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https://www.readbyqxmd.com/read/28550094/safety-of-outpatient-initiation-of-disopyramide-for-obstructive-hypertrophic-cardiomyopathy-patients
#1
Arnon Adler, Dana Fourey, Adaya Weissler-Snir, Waseem Hindieh, Raymond H Chan, Michael H Gollob, Harry Rakowski
BACKGROUND: Disopyramide is effective in ameliorating symptoms in patients with hypertrophic cardiomyopathy; however, its potential for proarrhythmic effect has raised concerns about its use in the ambulatory setting. The risk of initiating disopyramide in this manner has never been evaluated. METHODS AND RESULTS: All charts of patients seen in the outpatient hypertrophic cardiomyopathy clinic between 2010 and 2014 were screened for initiation of disopyramide and data were extracted...
May 26, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28549832/sudden-cardiac-death-secondary-to-severe-multivessel-coronary-spasm
#2
Paula Sánchez-Somonte, Eduardo Josué Flores-Umanzor, Marta Sitges Carreño
No abstract text is available yet for this article.
May 23, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28549245/analysis-of-heart-rate-variability-and-risk-factors-for-sudep-in-patients-with-drug-resistant-epilepsy
#3
Leyla Baysal-Kirac, Nail Güven Serbest, Erdi Şahin, Hava Özlem Dede, Candan Gürses, Ayşen Gökyiğit, Nerses Bebek, Ahmet Kaya Bilge, Betül Baykan
BACKGROUND: Cardiac problems have been suggested as causes of sudden unexpected death in epilepsy (SUDEP). Our aim was to investigate possible associations of cardiac autonomic functions based on heart rate variability (HRV) parameters with risk factors of SUDEP in patients with drug-resistant epilepsy. METHODS: Forty-seven patients with drug-resistant seizures and 45 healthy control subjects were enrolled in the study. Interictal time domain parameters of HRV were evaluated with 24-hour Holter recordings...
May 23, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28549118/an-unexpected-cause-of-angina-and-survived-sudden-cardiac-death
#4
Matthias P Nägele, Tardu Özkartal, Corinna Brunckhorst, Laurent Haegeli
No abstract text is available yet for this article.
May 26, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28546535/a-novel-prkag2-mutation-in-a-chinese-family-with-cardiac-hypertrophy-and-ventricular-pre-excitation
#5
Kun-Qi Yang, Chao-Xia Lu, Ying Zhang, Yan-Kun Yang, Jia-Cheng Li, Tian Lan, Xu Meng, Peng Fan, Tao Tian, Lin-Ping Wang, Ya-Xin Liu, Xue Zhang, Xian-Liang Zhou
PRKAG2 syndrome is a rare autosomal dominant inherited disorder that is characterized by cardiac hypertrophy, ventricular pre-excitation and conduction system abnormalities. There is little knowledge in cardiovascular magnetic resonance (CMR) characteristics of PRKAG2 cardiomyopathy. This study investigated the genetic defect in a three-generation Chinese family with cardiac hypertrophy and ventricular pre-excitation using whole-exome sequencing. A novel missense mutation, c.1006 G > T (p.V336L), was identified in PRKAG2...
May 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28545855/-a-complex-case-of-diabetes-due-to-lmna-mutation
#6
C Ambonville, M-A Bouldouyre, P Laforêt, P Richard, O Benveniste, C Vigouroux
INTRODUCTION: Laminopathies (diseases related to A/C mutations of lamines) are rare genetic diseases with an extensive phenotypic spectrum, including lipodystrophic syndromes-characterized by a selective loss of adipose tissue-of which the partial Dunnigan family type is the most frequent. CASE REPORT: We report on a 55-year-old woman with diabetes and long-term disabling myalgia. Her cushingoid morphotype, associated with cutaneous lipo-atrophy and muscle hypertrophy in addition to a genetic heritage, led us to the diagnosis of complex partial familial lipodystrophy heterozygous LMNA_c...
May 22, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28545633/seattle-heart-failure-and-proportional-risk%C3%A2-models-predict-benefit-from-implantable%C3%A2-cardioverter-defibrillators
#7
Kenneth C Bilchick, Yongfei Wang, Alan Cheng, Jeptha P Curtis, Kumar Dharmarajan, George J Stukenborg, Ramin Shadman, Inder Anand, Lars H Lund, Ulf Dahlström, Ulrik Sartipy, Aldo Maggioni, Karl Swedberg, Chris O'Conner, Wayne C Levy
BACKGROUND: Recent clinical trials highlight the need for better models to identify patients at higher risk of sudden death. OBJECTIVES: The authors hypothesized that the Seattle Heart Failure Model (SHFM) for overall survival and the Seattle Proportional Risk Model (SPRM) for proportional risk of sudden death, including death from ventricular arrhythmias, would predict the survival benefit with an implantable cardioverter-defibrillator (ICD). METHODS: Patients with primary prevention ICDs from the National Cardiovascular Data Registry (NCDR) were compared with control patients with heart failure (HF) without ICDs with respect to 5-year survival using multivariable Cox proportional hazards regression...
May 30, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28544662/tracheal-replacement-therapy-with-a-stem-cell-seeded-graft-lessons-from-compassionate-use-application-of-a-gmp-compliant-tissue-engineered-medicine
#8
Martin J Elliott, Colin R Butler, Aikaterini Varanou-Jenkins, Leanne Partington, Carla Carvalho, Edward Samuel, Claire Crowley, Peggy Lange, Nicholas J Hamilton, Robert E Hynds, Tahera Ansari, Paul Sibbons, Anja Fierens, Claire McLaren, Derek Roebuck, Colin Wallis, Nagarajan Muthialu, Richard Hewitt, David Crabbe, Sam M Janes, Paolo De Coppi, Mark W Lowdell, Martin A Birchall
Tracheal replacement for the treatment of end-stage airway disease remains an elusive goal. The use of tissue-engineered tracheae in compassionate use cases suggests that such an approach is a viable option. Here, a stem cell-seeded, decellularized tissue-engineered tracheal graft was used on a compassionate basis for a girl with critical tracheal stenosis after conventional reconstructive techniques failed. The graft represents the first cell-seeded tracheal graft manufactured to full good manufacturing practice (GMP) standards...
June 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28542808/ascending-aortic-rupture-through-a-penetrating-atherosclerotic-ulcer-a-rare-cause-of-sudden-unexpected-death
#9
Supawon Srettabunjong
Spontaneous rupture of the aorta through an atherosclerotic lesion without preexisting aortic aneurysm, dissection, or history of trauma is very rare. Without prompt aortic repair, all cases result in sudden death with a definitive diagnosis made only intraoperatively or during autopsy. The phenomenon has been uniformly found in individuals with hypertension. The author reports a sudden unexpected death caused by spontaneous rupture of the ascending aorta in a 57-year-old man with a history of hypertension...
May 23, 2017: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/28539571/do-implantable-cardioverter-defibrillators-reduce-mortality-in-patients-with-chronic-kidney-disease-at-all-stages
#10
Linghua Fu, Qiongqiong Zhou, Wengen Zhu, Huang Lin, Ying Ding, Yang Shen, Jinzhu Hu, Kui Hong
The benefits of implantable cardioverter defibrillator (ICD) implantation in chronic kidney disease (CKD) patients with high sudden cardiac death (SCD) risk are uncertain. To clarify the effects of receiving an ICD in CKD patients, we conducted this meta-analysis to identify the effects of ICDs on patients with CKD, including those on dialysis. We searched the Cochrane library, EMBASE, PubMed, and clinical trials for studies published before July 2016. Eleven studies including 20,196 CKD patients were considered for inclusion...
May 23, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28538763/myosin-binding-protein-c-compound-heterozygous-variant-effect-on-the-phenotypic-expression-of-hypertrophic-cardiomyopathy
#11
Julianny Freitas Rafael, Fernando Eugênio Dos Santos Cruz, Antônio Carlos Campos de Carvalho, Ilan Gottlieb, José Guilherme Cazelli, Ana Paula Siciliano, Glauber Monteiro Dias
Hypertrophic cardiomyopathy (HCM) is an autosomal dominant genetic disease caused by mutations in genes encoding sarcomere proteins. It is the major cause of sudden cardiac death in young high-level athletes. Studies have demonstrated a poorer prognosis when associated with specific mutations. The association between HCM genotype and phenotype has been the subject of several studies since the discovery of the genetic nature of the disease. This study shows the effect of a MYBPC3 compound variant on the phenotypic HCM expression...
April 2017: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28538416/case-report-of-2-sudden-deaths-after-surgery-for-bone-fracture-usefulness-of-immunohistochemical-analysis-of-coronary-artery-for-identifying-acute-myocardial-infarction
#12
Atsushi Kurata, Jun Nishida, Takashi Koyama, Tamotsu Miki, Hirotsugu Hashimoto, Kengo Yamamoto, Masahiko Kuroda
RATIONALE: Death following orthopedic surgery has become rare, but does occur. Acute myocardial infarction (AMI) can be a cause of such death, but diagnosis of AMI is often challenging, even by autopsy. PATIENT CONCERNS: We have recently experienced 2 cases of sudden death after bone fracture surgery, in which AMI and pulmonary thromboembolism were clinically suspected as causes of death. Case 1 was a 60-year-old male with a history of diabetes mellitus who died 7 days after surgery for Lisfranc dislocation fracture...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28536896/helical-distribution-of-hypertrophy-in-patients-with-hypertrophic-cardiomyopathy-prevalence-and-clinical-implications
#13
Dafne Viliani, Eduardo Pozo, Norma Aguirre, Alberto Cecconi, María J Olivera, Paloma Caballero, Luis J Jiménez-Borreguero, Fernando Alfonso
Recently a novel pattern of helical distribution of hypertrophy has been described in patients with hypertrophic cardiomyopathy (HCM). Our aim was to determine its prevalence and potential implications in an unselected cohort. One-hundred- and eight consecutive patients diagnosed with HCM by cardiac magnetic resonance (CMR) were included (median clinical follow up of 1718 days). All clinical and complementary test information was prospectively collected. The presence of a helical pattern was assessed by a simple measurement of the maximal left ventricle (LV) wall thickness (LVWT) for each of the 17 classical LV segments and it was classified in one of three types according to its extension...
May 23, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28536837/controlled-sedation-with-midazolam-and-analgesia-with-nalbuphine-to-alleviate-pain-in-patients-undergoing-subcutaneous-implantable-cardioverter-defibrillator-implantation
#14
Michaël Peyrol, Jérémie Barraud, Jennifer Cautela, Baptiste Maille, Marc Laine, Laurent Bonello, Franck Thuny, Franck Paganelli, Frédéric Franceschi, Linda Koutbi, Samuel Levy
PURPOSE: Subcutaneous implantable cardioverter defibrillator (S-ICD) is an alternative to transvenous ICD to prevent sudden cardiac death. Subcutaneous ICD implantation frequently requires general anesthesia because of procedure nociceptive steps during creation of a large device pocket and lead tunneling. This study aims to determine if a strategy of operator-guided controlled sedation with midazolam and analgesia with nalbuphine is effective in alleviating pain during S-ICD implantation...
May 23, 2017: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/28536684/gastrointestinal-cytomegalovirus-disease-in-a-patient-with-pemphigus-vulgaris-treated-with-corticosteroid-and-mycophenolate-mofetil
#15
Luiza Barbosa Oliveira, Celina Wakisaka Maruta, Denise Miyamoto, Fernanda Aburesi Salvadori, Claudia Giuli Santi, Valeria Aoki, Amaro Nunes Duarte-Neto
Pemphigus vulgaris is an autoimmune disease characterized by the formation of suprabasal intra-epidermal blisters on the skin and mucosal surfaces. Infectious diseases are the main cause of death in patients with pemphigus due to the disrupture of the physiological skin barrier, immune dysregulation, and the use of immunosuppressive medications leaving the patient prone to acquire opportunistic infections. We report the case of a 67-year-old woman diagnosed with pemphigus vulgaris, who was irregularly taking prednisone and mycophenolate mofetil...
January 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/28536668/brugada-syndrome-in-a-patient-with-vascular-ehlers-danlos-syndrome-sudden-death-risk-amplified
#16
Jason D'Souza, Divyanshu Malhotra, Aditya Goud, Chanukya Dahagam, George Everett
The vast majority of sudden cardiac arrests occur in patients with structural heart disease and in approximately 10% of the cases, it can occur in those with structurally normal hearts. Brugada syndrome is an autosomal dominant sodium channelopathy that has been implicated in sudden deaths. Given their low prevalence, our knowledge about Brugada syndrome is still evolving. Apart from schizophrenia, there have been no reports of associated medical conditions. We recently encountered a patient with vascular Ehlers-Danlos syndrome who was also found to have Brugada syndrome...
April 19, 2017: Curēus
https://www.readbyqxmd.com/read/28535530/mathematical-model-of-potassium-profiling-in-chronic-dialysis
#17
Mauro Ursino, Gabriele Donati
Potassium balance is a difficult task in hemodialysis: low potassium in the dialysate is associated with a high risk of sudden cardiac death, whereas excessive dialysate potassium may provoke insufficient removal and hyperkalemia. A better understanding of the problem can be achieved with the use of mathematical models of solute kinetics. This study is aimed at presenting an improved model of solute kinetics and fluid shifts during hemodialysis. It comprises a 2-compartment (intracellular and extracellular) description of sodium, potassium, and urea, including volume fluid shifts induced by osmotic forces...
2017: Contributions to Nephrology
https://www.readbyqxmd.com/read/28534967/whole%C3%A2-exome-sequencing-identifies-a-novel-mutation-r367g-in-scn5a-to-be-associated-with-familial-cardiac-conduction-disease
#18
Rong Yu, Xue-Feng Fan, Chan Chen, Zheng-Hua Liu
Cardiac conduction disease is a primary cause of sudden cardiac death. Sodium voltage‑gated channel‑α subunit 5 (SCN5A) mutations have been reported to underlie a variety of inherited arrhythmias. Numerous disease‑causing mutations of SCN5A have been identified in patients with ≥10 different conditions, including type 3 long‑QT syndrome and Brugada syndrome. The present study investigated a family with a history of arrhythmia, with the proband having a history of arrhythmia and syncope. Whole‑exome sequencing was applied in order to detect the disease‑causing mutation in this family, and Sanger sequencing was used to confirm the co‑segregation among the family members...
May 17, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28533990/anomalous-origin-of-right-coronary-artery-originating-from-the-pulmonary-trunk-arcapa-an-incidental-finding-in-a-patient-presenting-with-chest-pain
#19
Pragathi Balakrishna, Michael Illovsky, Youssef M Al-Saghir, Abdul M Minhas
Anomalous origin of the right coronary artery originating from the pulmonary trunk (ARCAPA) is a rare congenital coronary anomaly with an estimated prevalence of 0.002%. Most patients are asymptomatic and the anomaly is detected incidentally during evaluation for other problems. Occasionally, ARCAPA may lead to myocardial ischemia and/or sudden cardiac arrest. We present a case of a 55-year-old female with a history of hypertension who presented to the emergency department with intermittent chest discomfort for three days...
April 17, 2017: Curēus
https://www.readbyqxmd.com/read/28533740/correlation-of-ventricular-arrhythmogenesis-with-neuronal-remodeling-of-cardiac-postganglionic-parasympathetic-neurons-in-the-late-stage-of-heart-failure-after-myocardial-infarction
#20
Dongze Zhang, Huiyin Tu, Chaojun Wang, Liang Cao, Robert L Muelleman, Michael C Wadman, Yu-Long Li
Introduction: Ventricular arrhythmia is a major cause of sudden cardiac death in patients with chronic heart failure (CHF). Our recent study demonstrates that N-type Ca(2+) currents in intracardiac ganglionic neurons are reduced in the late stage of CHF rats. Rat intracardiac ganglia are divided into the atrioventricular ganglion (AVG) and sinoatrial ganglion. Only AVG nerve terminals innervate the ventricular myocardium. In this study, we tested the correlation of electrical remodeling in AVG neurons with ventricular arrhythmogenesis in CHF rats...
2017: Frontiers in Neuroscience
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