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Congenital cardiac

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https://www.readbyqxmd.com/read/28723882/altered-carnitine-homeostasis-in-children-with-increased-pulmonary-blood-flow-due-to-ventricular-septal-defects
#1
Stephen M Black, Aida Field-Ridley, Shruti Sharma, Sanjiv Kumar, Roberta L Keller, Rebecca Kameny, Emin Maltepe, Sanjeev A Datar, Jeffrey R Fineman
OBJECTIVES: Congenital heart disease with increased pulmonary blood flow results in progressive pulmonary vascular endothelial dysfunction and associated increased perioperative morbidity. Using our ovine model of congenital heart disease with increased pulmonary blood flow, we have previously demonstrated progressive endothelial dysfunction associated with disruption in carnitine homeostasis, mitochondrial dysfunction, decreased nitric oxide signaling, and enhanced reactive oxygen species generation...
July 18, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28722171/use-of-antibiotics-during-pregnancy-and-the-risk-of-major-congenital-malformations-a-population-based-cohort-study
#2
Flory T Muanda, Odile Sheehy, Anick Bérard
INTRODUCTION: Few studies have investigated the link between individual antibiotics and major congenital malformations (MCMs) including specific malformations owing to small sample size. We aimed to quantify the association between exposure to gestational antibiotic and the risk of MCMs. METHODS: Using the Quebec pregnancy cohort (1998 -2008), we included a total of 139,938 liveborn singleton alive whose mothers were covered by the «Régie de l'assurance maladie du Québec" drug plan for at least 12 months before and during pregnancy...
July 19, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28720136/bilateral-multiple-pulmonary-artery-aneurysms-associated-with-cavitary-pulmonary-tuberculosis-a-case-report
#3
Pedro Pallangyo, Frederick Lyimo, Smita Bhalia, Hilda Makungu, Bashir Nyangasa, Flora Lwakatare, Pal Suranyi, Mohamed Janabi
BACKGROUND: Pulmonary artery aneurysms constitute <1% of aneurysms occurring in the thoracic cavity. Congenital cardiac defects are responsible for the majority (>50%) of cases, however, pulmonary artery aneurysm is a rare sequelae of pulmonary tuberculosis reported in about 5% of patients with chronic cavitary tuberculosis on autopsy. The natural history of this potentially fatal condition remains poorly understood and guidelines for optimal management are controversial. CASE PRESENTATION: A 24-year-old man, a nursing student of African descent, was referred to us from an up-country regional hospital with a 4-week history of recurrent episodes of breathlessness, awareness of heartbeats and coughing blood 3 weeks after completing a 6-month course of anti-tuberculosis drugs...
July 19, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28720027/long-term-alteration-of-the-hypothalamic-pituitary-adrenal-axis-in-children-undergoing-cardiac-surgery-in-the-first-6-months-of-life
#4
Monica McGauran, Brigid Jordan, Roseriet Beijers, Irma Janssen, Candice Franich-Ray, Carolina de Weerth, Michael Cheung
Children with congenital heart disease (CHD) have poorer neurodevelopmental and psychological outcomes. The mechanisms underlying this remain unclear. One mechanism could be that the stressful experience of cardiac surgery early in life influences long-term hypothalamic-pituitary-adrenal (HPA) axis regulation. Dysregulation of the HPA axis has been linked to poorer neurocognitive and psychological outcomes in other study populations. This case-control study aims to compare HPA-axis regulation (circadian rhythm and reactivity) using salivary cortisol in 3- to 5-year-olds with CHD who did and did not have cardiac surgery prior to 6 months of age...
July 18, 2017: Stress: the International Journal on the Biology of Stress
https://www.readbyqxmd.com/read/28719142/changing-prevalence-of-severe-congenital-heart-disease-results-from-the-national-register-for-congenital-heart-defects-in-germany
#5
Constanze Pfitzer, Paul C Helm, Hannah Ferentzi, Lisa-Maria Rosenthal, Ulrike M M Bauer, Felix Berger, Katharina R L Schmitt
OBJECTIVE: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender. DESIGN: Cross-sectional registry study. SETTING: We analyzed data from patients with CHD born between 1996 and 2015. PATIENTS: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic capability for earlier identifying minor lesions)...
July 18, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28719069/inaccuracy-of-a-continuous-arterial-pressure-waveform-monitor-when-used-for-congenital-cardiac-catheterization
#6
Michael D Seckeler, Katri Typpo, Jendar Deschenes, Ruth Higgins, Ricardo Samson, Peter Lichtenthal
OBJECTIVE: To determine the accuracy of a continuous cardiac output monitor (FloTrac sensor) for measuring cardiac index in children with congenital heart disease undergoing cardiac catheterization. Cardiac index is a critical hemodynamic parameter measured during catheterizations in children with congenital heart disease. This has been challenging to measure accurately and many clinicians rely on predictive equations for calculating cardiac index. DESIGN: Prospective, nonrandomized trial...
July 18, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28716623/a-second-truncation-in-ttn-causes-early-onset-recessive-muscular-dystrophy
#7
Elizabeth Harris, Ana Töpf, Anna Vihola, Anni Evilä, Rita Barresi, Judith Hudson, Peter Hackman, Brian Herron, Daniel MacArthur, Hanns Lochmüller, Kate Bushby, Bjarne Udd, Volker Straub
Mutations in the gene encoding the giant skeletal muscle protein titin are associated with a variety of muscle disorders, including recessive congenital myopathies ±cardiomyopathy, limb girdle muscular dystrophy (LGMD) and late onset dominant distal myopathy. Heterozygous truncating mutations have also been linked to dilated cardiomyopathy. The phenotypic spectrum of titinopathies is emerging and expanding, as next generation sequencing techniques make this large gene amenable to sequencing. We undertook whole exome sequencing in four individuals with LGMD...
June 22, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28716564/endothelial-to-mesenchymal-transition-in-the-cardiovascular-system
#8
REVIEW
Hui Gong, Xing Lu, Qiong Wang, Min Hu, Xiangyu Zhang
Endothelial to mesenchymal transition (EndMT) is a special type of epithelial to mesenchymal transition. It is a process that is characterized by the loss of features of endothelial cells and acquisition of specific markers of mesenchymal cells. A variety of stimuli, such as inflammation, growth factors, and hypoxia, regulate EndMT through various signaling pathways and intracellular transcription factors. It has been demonstrated that epigenetic modifications are also involved in this process. Recent studies have identified the essential role of EndMT in the cardiovascular system...
July 14, 2017: Life Sciences
https://www.readbyqxmd.com/read/28713984/downregulation-of-microrna%C3%A2-199a%C3%A2-5p-protects-cardiomyocytes-in-cyanotic-congenital-heart-disease-by-attenuating-endoplasmic-reticulum-stress
#9
Yang Zhou, Wei-Kun Jia, Zhao Jian, Liang Zhao, Chen-Cheng Liu, Yong Wang, Ying-Bin Xiao
Chronic hypoxia is a key pathological change in patients with cyanotic congenital heart defect (CCHD). It has been demonstrated that enhanced myocardial unfolded protein response (UPR) increases the capacity to buffer endoplasmic reticulum (ER) stress and to avoid subsequent apoptosis caused by the hypoxia that underlies CCHD. The present study was performed to determine the regulatory role of microRNAs (miRNAs) in this cytoprotective UPR process. The results revealed that miR‑199a‑5p was markedly downregulated in the cardiac tissue of patients with CCHD and in human myocardial cells cultured in hypoxic conditions...
July 6, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28711963/transfer-of-neonates-with-critical-congenital-heart-disease-within-a-regionalized-network
#10
Michael F Swartz, Jill M Cholette, Jennifer M Orie, Marshall L Jacobs, Jeffrey P Jacobs, George M Alfieris
Regionalization of pediatric cardiac surgical care varies between and within states. In most geographic regions, at least some neonates with critical heart disease are transferred from their birth hospital to a different hospital for surgery. The impact of neonatal transfer for surgery, particularly over a considerable distance (>10 miles), has been largely unexplored. We sought to examine the impact of transferring neonates for cardiac surgery. We queried the New York State Cardiac Surgery database (2005-2014) from a single institution to identify neonates born within the cardiac surgery center and those transferred for surgery...
July 15, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28711608/do-anabolic-androgenic-steroids-have-performance-enhancing-effects-in-female-athletes
#11
REVIEW
Grace Huang, Shehzad Basaria
Doping with anabolic androgenic steroids (AAS) is common among both male and female athletes and is a growing public health problem. Review of historical data of systematic state-sponsored doping programs implemented by the German Democratic Republic in elite female athletes and from the clinical trials of testosterone administration in non-athlete women suggests that AAS have ergogenic effects in women. The use of AAS in female athletes has been associated with adverse effects that include acne, hirsutism, deepening of the voice and menstrual disturbances; life-threatening adverse effects such as cardiac arrhythmias and sudden death have also been reported...
July 12, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28711156/towards-a-structural-view-of-drug-binding-to-herg-k-channels
#12
REVIEW
Jamie I Vandenberg, Eduardo Perozo, Toby W Allen
The human ether-a-go-go-related gene (hERG) K(+) channel is of great medical and pharmaceutical relevance. Inherited mutations in hERG result in congenital long-QT syndrome which is associated with a markedly increased risk of cardiac arrhythmia and sudden death. hERG K(+) channels are also remarkably susceptible to block by a wide range of drugs, which in turn can cause drug-induced long-QT syndrome and an increased risk of sudden death. The recent determination of the near-atomic resolution structure of the hERG K(+) channel, using single-particle cryo-electron microscopy (cryo-EM), provides tremendous insights into how these channels work...
July 12, 2017: Trends in Pharmacological Sciences
https://www.readbyqxmd.com/read/28711067/pa-6-inhibits-inward-rectifier-currents-carried-by-v93i-and-d172n-gain-of-function-kir2-1-channels-but-increases-channel-protein-expression
#13
Yuan Ji, Marlieke G Veldhuis, Jantien Zandvoort, Fee L Romunde, Marien J C Houtman, Karen Duran, Gijs van Haaften, Eva-Maria Zangerl-Plessl, Hiroki Takanari, Anna Stary-Weinzinger, Marcel A G van der Heyden
BACKGROUND: The inward rectifier potassium current IK1 contributes to a stable resting membrane potential and phase 3 repolarization of the cardiac action potential. KCNJ2 gain-of-function mutations V93I and D172N associate with increased IK1, short QT syndrome type 3 and congenital atrial fibrillation. Pentamidine-Analogue 6 (PA-6) is an efficient (IC50 = 14 nM with inside-out patch clamp methodology) and specific IK1 inhibitor that interacts with the cytoplasmic pore region of the KIR2...
July 15, 2017: Journal of Biomedical Science
https://www.readbyqxmd.com/read/28710297/association-of-surgeon-age-and-experience-with-congenital-heart-surgery-outcomes
#14
Brett R Anderson, Amelia S Wallace, Kevin D Hill, Brian C Gulack, Roland Matsouaka, Jeffrey P Jacobs, Emile A Bacha, Sherry A Glied, Marshall L Jacobs
BACKGROUND: Surgeon experience concerns both families of children with congenital heart disease and medical providers. Relationships between surgeon seniority and patient outcomes are often assumed, yet there are little data. METHODS AND RESULTS: This national study used linked data from the American Medical Association Physician Masterfile and the Society of Thoracic Surgeons-Congenital Heart Surgery Database to examine associations between surgeon years since medical school and major morbidity/mortality for children undergoing cardiac surgery...
July 2017: Circulation. Cardiovascular Quality and Outcomes
https://www.readbyqxmd.com/read/28709919/morbidity-after-cardiac-surgery-in-patients-with-adult-congenital-heart-disease-in-comparison-with-acquired-disease
#15
Dimos Karangelis, Amine Mazine, Sreekanth Narsupalli, Shamarli Mendis, Gruschen Veldtman, Nicolas Nikolaidis
BACKGROUND: Due to the advancements in congenital cardiac surgery and interventional cardiology in the last five decades, more than 85% of congenital heart patients now survive to adulthood. METHODS: This retrospective study included 135 Adult Congenital Heart Disease (ACHD) patients, who had cardiac surgery at Southampton General Hospital over three consecutive years. We also included 42 patients with a structurally normal heart who had cardiac surgery for acquired cardiac conditions as a control group...
June 28, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28709918/unroofed-coronary-sinus-syndrome-an-easily-corrected-congenital-anomaly-but-more-diagnostic-suspicions-are-needed
#16
Changcheng Chen, Lili Xu, Yi Xu, Ping Li, Shuo Liu, Bin You
BACKGROUND: Unroofed coronary sinus syndrome (URCSS) is a spectrum of cardiac anomalies in which part (partial type) or the entire common wall (complete type) between the coronary sinus (CS) and left atrium is absent. It is commonly associated with a persistent left superior vena cava (PLSVC). The PLSVC can even anomalously connect to the left atrium in complete type anomaly. URCSS has been reported to be associated with delayed diagnosis and life-threatening cerebral injury. The purpose of the present study was to review our experience with surgical correction of this often-concealed malformation and discuss methods for reducing diagnostic omission...
June 22, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28709760/clinical-and-pathologic-implications-of-extending-the-spectrum-of-maternal-autoantibodies-reactive-with-ribonucleoproteins-associated-with-cutaneous-and-now-cardiac-neonatal-lupus-from-ssa-ro-and-ssb-la-to-u1rnp
#17
REVIEW
Peter M Izmirly, Marc K Halushka, Avi Z Rosenberg, Sean Whelton, Khodayar Rais-Bahrami, Dilip S Nath, Hilary Parton, Robert M Clancy, Sara Rasmussen, Amit Saxena, Jill P Buyon
While the relationship between maternal connective tissue diseases and neonatal rashes was described in the 1960s and congenital heart block in the 1970s, the "culprit" antibody reactivity to the SSA/Ro-SSB/La ribonucleoprotein complex was not identified until the 1980s. However, studies have shown that approximately 10-15% of cases of congenital heart block are not exposed to anti-SSA/Ro-SSB/La. Whether those cases represent a different disease entity or whether another antibody is associated has yet to be determined...
July 11, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28707420/transthoracic-intracardiac-catheters-in-pediatric-cardiac-patients-a-single-center-experience
#18
Kristoffer Beham, Hitendu Dave, Janet Kelly, Bernhard Frey, Maja I Hug, Barbara Brotschi
BACKGROUND: Transthoracic intracardiac catheters are frequently inserted in children during congenital heart surgery for monitoring and vascular access purposes. Their use entails a small potential risk. AIM: We aimed to evaluate both catheter-associated morbidities related to maintenance and removal of transthoracic intracardiac catheters in pediatric cardiac patients, and predictors for catheter-associated adverse events. METHODS: Single-center retrospective cohort study of prospectively collected data of children aged 0-14 years receiving a transthoracic intracardiac catheter inserted in the operating room during 7 consecutive years at the University Children's Hospital Zurich...
July 14, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28707365/use-of-3-d-digital-subtraction-rotational-angiography-during-cardiac-catheterization-of-infants-and-adults-with-congenital-heart-diseases
#19
Sushitha Surendran, B Rush Waller, Lucas Elijovich, Vijaykumar Agrawal, Andrew Kuhls-Gilcrist, Jason Johnson, Thomas Fagan, Shyam K Sathanandam
OBJECTIVE: To compare image quality, radiation and contrast doses required to obtain 3D-Digital subtraction rotational angiography (3D-DSRA) with 3D-Digital rotational angiography (3D-DRA) in infants (children ≤ 2 years of age) and adults with congenital heart diseases (ACHD). BACKGROUND: 3D-DRA can be performed with radiation doses comparable to bi-plane cine-angiography. However, 3D-DRA in infants requires a large contrast volume. The resolution of 3D-DRA performed in ACHD patients is limited by their soft tissue density...
July 14, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28707262/exercise-n-13-cardiac-positron-emission-tomography-myocardial-perfusion-imaging-detecting-ischemia-in-an-adult-patient-with-anomalous-aortic-origin-of-the-left-main-coronary-artery-from-the-right-coronary-sinus
#20
Matthew R Summers, Hani Najm, James L Gentry Iii, Wael A Jaber
Anomalous aortic origin of a coronary artery is a rare congenital condition that has variable presentations from atypical chest pain to syncope and cardiac arrest. Commonly used myocardial perfusion imaging techniques, stress agents, and perfusion agents may have limited ability to detect inducible ischemia in this rare patient group. We herein describe a unique case of anomalous left main coronary artery from a common right coronary sinus ostium with a subpulmonic and intramyocardial course. This patient had multiple atypical chest pain presentations and multiple-negative pharmacologic single-photon emission-computed tomography stress tests performed...
July 13, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
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