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Congenital heart diseases

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https://www.readbyqxmd.com/read/28820561/quadricuspid-aortic-valve-associated-with-aortic-regurgitation-mitral-regurgitation-and-aortic-dilatation
#1
Serkan Sivri, Serdal Bastug, Murat Can Guney, Yakup Alsancak, Elcin Ozdemir, Engin Bozkurt
The quadricuspid aortic valve (QAV) is a very uncommon congenital malformation with an estimated incidence of 0.003% to 0.043% of all congenital heart diseases. Combinations of QAV with several different congenital malformations have been described. The case is reported of a type A QAV associated with moderate aortic regurgitation, mild mitral regurgitation, and ascending aorta dilatation. This interesting case was referred for close follow up.
March 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28819713/plasma-growth-differentiation-factor-15-is-a-potential-biomarker-for-pediatric-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#2
Gang Li, Yan Li, Xiao-Qiu Tan, Peng Jia, Jian Zhao, Dong Liu, Ting Wang, Bin Liu
We aimed to investigate plasma growth differentiation factor-15 (GDF-15) levels in pediatric pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD), and assess the association with hemodynamic parameters. Plasma GDF-15 levels were measured in children with PAH-CHD (n = 46) and compared to children with CHD without PAH (n = 39). Normal individuals (n = 30) served as health control group. Plasma GDF-15 levels were significantly elevated in patients with PAH-CHD compared with those with CHD without PAH (median 1415 ng/L, interquartile range [IQR] 926...
August 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28819628/abnormal-biomarkers-of-homocysteine-metabolism-in-neonates-with-conotruncal-heart-defects
#3
Piotr Surmiak, Małgorzata Baumert, Magdalena Paprotny
OBJECTIVES: The etiology of conotruncal heart defects (CHD) remains unknown; however relation between homocysteine, folate levels, and congenital heart disease was found. With this perspective in mind, the aim of the study was to investigate biomarkers of homosyteine metabolism pathway in mothers and their neonates with CHD. MATERIAL AND METHODS: Forty-three pairs of mothers and their neonates with CHD and forty pairs of mothers and neonates with nonconotruncal heart defects (non-CHD) were enrolled...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28811286/acute-arrhythmias-in-adults-with-congenital-heart-disease
#4
Christopher J McLeod
No abstract text is available yet for this article.
September 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28811093/risk-factors-for-infective-endocarditis-in-children-with-congenital-heart-diseases-a-nationwide-population-based-case-control-study
#5
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/28810576/application-of-low-dose-dual-source-computed-tomography-angiography-in-children-with-complex-congenital-heart-disease
#6
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
#7
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
#8
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
#9
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
#10
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/28807008/phenotypic-and-molecular-characterisation-of-cdk13-related-congenital-heart-defects-dysmorphic-facial-features-and-intellectual-developmental-disorders
#11
Bret L Bostwick, Scott McLean, Jennifer E Posey, Haley E Streff, Karen W Gripp, Alyssa Blesson, Nina Powell-Hamilton, Jessica Tusi, David A Stevenson, Ellyn Farrelly, Louanne Hudgins, Yaping Yang, Fan Xia, Xia Wang, Pengfei Liu, Magdalena Walkiewicz, Marianne McGuire, Dorothy K Grange, Marisa V Andrews, Marybeth Hummel, Suneeta Madan-Khetarpal, Elena Infante, Zeynep Coban-Akdemir, Karol Miszalski-Jamka, John L Jefferies, Jill A Rosenfeld, Lisa Emrick, Kimberly M Nugent, James R Lupski, John W Belmont, Brendan Lee, Seema R Lalani
BACKGROUND: De novo missense variants in CDK13 have been described as the cause of syndromic congenital heart defects in seven individuals ascertained from a large congenital cardiovascular malformations cohort. We aimed to further define the phenotypic and molecular spectrum of this newly described disorder. METHODS: To minimise ascertainment bias, we recruited nine additional individuals with CDK13 pathogenic variants from clinical and research exome laboratory sequencing cohorts...
August 14, 2017: Genome Medicine
https://www.readbyqxmd.com/read/28806996/improved-passive-catheter-tracking-with-positive-contrast-for-cmr-guided-cardiac-catheterization-using-partial-saturation-psat
#12
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
#13
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/28799175/low-age-low-birth-weight-and-congenital-heart-disease-are-risk-factors-for-intensive-care-in-infants-with-bronchiolitis
#14
Minna Mecklin, Paula Heikkilä, Matti Korppi
AIM: This study evaluated the incidence and risk factors for intensive care and respiratory support in infant bronchiolitis. METHODS: This retrospective descriptive case-control study focused on 105 patients treated in the paediatric intensive care unit (PICU) and 210 controls treated in the emergency department or on the paediatric ward in Tampere University Hospital in Finland between 2000-2015. Statistically significant risk factors in non-adjusted analyses were included in the adjusted logistic regression...
August 11, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28797356/prevalence-of-atrial-fibrillation-and-permanent-atrial-arrhythmias-in-congenital-heart-disease-sequel-or-series
#15
EDITORIAL
Michael J Silka, Yaniv Bar-Cohen
No abstract text is available yet for this article.
August 15, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28797355/increasing-prevalence-of-atrial-fibrillation-and-permanent-atrial-arrhythmias-in-congenital-heart-disease
#16
Fabien Labombarda, Robert Hamilton, Azadeh Shohoudi, Jamil Aboulhosn, Craig S Broberg, Marie A Chaix, Scott Cohen, Stephen Cook, Annie Dore, Susan M Fernandes, Anne Fournier, Joseph Kay, Laurent Macle, Blandine Mondésert, François-Pierre Mongeon, Alexander R Opotowsky, Anna Proietti, Lena Rivard, Jennifer Ting, Bernard Thibault, Ali Zaidi, Paul Khairy
BACKGROUND: Atrial arrhythmias are the most common complication encountered in the growing and aging population with congenital heart disease. OBJECTIVES: This study sought to assess the types and patterns of atrial arrhythmias, associated factors, and age-related trends. METHODS: A multicenter cohort study enrolled 482 patients with congenital heart disease and atrial arrhythmias, age 32.0 ± 18.0 years, 45.2% female, from 12 North American centers...
August 15, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28794993/limitations-of-galactose-therapy-in-phosphoglucomutase-1-deficiency
#17
Kristine Nolting, Julien H Park, Laura C Tegtmeyer, Andrea Zühlsdorf, Marianne Grüneberg, Stephan Rust, Janine Reunert, Ingrid Du Chesne, Volker Debus, Eric Schulze-Bahr, Robert C Baxter, Yoshinao Wada, Christian Thiel, Emile van Schaftingen, Ralph Fingerhut, Thorsten Marquardt
INTRODUCTION: Phosphoglucomutase 1 deficiency (PGM1 deficiency) has been identified as both, glycogenosis and congenital disorder of glycosylation (CDG). The phenotype includes hepatopathy, myopathy, oropharyngeal malformations, heart disease and growth retardation. Oral galactose supplementation at a dosage of 1 g per kg body weight per day is regarded as the therapy of choice. RESULTS: We report on a patient with a novel disease causing mutation, who was treated for 1...
December 2017: Molecular Genetics and Metabolism Reports
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
#18
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/28794135/pulmonary-vasodilator-therapy-is-associated-with-greater-survival-in-eisenmenger-syndrome
#19
Clare Arnott, Geoff Strange, Andrew Bullock, Adrienne C Kirby, Clare O'Donnell, Dorothy J Radford, Leeanne E Grigg, David S Celermajer
OBJECTIVE: Eisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT). METHODS: From January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand...
August 9, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28790229/autoimmune-disease-with-cardiac-valves-involvement-libman-sacks-endocarditis
#20
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
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