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https://www.readbyqxmd.com/read/28811695/pai-1-study-in-thalassemia-major-patients-receiving-multiple-blood-transfusion
#1
Ashwini Kumar, H S Batra, Mithu Banerjee, S Bandyopadhyay, T K Saha, Pratibha Misra, Vivek Ambade
Thalassemia is a congenital hemolytic disease which is treated by repeated blood transfusion. Chronic iron overload is currently considered to be the primary cause of mortality in β-thalassemia, mainly due to the induction of left-sided cardiac failure. Iron overload results from a number of mechanisms associated with the disease itself. In addition to chronic iron overload thalassemic patients are more prone for procoagulant status which in turn lead to clinical thrombotic events. The hypercoagulable state in thalassemia is due to multiple elements, a combination of which is often the drive behind a clinical thromboembolic events...
July 2017: Indian Journal of Clinical Biochemistry: IJCB
https://www.readbyqxmd.com/read/28811093/risk-factors-for-infective-endocarditis-in-children-with-congenital-heart-diseases-a-nationwide-population-based-case-control-study
#2
Li-Chuan Sun, Chih-Cheng Lai, Cheng-Yi Wang, Ya-Hui Wang, Jen-Yu Wang, Yo-Ling Hsu, Yin-Lan Hu, En-Ting Wu, Ming-Tai Lin, Leticia B Sy, Likwang Chen
BACKGROUND: Infective endocarditis (IE) is uncommon in childhood. Its associated epidemiological characteristics in patients with congenital heart disease (CHD) remain unclear. METHODS: The study population included children born in Taiwan during the years 1997 to 2005 who were diagnosed as having CHD before 3years of age. All children were followed up until the end year of 2010, the diagnosis of infective endocarditis, or death. The demographic characteristics of patients with and without IE, the invasive procedures performed during 6months before the index date, the prophylactic antibiotics related to dental procedures, and in-hospital mortality were collected...
August 12, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28810874/sudden-cardiac-death-focus-on-the-genetics-of-channelopathies-and-cardiomyopathies
#3
REVIEW
Simona Magi, Vincenzo Lariccia, Marta Maiolino, Salvatore Amoroso, Santo Gratteri
Sudden cardiac death (SCD) describes a natural and unexpected death from cardiac causes occurring within a short period of time (generally within 1 h of symptom onset) in the absence of any other potentially lethal condition. Most SCD-related diseases have a genetic basis; in particular congenital cardiac channelopathies and cardiomyopathies have been described as leading causes of SCD. Congenital cardiac channelopathies are primary electric disorders caused by mutations affecting genes encoding cardiac ion channels or associated proteins, whereas cardiomyopathies are related to mutations in genes encoding several categories of proteins, including those of sarcomeres, desmosomes, the cytoskeleton, and the nuclear envelope...
August 15, 2017: Journal of Biomedical Science
https://www.readbyqxmd.com/read/28810576/application-of-low-dose-dual-source-computed-tomography-angiography-in-children-with-complex-congenital-heart-disease
#4
Xian-Feng Chen, Fan Jiang, Lin Li, Yan Chen, Xin Chen, Yan-Yan Jiang, Li Xiang, Xiao-Jing Ma
The objective of the present study was to evaluate image quality and radiation dosage using a low-dose prospectively electrocardiogram (ECG)-gated computed tomography (CT) protocol for dual-source angiography in children with complex congenital heart disease. A total of 206 patients with complex congenital heart disease were equally assigned into two groups at random. The children in group A underwent low-dose retrospective ECG-gated CT scanning with an ECG-pulsing technique, and group B underwent prospective ECG-gated scanning with an ECG-pulsing technique...
August 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28809063/color-and-power-doppler-combined-with-fetal-intelligent-navigation-echocardiography-fine-to-evaluate-the-fetal-heart
#5
L Yeo, R Romero
OBJECTIVE: To evaluate the performance of color and bidirectional power Doppler ultrasound combined with Fetal Intelligent Navigation Echocardiography (FINE) in examining the fetal heart. METHODS: A prospective cohort study was conducted of fetuses in the second and third trimesters with a normal heart or with congenital heart disease (CHD). One or more spatiotemporal image correlation (STIC) volume datasets, combined with color or bidirectional power Doppler (S-flow) imaging, were acquired in the apical four-chamber view...
August 14, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28807900/heart-morphogenesis-gene-regulatory-networks-revealed-by-temporal-expression-analysis
#6
Jonathon T Hill, Bradley Demarest, Megan Smith, Bushra Gorsi, H Joseph Yost
During embryogenesis, the heart forms as a linear tube that then undergoes multiple simultaneous morphogenetic events to obtain its mature shape. To understand the gene regulatory networks (GRNs) driving this phase of heart development, during which many congenital heart disease malformations likely arise, we conducted an RNA-seq time course in zebrafish from 30 hpf to 72 hpf and identified 5,861 genes with altered expression. We then clustered the genes by temporal expression pattern, identified transcription factor binding motifs enriched in each cluster, and generated a model GRN for the major gene batteries in heart morphogenesis...
August 14, 2017: Development
https://www.readbyqxmd.com/read/28807509/-18-f-fdg-pet-ct-angiography-in-the-diagnosis-of-infective-endocarditis-and-cardiac-device-infection-in-adult-patients-with-congenital-heart-disease-and-prosthetic-material
#7
María N Pizzi, L Dos-Subirà, Albert Roque, Nuria Fernández-Hidalgo, Hug Cuéllar-Calabria, Antonia Pijuan Domènech, María T Gonzàlez-Alujas, M T Subirana-Domènech, B Miranda-Barrio, Ignacio Ferreira-González, Juan J González-López, Albert Igual, Olga Maisterra-Santos, David García-Dorado, Joan Castell-Conesa, Benito Almirante, Manuel Escobar Amores, Pilar Tornos, Santiago Aguadé-Bruix
OBJECTIVES: Infective endocarditis (IE) and cardiac device infection (CDI) are a major complication in the growing number of patients with congenital heart disease (CHD) reaching adulthood. We aimed to evaluate the added value of (18)F-FDG-PET/CT angiography (PET/CTA) in the diagnosis of IE-CDI in adults with CHD and intravascular or intracardiac prosthetic material, in whom echocardiography (ECHO) and modified Duke Criteria (DC) have limitations because of the patients' complex anatomy...
August 9, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28807423/coping-with-the-stress-in-the-cardiac-intensive-care-unit-can-mindfulness-be-the-answer
#8
Nadya Golfenshtein, Janet A Deatrick, Amy J Lisanty, Barbara Medoff-Cooper
BACKGROUND: Mothers of infants with complex congenital heart disease are exposed to increased stress which has been associated with numerous adverse health outcomes. The coping mechanisms these mothers use critically effect the familial illness adaptation and most likely infant outcomes. Currently no data-based strategies have been developed for mothers to facilitate their coping, and proactively promote their adaptation in the critical care settings. A potential strategy is mindfulness which is currently used in other clinical settings with stress-reduction effects...
August 11, 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28806996/improved-passive-catheter-tracking-with-positive-contrast-for-cmr-guided-cardiac-catheterization-using-partial-saturation-psat
#9
Mari Nieves Velasco Forte, Kuberan Pushparajah, Tobias Schaeffter, Israel Valverde Perez, Kawal Rhode, Bram Ruijsink, Mazen Alhrishy, Nicholas Byrne, Amedeo Chiribiri, Tevfik Ismail, Tarique Hussain, Reza Razavi, Sébastien Roujol
BACKGROUND: Cardiac catheterization is a common procedure in patients with congenital heart disease (CHD). Although cardiovascular magnetic resonance imaging (CMR) represents a promising alternative approach to fluoroscopy guidance, simultaneous high contrast visualization of catheter, soft tissue and the blood pool remains challenging. In this study, a novel passive tracking technique is proposed for enhanced positive contrast visualization of gadolinium-filled balloon catheters using partial saturation (pSAT) magnetization preparation...
August 15, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28803248/a-functional-assay-for-sick-sinus-syndrome-genetic-variants
#10
Chuanchau J Jou, Cammon B Arrington, Spencer Barnett, Jiaxiang Shen, Scott Cho, Xiaoming Sheng, Patrick C McCullagh, Neil E Bowles, Chase M Pribble, Elizabeth V Saarel, Thomas A Pilcher, Susan P Etheridge, Martin Tristani-Firouzi
BACKGROUND/AIMS: Congenital Sick Sinus Syndrome (SSS) is a disorder associated with sudden cardiac death due to severe bradycardia and prolonged pauses. Mutations in HCN4, the gene encoding inward Na+/K+ current (If), have been described as a cause of congenital SSS. The objective of this study is to develop an SSS model in embryonic zebrafish, and use zebrafish as a moderate-throughput assay to functionally characterize HCN4 variants. METHODS: To determine the function of hcn4 in zebrafish, embryos were either bathed in the If -specific blocker (ZD-7288), or endogenous hcn4 expression was knocked down using splice-blocking morpholinos...
August 11, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28795028/neonatal-patent-ductus-arteriosus-ligation-operations-performed-by-adult-cardiac-surgeons
#11
Yoon Sang Chung, Dai Yun Cho, Hyun Kang, Na Mi Lee, Joonhwa Hong
BACKGROUND: Patent ductus arteriosus (PDA) ligation is usually performed by congenital cardiac surgeons. However, due to the uneven distribution of congenital cardiac surgeons in South Korea, many institutions depend solely on adult cardiac surgeons for congenital cardiac diseases. We report the outcomes of PDA ligations performed by adult cardiac surgeons at our institution. METHODS: The electronic medical records of 852 neonates at Chung-Ang University Hospital, Seoul, South Korea from November 2010 to May 2014 were reviewed to identify patients with PDA...
August 2017: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28794526/effect-of-ketamine-on-pro-and-anti-inflammatory-cytokine-response-in-paediatric-cardiac-surgery-a-prospective-randomised-controlled-study
#12
Tamer Hamed Ibrahim, Hassan Saad Abdelrahman, Mohammed A Alharbi, Ibrahim A Zabani, Mohamed Fouad Ismail, Heba Kary
BACKGROUND AND AIMS: Paediatric cardiac surgery with cardiopulmonary bypass (CPB) is associated with a marked inflammatory response and triggers release of inflammatory cytokines. The aim of this study was to study the effect of ketamine on the inflammatory response during correction of congenital cyanotic heart diseases. METHODS: Sixty-six patients with congenital cyanotic heart diseases scheduled for cardiac surgery were randomised into three groups. Group A patients did not receive ketamine (control group), Group B patients received 2 mg/kg ketamine intravenous (IV) and Group C patients received ketamine 2 mg/kg IV and an IV infusion of ketamine (50 μg/kg/min)...
July 2017: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/28790229/autoimmune-disease-with-cardiac-valves-involvement-libman-sacks-endocarditis
#13
Eka Ginanjar, Yulianto Yulianto
This case study aim to evaluate the response of steroid treatment for autoimmune endocarditis. Valvular heart disease is relatively rising in both congenital and acquired cases, but the autoimmune endocarditis remains rare. In this case, a 34 year old woman with clinical manifestation resembling systemic lupus erythematosus (SLE) is diagnosed with Libman-sacks Endocarditis. After six months of steroid treatment, her clinical manifestations and heart structure improved.
April 2017: Acta Medica Indonesiana
https://www.readbyqxmd.com/read/28780716/epigenetics-a-potential-key-mechanism-involved-in-the-pathogenesis-of-cardiorenal-syndromes
#14
REVIEW
Grazia Maria Virzì, Anna Clementi, Alessandra Brocca, Massimo de Cal, Claudio Ronco
Epigenetics is defined as the heritable changes in gene expression patterns which are not directly encoded by modifications in the nucleotide DNA sequence of the genome, including higher order chromatin organization, DNA methylation, cytosine modifications, covalent histone tail modifications, and short non-coding RNA molecules. Recently, much attention has been paid to the role and the function of epigenetics and epimutations in the cellular and subcellular pathways and in the regulation of genes in the setting of both kidney and cardiovascular disease...
August 5, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/28779863/neurological-complications-of-cardiac-disease
#15
Nandini Madan, Karen S Carvalho
This article focuses on the complex interactions between the cardiovascular and neurologic systems. Initially, we focus on neurological complications in children with congenital heart disease both secondary to the underlying cardiac disease and complications of interventions. We later discuss diagnosis and management of common syncope syndromes with emphasis on vasovagal syncope. We also review the diagnosis, classification, and management of children and adolescents with postural orthostatic tachycardia syndrome...
February 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28774644/preincision-initiation-of-dexmedetomidine-maximally-reduces-the-risk-of-junctional-ectopic-tachycardia-in-children-undergoing-ventricular-septal-defect-repairs
#16
Nischal K Gautam, Yuliya Turiy, Chandra Srinivasan
OBJECTIVE: To evaluate whether initiation of dexmedetomidine (DEX) infusion before surgical incision and cardiopulmonary bypass (CPB) versus initiation after CPB had an impact on the incidence of junctional ectopic tachycardia (JET). DESIGN: Retrospective cohort study. SETTING: Single tertiary-care cardiac center. PARTICIPANTS: Children undergoing cardiopulmonary bypass for repair of congenital heart disease involving ventricular septal defects between January 2010 and February 2013...
April 10, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28774381/first-in-human-closed-chest-transcatheter-superior-cavopulmonary-anastomosis
#17
Kanishka Ratnayaka, John W Moore, Rodrigo Rios, Robert J Lederman, Sanjeet R Hegde, Howaida G El-Said
BACKGROUND: In the care of patients with congenital heart disease, percutaneous interventional treatments have supplanted many surgical approaches for simple lesions, such as atrial septal defect. By contrast, complex congenital heart defects continue to require open-heart surgery. In single-ventricle patients, a staged approach is employed, which requires multiple open-heart surgeries and significant attendant morbidity and mortality. A nonsurgical transcatheter alternative would be attractive...
August 8, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28770972/prophylactic-levosimendan-for-the-prevention-of-low-cardiac-output-syndrome-and-mortality-in-paediatric-patients-undergoing-surgery-for-congenital-heart-disease
#18
REVIEW
Johanna Hummel, Gerta Rücker, Brigitte Stiller
BACKGROUND: Low cardiac output syndrome remains a serious complication, and accounts for substantial morbidity and mortality in the postoperative course of paediatric patients undergoing surgery for congenital heart disease. Standard prophylactic and therapeutic strategies for low cardiac output syndrome are based mainly on catecholamines, which are effective drugs, but have considerable side effects. Levosimendan, a calcium sensitiser, enhances the myocardial function by generating more energy-efficient myocardial contractility than achieved via adrenergic stimulation with catecholamines...
August 2, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28766834/transseptal-puncture-using-surgical-electrocautery-in-children-and-adults-with-and-without-complex-congenital-heart-disease
#19
Srinath T Gowda, Athar M Qureshi, Daniel Turner, Nitin Madan, Justin Weigand, Richard Lorber, Harinder R Singh
BACKGROUND: Atrial transseptal puncture (TSP) for cardiac catheterization procedures remain challenging in children and adults with complex congenital heart disease (CHD). OBJECTIVES: We sought to evaluate our experience using radiofrequency (RF) current via surgical electrocautery needle for TSP to facilitate diagnostic and interventional procedures. METHODS: Retrospective chart review of all patients (pts) who underwent TSP using RF energy (10-25 W) via surgical electrocautery from three centers from January 2011 to January 2017 were evaluated...
August 2, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28766718/left-ventricular-aneurysm-sudden-unexpected-deaths-in-a-29-year-old-man
#20
Supawon Srettabunjong
Left ventricular aneurysm (LVA) is an abnormal dilated heart structure, either congenital or acquired. LVA is a rare cardiac condition with no symptoms in most cases, thus occasionally diagnosed during investigations of other diseases. Its association with certain cardiac complications and sudden cardiac deaths has been reported. However, its role as a cause of sudden unexpected death is rare. The author reported a sudden cardiac death in a 29-year-old man with LVA. Without a significant coronary artery disease and known etiologies of LVA, such an abnormal heart structure in the present case was considered congenital LVA...
August 2, 2017: Journal of Forensic Sciences
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