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https://www.readbyqxmd.com/read/27909533/patient-specific-induced-pluripotent-stem-cell-derived-cardiomyocytes-for-drug-development-and-screening-in-catecholaminergic-polymorphic-ventricular-tachycardia
#1
REVIEW
Ben Jehuda Ronen, Barad Lili
Catecholaminergic polymorphic ventricular tachycardia (CPVT), an inherited arrhythmia often leading to sudden cardiac death in children and young adults, is characterized by polymorphic/bidirectional ventricular tachycardia induced by adrenergic stimulation associated with emotionally stress or physical exercise. There are two forms of CPVT: 1. CPVT1 is caused by mutations in the RYR2 gene, encoding for ryanodine receptor type 2. CPVT1 is the most common form of CPVT in the population, and is inherited by a dominant mechanism...
August 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27892886/safety-profile-and-utility-of-treadmill-exercise-in-patients-with-high-gradient-hypertrophic-cardiomyopathy
#2
Lars Lindholm Sorensen, Hsin-Yueh Liang, Aurelio Pinheiro, Alex Hilser, Veronica Dimaano, Niels Thue Olsen, Thomas Fritz Hansen, Peter Sogaard, Alexandra Nowbar, Chiara Pisanello, Iraklis Pozios, Susan Phillip, Xun Zhou, Roselle Abraham, Theodore P Abraham
: Exercise echocardiography in the evaluation of hypertrophic cardiomyopathy (HCM) provides valuable information for risk stratification, selection of optimal treatment, and prognostication. However, HCM patients with left ventricular outflow tract gradients ≥30mm Hg are often excluded from exercise testing because of safety considerations. We examined the safety and utility of exercise testing in patients with high-gradient HCM. METHODS: We evaluated clinical characteristics, hemodynamics, and imaging variables in 499 consecutive patients with HCM who performed 959 exercise tests...
October 20, 2016: American Heart Journal
https://www.readbyqxmd.com/read/27882733/anomalous-origin-of-the-right-coronary-artery-first-familial-cases-in-asia
#3
Hideki Motomura, Mari Yokokawa, Hirofumi Fukunaga, Mari Nakagaki, Tomoyuki Hasuwa, Hiroyuki Moriuchi
Congenital coronary artery abnormalities may cause sudden death, particularly in athletes. Two siblings, aged 10 and 9 years, respectively, were diagnosed with anomalous origin of the right coronary artery on multi-detector computed tomography (MDCT). The right coronary artery arose from the left coronary cusp, and was wedged between the aorta and pulmonary artery. This was also noted on cardiac ultrasonography (UCG), but in general this might not be seen on electrocardiography at rest. Although the surgical indications are unclear in the case of unproven ischemia, early recognition of the condition may reduce risk of the cardiac events during exercise...
November 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27869041/wolff-parkinson-white-syndrome-a-single-exercise-stress-test-might-be-misleading
#4
Arash Salavitabar, Eric S Silver, Leonardo Liberman
Risk stratification of patients with Wolff-Parkinson-White syndrome for sudden death is a complex process, particularly in understanding the utility of the repeat exercise stress test. We report a case of an 18-year-old patient who was found to have a high-risk pathway by both invasive and exercise stress testing after an initial exercise stress test showing beat-to-beat loss of pre-excitation.
November 21, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27861184/catecholaminergic-polymorphic-ventricular-tachycardia-a-model-for-genotype-specific-therapy
#5
Thomas M Roston, Filip Van Petegem, Shubhayan Sanatani
PURPOSE OF REVIEW: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a life-threatening syndrome defined by exercise-induced or emotion-induced ventricular arrhythmias, typically caused by gain-of-function mutations in RYR2-encoded ryanodine receptor-2 (RyR2). This review will discuss recent advances and ongoing challenges in devising genotype-specific CPVT therapies. RECENT FINDINGS: CPVT patients were once universally thought to be at high risk of sudden death; however, as more cases emerge, CPVT is being re-defined as a complex syndrome of variable expressivity...
January 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/27856464/longitudinal-association-among-sickle-cell-trait-fitness-and-cardiovascular-disease-risk-factors-in-african-americans-in-cardia
#6
Robert I Liem, Cheeling Chan, Thanh-Huyen T Vu, Myriam Fornage, Alexis A Thompson, Kiang Liu, Mercedes R Carnethon
The contribution of sickle cell trait (SCT) to racial disparities in cardiopulmonary fitness is not known despite concerns that SCT is associated with exertion-related sudden death. We evaluated the association of SCT status with cross-sectional and longitudinal changes in fitness and risk of hypertension, diabetes and metabolic syndrome over 25 years among 1,995 African Americans (56% women, 18 to 30 years old) in the Coronary Artery Risk Development in Young Adults (CARDIA) study. Overall, the prevalence of SCT was 6...
November 16, 2016: Blood
https://www.readbyqxmd.com/read/27826330/management-of-patients-with-long-qt-syndrome
#7
REVIEW
Yongkeun Cho
Long QT syndrome (LQTS) is a rare cardiac channelopathy associated with syncope and sudden death due to torsades de pointes and ventricular fibrillation. Syncope and sudden death are frequently associated with physical and emotional stress. Management of patients with LQTS consists of life-style modification, β-blockers, left cardiac sympathetic denervation (LCSD), and implantable cardioverter-defibrillator (ICD) implantation. Prohibition of competitive exercise and avoidance of QT-prolonging drugs are important issues in life-style modification...
November 2016: Korean Circulation Journal
https://www.readbyqxmd.com/read/27805707/assessment-of-indirect-inflammatory-markers-in-patients-with-myocardial-bridging
#8
Levent Cerit
INTRODUCTION: Myocardial bridging (MB) is a congenital variant of the coronary artery in which a portion of the epicardial coronary artery takes an intramuscular course. Although it is considered a benign anomaly, it may lead to such complications as myocardial ischaemia, acute coronary syndrome, coronary spasm, exercise-induced dysrhythmias or even sudden death. MB may be related to increased inflammatory and atherosclerotic processes. This study was conducted with the aim of evaluating the relationship between neutrophil/lymphocyte ratio (NLR) and MB...
August 19, 2016: Cardiovascular Journal of Africa
https://www.readbyqxmd.com/read/27801691/sudden-cardiac-death-in-transposition-of-the-great-arteries-with-a-mustard-or-senning-baffle-the-myocardial-ischemia-hypothesis
#9
Paul Khairy
PURPOSE OF REVIEW: The literature on sudden death in transposition of the great arteries (D-TGA) with atrial switch surgery is reviewed and a pathophysiological mechanism is proposed. RECENT FINDINGS: Over 80% of sudden deaths in patients with D-TGA and Mustard or Senning baffles occur during exercise. Factors most consistently associated with ventricular arrhythmias and sudden death include heart failure parameters and atrial arrhythmias. Atrial arrhythmias have been observed to trigger malignant ventricular arrhythmias...
January 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/27798055/exercise-induced-syncope-in-a-22-year-old-man
#10
Colin Yeo, Vern Hsen Tan, Kelvin Ck Wong
A 22-year-old man was referred to us for syncope during a game of Captain's ball. There was no prodrome. His friends did not notice any ictal movements. He was otherwise well prior to passing out. He was not taking any medications or supplements. He was not usually physically active, but was otherwise well with no significant medical history. This is his first episode of syncope. There was no history of cardiac arrest or seizures. There is no family history of premature sudden cardiac death.Physical examination was normal...
October 25, 2016: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/27792707/-hypertrophic-miocardiopathy-an-historical-and-anatomopathological-review
#11
Manlio F Márquez, Teresita de Jesús Ruíz-Siller, Rosario Méndez-Ramos, Erick Karabut, Alberto Aranda-Fraustro, Silvia Jiménez-Becerra
Hypertrophic cardiomyopathy (HCM) is characterized by the presence of an abnormal hypertrophy of the left ventricle (LV), without dilation, and in the absence of any condition or another cardiac or systemic disease capable of inducing such hypertrophy. This primary or idiopathic hypertrophy can occur with or without dynamic obstruction (induced by exercise) of the LV outflow tract, so in its natural history two fundamental aspects are highlighted: the production of symptoms by blocking the LV outflow tract and the occurrence of sudden cardiac death secondary to ventricular arrhythmias...
September 2016: Gaceta Médica de México
https://www.readbyqxmd.com/read/27788932/causes-and-predictors-of-death-in-patients-with-coronary-heart-disease-from-the-heart-and-soul-study
#12
Elizabeth Y Wang, Jeffrey Dixson, Nelson B Schiller, Mary A Whooley
Although the prevalence of coronary heart disease (CHD) in the United States has increased during the past 25 years, cardiovascular mortality has decreased due to advances in CHD therapy and prevention. We sought to determine the proportion of patients with CHD who die from cardiovascular versus noncardiovascular causes and the causes and predictors of death, in a cohort of patients with CHD. The Heart and Soul Study enrolled 1,024 participants with stable CHD from 2000 to 2002 and followed them for 10 years...
September 29, 2016: American Journal of Cardiology
https://www.readbyqxmd.com/read/27770771/evaluation-of-efficacy-safety-and-tolerability-of-ambrisentan-in-chinese-adults-with-pulmonary-arterial-hypertension-a-prospective-open-label-cohort-study
#13
Y Huo, Z C Jing, X F Zeng, J M Liu, Z X Yu, G C Zhang, Y Li, Y Wang, Q S Ji, P Zhu, B X Wu, Y Zheng, P P Wang, J Li
BACKGROUND: Although several new drugs have been approved in recent years, pulmonary arterial hypertension (PAH) remains a rapidly progressive disease with a poor prognosis. Ambrisentan, a selective endothelin type A antagonist, has been approved for treatment of PAH. This open label study assessed the efficacy and safety of ambrisentan in Chinese subjects with PAH. METHODS: Eligible patients with PAH (World Health Organisation [WHO] functional class [FC] II orIII) were enrolled and received Ambrisentan (5 mg) once daily for a 12-week preliminary evaluation period, and a 12-week dose-adjustment period (dose titration to 10 mgallowed)...
October 22, 2016: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/27761157/current-topics-in-catecholaminergic-polymorphic-ventricular-tachycardia
#14
Naokata Sumitomo
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by emotions or exercise in patients without organic heart disease and may be polymorphic or bidirectional in nature. The prognosis of CPVT is not good, and therefore prevention of sudden death is of utmost importance. Genetic variants of CPVT include RyR2, CASQ2, CALM2, TRD, and possibly KCNJ2 and ANK2 gene mutations. Hypotheses that suggest the causes of CPVT include weakened binding of FKBP12.6 and RyR2, a store overload-induced Ca(2+) release (SOICR), unzipping of intramolecular domain interactions in RyR2, and molecular and functional abnormalities caused by mutations in the CASQ2 gene...
October 2016: Journal of Arrhythmia
https://www.readbyqxmd.com/read/27751241/decision-analysis-to-define-the-optimal-management-of-athletes-with-anomalous-aortic-origin-of-a-coronary-artery
#15
Carlos M Mery, Keila N Lopez, Silvana Molossi, S Kristen Sexson-Tejtel, Rajesh Krishnamurthy, E Dean McKenzie, Charles D Fraser, Scott B Cantor
OBJECTIVES: The goal of this study was to use decision analysis to evaluate the impact of varying uncertainties on the outcomes of patients with anomalous aortic origin of a coronary artery. METHODS: Two separate decision analysis models were created: one for anomalous left coronary artery (ALCA) and one for anomalous right coronary artery (ARCA). Three strategies were compared: observation, exercise restriction, and surgery. Probabilities and health utilities were estimated on the basis of existing literature...
November 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27720455/myocardial-fibrosis-in-athletes
#16
REVIEW
Freek R van de Schoor, Vincent L Aengevaeren, Maria T E Hopman, David L Oxborough, Keith P George, Paul D Thompson, Thijs M H Eijsvogels
Myocardial fibrosis (MF) is a common phenomenon in the late stages of diverse cardiac diseases and is a predictive factor for sudden cardiac death. Myocardial fibrosis detected by magnetic resonance imaging has also been reported in athletes. Regular exercise improves cardiovascular health, but there may be a limit of benefit in the exercise dose-response relationship. Intense exercise training could induce pathologic cardiac remodeling, ultimately leading to MF, but the clinical implications of MF in athletes are unknown...
November 2016: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/27719691/a-survey-of-paediatricians-on-the-use-of-electrocardiogram-for-pre-participation-sports-screening
#17
Angira Patel, Gregory Webster, Kendra Ward, John Lantos
: Aim The aim of the present study was to determine general paediatrician knowledge, practices, and attitudes towards electrocardiogram (ECG) screening in school athletes during pre-participation screening exam (PPSE). METHODS: Paediatricians affiliated with a tertiary children's hospital completed a survey about ECGs for PPSE. RESULTS: In total, 205/498 (41%) responded; 92% of the paediatricians did not include an ECG as part of PPSE; 56% were aware of a case in which a student athlete in their own community had died of sudden unexplained death; 4% had an athlete in their practice die...
October 10, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27707537/exercise-in-patients-with-hypertrophic-cardiomyopathy-a-review-of-current-evidence-national-guideline-recommendations-and-a-proposal-for-a-new-direction-to-fitness
#18
Waseem Hindieh, Arnon Adler, Adaya Weissler-Snir, Dana Fourey, Sarah Harris, Harry Rakowski
Hypertrophic cardiomyopathy is a common genetic disorder with a prevalence of 1:500 in the general population. Amongst a varied spectrum of clinical presentations, the most feared complication of this cardiac disorder is sudden cardiac death. Although only a minority of patients with hypertrophic cardiomyopathy who suffer sudden cardiac death or resuscitated cardiac arrest do so during exercise, strenuous physical activity is regarded as an important trigger for these tragic outcomes. Furthermore, during exercise, patients with hypertrophic cardiomyopathy may develop augmentation of left ventricular outflow tract obstruction, myocardial ischemia, diastolic dysfunction and/or inappropriate vasodilation in non-exercising vascular beds...
September 20, 2016: Journal of Science and Medicine in Sport
https://www.readbyqxmd.com/read/27692227/exercise-prescription-for-the-athlete-with-cardiomyopathy
#19
REVIEW
Sara Saberi, Sharlene M Day
Inherited cardiomyopathies have highly variable expression in terms of symptoms, functional limitations, and disease severity. Associated risk of sudden cardiac death is also variable. International guidelines currently recommend restriction of all athletes with cardiomyopathy from participation in competitive sports. While the guidelines are necessarily conservative because predictive risk factors for exercise-triggered SCD have not been clearly identified, the risk is clearly not uniform across all athletes and all sports...
November 2016: Cardiology Clinics
https://www.readbyqxmd.com/read/27692223/using-the-12-lead-electrocardiogram-in-the-care-of-athletic-patients
#20
REVIEW
Tee Joo Yeo, Sanjay Sharma
This article summarizes the role of the 12-lead electrocardiogram (ECG) for the clinical care of athletes, with particular reference to the influence of age, gender, ethnicity, and type of sport on the appearance of the ECG, and its role in differentiating physiologic exercise-related changes from pathologic conditions implicated in sudden cardiac death (SCD). The article also explores the potential role of the ECG in detecting athletes at risk of SCD. In addition, the article reviews the evolution of ECG interpretation criteria and emphasizes the limitations of the ECG as well as the potential for future research...
November 2016: Cardiology Clinics
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