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Congenital heart defect

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https://www.readbyqxmd.com/read/27931195/prediction-of-spontaneous-closure-of-isolated-ventricular-septal-defects-in-utero-and-postnatal-life
#1
Xing Li, Gui-Xian Song, Li-Jie Wu, Yu-Mei Chen, Yi Fan, Yun Wu, Ya-Hui Shen, Li Cao, Ling-Mei Qian
BACKGROUND: Ventricular septal defect (VSD) is a highly prevalent fetal congenital heart defect, which can become spontaneously closed during infancy. The current study aims to characterize fetal VSDs that were subsequently spontaneously closed in the first 2 years of life in eastern China. METHODS: Between January 2011 and December 2013, 257 fetal patients diagnosed with isolated VSD by fetal echocardiography at Nanjing Maternity and Child Health Care Hospital, China, were enrolled in the study...
December 8, 2016: BMC Pediatrics
https://www.readbyqxmd.com/read/27931082/pharmacotherapeutic-considerations-for-individuals-with-down-syndrome
#2
Erik J Hefti, Javier G Blanco
Down syndrome (DS, trisomy 21) is the most common survivable disorder due to aneuploidy. Individuals with DS may experience multiple comorbid health problems including congenital heart defects, endocrine abnormalities, skin and dental problems, seizure disorders, leukemia, dementia, and obesity. These associated conditions may necessitate pharmacotherapeutic management with various drugs. The complex pathobiology of DS may alter drug disposition and drug response in some individuals. For example, reports have documented increased rates of adverse drug reactions in patients with DS treated for leukemia and dementia...
December 8, 2016: Pharmacotherapy
https://www.readbyqxmd.com/read/27928836/atrial-septal-defect-repair-gone-wrong
#3
Richard Bloomingdale, Said Ashraf, Shaun Cardozo
Isolated atrial septal defect (ASD) accounts for 13% of congenital heart disorders. The anatomic location, size, and coexistence of other cardiac anomalies determine outcomes of repair. Surgical closure was the first-choice treatment until the 1990s and remains the only treatment for large defects. We describe a case of a 64-year-old woman who underwent surgical repair for an ASD as a child in 1959. She presented with dyspnea to the hospital almost 53 years after the surgery. Diagnostic cardiac imaging revealed interesting anatomy of the repair surgery...
December 8, 2016: Echocardiography
https://www.readbyqxmd.com/read/27928261/iatrogenic-diversion-of-inferior-vena-cava-into-left-atrium-after-surgery-for-a-rare-combination-of-congenital-heart-diseases
#4
Feridoun Sabzi
Atrial septal defect (ASD) is a common congenital anomaly that has low surgical mortality and morbidity. We report a very rare case of a low-lying ASD, combined with the drainage of the inferior vena cava and the left superior vena cava into the left atrium. This combination was associated with an unroofed coronary sinus. We also describe an iatrogenic surgical diversion of the inferior vena cava into the left atrium with its complication. The patient presented with moderate cyanosis and was referred for elective ASD repair...
April 13, 2016: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/27928258/evaluation-of-pulse-oximetry-in-the-early-detection-of-cyanotic-congenital-heart-disease-in-newborns
#5
Amir Hosein Movahedian, Ziba Mosayebi, Setareh Sagheb
Background: Delayed or missed diagnosis of critical and cyanotic congenital heart disease (CHD) in asymptomatic newborns may result in significant morbidity and mortality. The aim of this study was to determine the accuracy of pulse oximetry screening performed on the first day of life for the early detection of critical and cyanotic CHD in apparently normal newborns. Methods: This cross-sectional study used postductal pulse oximetry to evaluate term neonates born between 2008 and 2011 with normal physical examinations...
April 13, 2016: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/27925466/a-prospective-evaluation-of-contrast-and-radiation-dose-and-image-quality-in-cardiac-ct-in-children-with-complex-congenital-heart-disease-using-low-concentration-iodinated-contrast-agent-and-low-tube-voltage-and-current
#6
Qiao-Ru Hou, Wei Gao, Yu Min Zhong, Ai Min Sun, Qian Wang, Hai Sheng Qiu, Fang Wang, Jian Ying Li, Liwei Hu
OBJECTIVE: To assess image quality, contrast dose and radiation dose in cardiac CT in children with congenital heart disease using low-concentration iodinated contrast agent and low tube voltage and current in comparison with standard-dose protocol. METHODS: 110 patients with congenital heart disease were randomized to one of the two scan protocols: Group A (n=45) with 120mA tube current and contrast agent of 270mgI/ml in concentration (Visipaque, GE Healthcare), and Group B (n=65) with the conventional 160mA and 370mgI/mL concentration contrast (Iopamiro, Bracco)...
December 7, 2016: British Journal of Radiology
https://www.readbyqxmd.com/read/27924207/combined-spinal-epidural-anesthesia-for-urgent-cesarean-section-in-a-parturient-with-a-single-ventricle-a-case-report
#7
Stefano Catarci, Fabio Sbaraglia, Bruno Antonio Zanfini, Salvatore Vagnoni, Luciano Frassanito, Gaetano Draisci
The number of women with major congenital heart defects reaching reproductive age is likely increasing. We herein describe the anesthetic management of a 33-year-old woman at 37 gestational weeks with a history of Glenn surgery who was undergoing an urgent cesarean section due to pathological cardiotocography. Combined spinal-epidural anesthesia was the most suitable technique for urgent cesarean section in our patient with a single ventricle and phasic flow in the pulmonary artery because it provided rapid-onset anesthesia with negligible hemodynamic effects...
December 2016: Korean Journal of Anesthesiology
https://www.readbyqxmd.com/read/27920638/interstitial-1q21-1-microdeletion-is-associated-with-severe-skeletal-anomalies-dysmorphic-face-and-moderate-intellectual-disability
#8
Bruno F Gamba, Roseli M Zechi-Ceide, Nancy M Kokitsu-Nakata, Siulan Vendramini-Pittoli, Carla Rosenberg, Ana C V Krepischi Santos, Lucilene Ribeiro-Bicudo, Antonio Richieri-Costa
We report on a Brazilian patient with a 1.7-Mb interstitial microdeletion in chromosome 1q21.1. The phenotypic characteristics include microcephaly, a peculiar facial gestalt, cleft lip/palate, and multiple skeletal anomalies represented by malformed phalanges, scoliosis, abnormal modeling of vertebral bodies, hip dislocation, abnormal acetabula, feet anomalies, and delayed neuropsychological development. Deletions reported in this region are clinically heterogeneous, ranging from subtle phenotypic manifestations to severe congenital heart defects and/or neurodevelopmental findings...
November 2016: Molecular Syndromology
https://www.readbyqxmd.com/read/27920458/assessing-extensive-cardiac-echography-examination-for-detecting-foetal-congenital-heart-defects-during-early-and-late-gestation-a-systematic-review-and-meta-analysis
#9
https://www.readbyqxmd.com/read/27914791/aberrant-developmental-titin-splicing-and-dysregulated-sarcomere-length-in-thymosin-%C3%AE-4-knockout-mice
#10
Nicola Smart, Johannes Riegler, Cameron W Turtle, Craig A Lygate, Debra J McAndrew, Katja Gehmlich, Karina N Dubé, Anthony N Price, Vivek Muthurangu, Andrew M Taylor, Mark F Lythgoe, Charles Redwood, Paul R Riley
Sarcomere assembly is a highly orchestrated and dynamic process which adapts, during perinatal development, to accommodate growth of the heart. Sarcomeric components, including titin, undergo an isoform transition to adjust ventricular filling. Many sarcomeric genes have been implicated in congenital cardiomyopathies, such that understanding developmental sarcomere transitions will inform the aetiology and treatment. We sought to determine whether Thymosin β4 (Tβ4), a peptide that regulates the availability of actin monomers for polymerization in non-muscle cells, plays a role in sarcomere assembly during cardiac morphogenesis and influences adult cardiac function...
November 30, 2016: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/27913766/myocardial-cytochrome-oxidase-activity-increases-with-age-and-hypoxemia-in-patients-with-congenital-heart-disease
#11
Michael Onwugbufor, Richard J Levy, David Zurakowski, Richard A Jonas, Pranava Sinha
BACKGROUND: Myocardial tolerance to ischemia is influenced by age and preoperative cyanosis through unknown mechanisms and significantly affects postoperative outcomes. Cytochrome c oxidase (CcOx), the terminal enzyme of the mitochondrial electron transport chain, may play a role in the susceptibility to ischemic-reperfusion (IR) injury. Our study aimed at investigating changes in human myocardial CcOx activity based on age and preoperative oxygen saturation to understand its role in transition from neonatal to mature myocardium and hypoxic conditions...
December 1, 2016: Perfusion
https://www.readbyqxmd.com/read/27912209/databases-for-congenital-heart-defect-public-health-studies-across-the-lifespan
#12
REVIEW
Tiffany J Riehle-Colarusso, Lisa Bergersen, Craig S Broberg, Cynthia H Cassell, Darryl T Gray, Scott D Grosse, Jeffrey P Jacobs, Marshall L Jacobs, Russell S Kirby, Lazaros Kochilas, Asha Krishnaswamy, Arianne Marelli, Sara K Pasquali, Thalia Wood, Matthew E Oster
No abstract text is available yet for this article.
October 26, 2016: Journal of the American Heart Association
https://www.readbyqxmd.com/read/27911332/type-0-spinal-muscular-atrophy-further%C3%A2-delineation-of-prenatal-and%C3%A2-postnatal-features-in-16-patients
#13
Sarah Grotto, Jean-Marie Cuisset, Stéphane Marret, Séverine Drunat, Patricia Faure, Séverine Audebert-Bellanger, Isabelle Desguerre, Vincent Flurin, Anne-Gaëlle Grebille, Anne-Marie Guerrot, Hubert Journel, Gilles Morin, Ghislaine Plessis, Sylvain Renolleau, Joëlle Roume, Brigitte Simon-Bouy, Renaud Touraine, Marjolaine Willems, Thierry Frébourg, Eric Verspyck, Pascale Saugier-Veber
BACKGROUND: Spinal muscular atrophy (SMA) is caused by homozygous inactivation of the SMN1 gene. The SMN2 copy number modulates the severity of SMA. The 0SMN1/1SMN2 genotype, the most severe genotype compatible with life, is expected to be associated with the most severe form of the disease, called type 0 SMA, defined by prenatal onset. OBJECTIVE: The aim of the study was to review clinical features and prenatal manifestations in this rare SMA subtype. METHODS: SMA patients with the 0SMN1/1SMN2 genotype were retrospectively collected using the UMD-SMN1 France database...
November 29, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27909690/intellectual-functioning-in-children-with-congenital-heart-defects-treated-with-surgery-or-by-catheter-interventions
#14
Carmen Ryberg, Jan Sunnegårdh, Maria Thorson, Malin Broberg
BACKGROUND: Studies suggest that children with congenital heart defects (CHD) are at risk for adverse intellectual functioning. However, factors related to lower intellectual functioning in this group are largely unknown. This study describes intellectual functioning in children with CHD in relation to severity of the heart defect, the child's age, and the socioeconomic status of the family (SES). METHODS: Two hundred twenty-eight children treated with surgery or by catheter technique were tested using the Wechsler intelligence scales to determine full scale IQ (FSIQ)...
2016: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/27909479/atrial-fibrillation-ablation-in-adults-with-repaired-congenital-heart-disease
#15
REVIEW
Marta Acena Md, Ignasi Anguera Md PhD, Paolo D Dallaglio Md, Marcos Rodriguez Md, Xavier Sabaté Md PhD
The incidence of atrial fibrillation (AF) in congenital heart disease (CHD) adults has increased in the past decades due to a longer life expectancy of this population where the subjects are exposed to cardiac overflow, overpressure and structural changes for years. The literature regarding AF ablation in repaired CHD adults emphasizes the importance of intracardiac echocardiography (ICE) to perform the transseptal puncture and the ablation procedure in the left atrium (LA), both effectively and safely. In small case control studies, where the predominant congenital cardiomyopathy was the atrial septal defect, the most common strategy for ablation was antral isolation of the pulmonary veins showing results, at one year follow-up, similar to those in the general population...
February 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27906972/selective-serotonin-reuptake-inhibitor-ssri-antidepressants-in-pregnancy-and-congenital-anomalies-analysis-of-linked-databases-in-wales-norway-and-funen-denmark
#16
Sue Jordan, Joan K Morris, Gareth I Davies, David Tucker, Daniel S Thayer, Johannes M Luteijn, Margery Morgan, Ester Garne, Anne V Hansen, Kari Klungsøyr, Anders Engeland, Breidge Boyle, Helen Dolk
BACKGROUND: Hypothesised associations between in utero exposure to selective serotonin reuptake inhibitors (SSRIs) and congenital anomalies, particularly congenital heart defects (CHD), remain controversial. We investigated the putative teratogenicity of SSRI prescription in the 91 days either side of first day of last menstrual period (LMP). METHODS AND FINDINGS: Three population-based EUROCAT congenital anomaly registries- Norway (2004-2010), Wales (2000-2010) and Funen, Denmark (2000-2010)-were linked to the electronic healthcare databases holding prospectively collected prescription information for all pregnancies in the timeframes available...
2016: PloS One
https://www.readbyqxmd.com/read/27906085/tissue-engineered-cardiac-patch-seeded-with-human-induced-pluripotent-stem-cell-derived-cardiomyocytes-promoted-the-regeneration-of-host-cardiomyocytes-in-a-rat-model
#17
Tadahisa Sugiura, Narutoshi Hibino, Christopher K Breuer, Toshiharu Shinoka
BACKGROUND: Thousands of babies are born with congenital heart defects that require surgical repair involving a prosthetic implant. Lack of growth in prosthetic grafts is especially detrimental in pediatric surgery. Cell seeded biodegradable tissue engineered grafts are a novel solution to this problem. The purpose of the present study is to evaluate the feasibility of seeding human induced pluripotent stem cell derived cardiomyocytes (hiPS-CMs) onto a biodegradable cardiac patch. METHODS: The hiPS-CMs were cultured on a biodegradable patch composed of a polyglycolic acid (PGA) and a 50:50 poly (l-lactic-co-ε-caprolactone) copolymer (PLCL) for 1 week...
December 1, 2016: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/27904846/complete-congenital-heart-block-in-a-neonate-with-a-complex-congenital-heart-defect-in-africa
#18
Clovis Nkoke, Edvine Yonta Wawo, Liliane Kuate Mfeukeu, Larissa Makamte, Sandrine Dikosso Edie, Flore Esiene Balana
Congenital heart block (CHB) is rare disorder that has a higher mortality when associated with structural congenital heart defects. Very few cases have been reported in Sub-Saharan Africa (SSA). We present a case of complete CHB associated with a complex congenital heart defect in a neonate in Cameroon. A 1-month-old neonate in Cameroon was referred for the evaluation of bradycardia. The obstetrical ultrasound done during pregnancy revealed fetal bradycardia without further evaluation. Clinical examination showed well a developed neonate with bradycardia at 62 beats/minute, and mild cyanosis with oxygen saturation at 93% at room air...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27904843/cardiac-surgery-in-africa-a-thirty-five-year-experience-on-open-heart-surgery-in-cote-d-ivoire
#19
Koffi Herve Yangni-Angate, Christophe Meneas, Florent Diby, Manga Diomande, Anicet Adoubi, Yves Tanauh
BACKGROUND: Few centers for open heart surgery (OHS) are in Sub-Saharan Africa. Lack of OHS results is also noted. By reporting our African experience on OHS, the aim of this study was to fill the gap. METHODS: It is a retrospective study on 2,612 patients who were subject to an OHS between 1978 and 2013. Data were collected from demographical, clinical, investigative studies, surgical and outcomes parameters. RESULTS: There were 1,475 cases of rheumatic heart diseases (RHD), 126 endomyocardial fibrosis (EMF), 741 congenital heart diseases (CHDs) and 270 various affections...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27904570/existence-of-mutations-in-the-homeodomain-encoding-region-of-nkx2-5-gene-in-iranian-patients-with-tetralogy-of-fallot
#20
Majid Kheirollahi, Fereshteh Khosravi, Saeideh Ashouri, Alireza Ahmadi
BACKGROUND: Tetralogy of Fallot (TOF), the most common cyanotic heart defect and one of the most common congenital heart diseases, occurs mostly sporadically and nonsyndromically. The underlying molecular genetic mechanism is not known. Therefore, the existence of mutations in the homeodomain-encoding region of NKX2.5 gene in Iranian patients with tetralogy of Fallot is evaluated. MATERIALS AND METHODS: In the present study, we analyzed the peripheral blood samples of27 patients in order to find any mutation in the 180 bp homeodomain-encoding region of NKX2...
2016: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
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