toxicological analysis. In such cases, reaching a diagnosis is even more challenging and post-mortem genetic testing can crucially contribute to the identification of the underlying cause of death...
2016: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/27255966/the-prevalence-and-significance-of-the-early-repolarization-pattern-in-sudden-arrhythmic-death-syndrome-families
#16
Greg Mellor, Christopher P Nelson, Claire Robb, Hariharan Raju, Yanushi Wijeyeratne, Christian Hengstenberg, Wibke Reinhard, Michael Papadakis, Sanjay Sharma, Nilesh J Samani, Elijah R Behr
BACKGROUND: The early repolarization (ER) pattern is associated with sudden death and has been shown to be heritable. Its significance when identified in families affected by sudden arrhythmic death syndrome (SADS) remains unclear. METHODS AND RESULTS: We analyzed 12-lead ECGs of 401 first-degree relatives of individuals who had died from SADS. The prevalence of ER patterns was compared with family-clustered controls. ER was more common in SADS family members than in controls (21% versus 8%; odds ratio: 5...
June 2016: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/27151341/etiology-of-sudden-death-in-sports-insights-from-a-united-kingdom-regional-registry
#17
Gherardo Finocchiaro, Michael Papadakis, Jan-Lukas Robertus, Harshil Dhutia, Alexandros Klavdios Steriotis, Maite Tome, Greg Mellor, Ahmed Merghani, Aneil Malhotra, Elijah Behr, Sanjay Sharma, Mary N Sheppard
BACKGROUND: Accurate knowledge of causes of sudden cardiac death (SCD) in athletes and its precipitating factors is necessary to establish preventative strategies. OBJECTIVES: This study investigated causes of SCD and their association with intensive physical activity in a large cohort of athletes. METHODS: Between 1994 and 2014, 357 consecutive cases of athletes who died suddenly (mean 29 ± 11 years of age, 92% males, 76% Caucasian, 69% competitive) were referred to our cardiac pathology center...
May 10, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27033637/prognostic-significance-of-fever-induced-brugada-syndrome
#18
Yuka Mizusawa, Hiroshi Morita, Arnon Adler, Ofer Havakuk, Aurélie Thollet, Philippe Maury, Dao W Wang, Kui Hong, Estelle Gandjbakhch, Frédéric Sacher, Dan Hu, Ahmad S Amin, Najim Lahrouchi, Hanno L Tan, Charles Antzelevitch, Vincent Probst, Sami Viskin, Arthur A M Wilde
BACKGROUND: In Brugada syndrome (BrS), spontaneous type 1 electrocardiogram (ECG) is an established risk marker for fatal arrhythmias whereas drug-induced type 1 ECG shows a relatively benign prognosis. No study has analyzed the prognosis of fever-induced type 1 ECG (F-type1) in a large BrS cohort. OBJECTIVES: The objectives of this study were to assess the prognosis of F-type1 in asymptomatic BrS and to compare the effects of fever and drugs on ECG parameters. METHODS: One hundred twelve patients with BrS who developed F-type1 were retrospectively enrolled...
July 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27026928/effects-of-cardioactive-drugs-on-human-induced-pluripotent-stem-cell-derived-long-qt-syndrome-cardiomyocytes
#19
Jukka Kuusela, Ville J Kujala, Anna Kiviaho, Marisa Ojala, Heikki Swan, Kimmo Kontula, Katriina Aalto-Setälä
Human induced pluripotent stem cells (hiPSC) have enabled a major step forward in pathophysiologic studies of inherited diseases and may also prove to be valuable in in vitro drug testing. Long QT syndrome (LQTS), characterized by prolonged cardiac repolarization and risk of sudden death, may be inherited or result from adverse drug effects. Using a microelectrode array platform, we investigated the effects of six different drugs on the electrophysiological characteristics of human embryonic stem cell-derived cardiomyocytes as well as hiPSC-derived cardiomyocytes from control subjects and from patients with type 1 (LQT1) and type 2 (LQT2) of LQTS...
2016: SpringerPlus
https://www.readbyqxmd.com/read/27012403/a-new-electrocardiographic-marker-of-sudden-death-in-brugada-syndrome-the-s-wave-in-lead-i
#20
RANDOMIZED CONTROLLED TRIAL
Leonardo Calò, Carla Giustetto, Annamaria Martino, Luigi Sciarra, Natascia Cerrato, Marta Marziali, Jessica Rauzino, Giulia Carlino, Ermenegildo de Ruvo, Federico Guerra, Marco Rebecchi, Chiara Lanzillo, Matteo Anselmino, Antonio Castro, Federico Turreni, Maria Penco, Massimo Volpe, Alessandro Capucci, Fiorenzo Gaita
BACKGROUND: Risk stratification in asymptomatic patients remains by far the most important yet unresolved clinical problem in the Brugada syndrome (BrS). OBJECTIVES: This study sought to analyze the usefulness of electrocardiographic parameters as markers of sudden cardiac death (SCD) in BrS. METHODS: This study analyzed data from 347 consecutive patients (78.4% male; mean age 45 ± 13.1 years) with spontaneous type 1 BrS by ECG parameters but with no history of cardiac arrest (including 91...
March 29, 2016: Journal of the American College of Cardiology
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