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https://www.readbyqxmd.com/read/28549113/restrictive-enlargement-of-the-pulmonary-annulus-at-repair-of-tetralogy-of-fallot-a-comparative-10-year-follow-up-study%C3%A2
#1
Jana Logoteta, Lars Dullin, Jan Hinnerk Hansen, Carsten Rickers, Mona Salehi Ravesh, Abdullah Al Bulushi, Ines Kristo, Philip Wegner, Michael Schumacher, Tim Attmann, Jens Scheewe, Hans-Heiner Kramer
OBJECTIVES: Since 1996, our centre performs restrictive enlargement of the pulmonary annulus at surgical repair of tetralogy of Fallot. A transannular patch is only used if the z-score of the pulmonary annulus is smaller than -2. We sought to determine whether this strategy reduces pulmonary insufficiency (PI) and reoperation rate compared to a nationwide contemporary cohort that has not been operated using a uniform strategy. METHODS: Eighty-seven tetralogy of Fallot patients were included in the study (Group 1)...
May 26, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28549022/pictorial-review-of-surgical-anatomy-in-adult-congenital-heart-disease
#2
Carlo N De Cecco, Giuseppe Muscogiuri, José M Madrid Pérez, Marwen Eid, Pal Suranyi, Virginia W Lesslie, Gorka Bastarrika
The survival rate of patients with congenital heart disease (CHD) has dramatically improved over the last 2 decades because of technological and surgical advances in diagnosis and treatment, respectively. The vast majority of CHD patients are, in fact, amenable to treatment by either device closure or surgery. Considering the wide spectrum of surgical procedures and complex native and derived anatomy, continuous and detailed follow-up is of paramount importance. Cardiac magnetic resonance and cardiac computed tomography angiography are the cornerstones of diagnosis and follow-up of CHD, allowing for comprehensive noninvasive assessment of the heart, coronary tree, and intrathoracic great vessels, along with both morphological and functional evaluation...
May 25, 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28548989/imaging-adult-patients-with-fontan-circulation
#3
Salil Ginde, Benjamin H Goot, Peter C Frommelt
PURPOSE OF REVIEW: Survival after the Fontan procedure for palliation of single ventricle congenital heart disease has improved. However, adults with Fontan circulation are at risk for several complications including heart failure, thromboembolism, and protein-losing enteropathy. This review discusses the role of noninvasive imaging for surveillance and early detection of anatomic and functional abnormalities of the Fontan circulation that can impact the risk for Fontan failure over time...
May 25, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28546762/cardiovascular-magnetic-resonance-imaging-clinical-implications-in-the-evaluation-of-connective-tissue-diseases
#4
REVIEW
Sophie Mavrogeni, George Markousis-Mavrogenis, Loukia Koutsogeorgopoulou, Genovefa Kolovou
Cardiovascular magnetic resonance imaging is a recently developed noninvasive, nonradiating, operator-independent technique that has been successfully used for the evaluation of congenital heart disease, valvular and pericardial diseases, iron overload, cardiomyopathies, great and coronary vessel diseases, cardiac inflammation, stress-rest myocardial perfusion, and fibrosis. Rheumatoid arthritis and other spondyloarthropathies, systemic lupus erythematosus, inflammatory myopathies, mixed connective tissue diseases (CTDs), systemic sclerosis, vasculitis, and sarcoidosis are among CTDs with serious cardiovascular involvement; this is due to multiple causative factors such as myopericarditis, micro/macrovascular disease, coronary artery disease, myocardial fibrosis, pulmonary hypertension, and finally heart failure...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28545873/reveal-linq-versus-reveal-xt-implantable-loop-recorders-intra-and-post-procedural-comparison
#5
Hoang H Nguyen, Ian H Law, Michael W Rudokas, Jennifer Lampe, Tammy M Bowman, George F Van Hare, Jennifer N Avari Silva
OBJECTIVES: To compare the procedure, recovery, hospitalization times, and costs along with patient/parent satisfaction after newer-generation cardiac implantable loop recorder (Reveal LINQ; Medtronic Inc, Minneapolis, Minnesota) and previous-generation implantable loop recorder (Reveal XT; Medtronic Inc). STUDY DESIGN: A prospective study of patients undergoing LINQ implantations between April 2014 and October 2015 was performed. Retrospective chart review of patients undergoing XT implantations was performed for comparison...
May 22, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28545820/extracardiac-conduit-fontan-outcome-data-in-early-adulthood
#6
Dominica Zentner, Caitlin Cheshire, Leeanne Grigg
BACKGROUND: To describe the survival and health outcome status of young adults with an extracardiac Fontan procedure performed either as a primary or conversion (secondary) Fontan surgery. METHODS: The database of the Adult Congenital Heart disease service at the Royal Melbourne Hospital was interrogated to identify all adults who had undergone a primary extracardiac conduit Fontan (n=29) or a Fontan conversion with this procedure (n=8). We then determined vital status, age, original anatomy and functional status in early adulthood in both groups...
May 3, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28545044/differentiation-of-spontaneously-contracting-cardiomyocytes-from-non-virally-reprogrammed-human-amniotic-fluid-stem-cells
#7
Aaron J Velasquez-Mao, Christopher J M Tsao, Madeline N Monroe, Xavier Legras, Beatrice Bissig-Choisat, Karl-Dimiter Bissig, Rodrigo Ruano, Jeffrey G Jacot
Congenital heart defects are the most common birth defect. The limiting factor in tissue engineering repair strategies is an autologous source of functional cardiomyocytes. Amniotic fluid contains an ideal cell source for prenatal harvest and use in correction of congenital heart defects. This study aims to investigate the potential of amniotic fluid-derived stem cells (AFSC) to undergo non-viral reprogramming into induced pluripotent stem cells (iPSC) followed by growth-factor-free differentiation into functional cardiomyocytes...
2017: PloS One
https://www.readbyqxmd.com/read/28544839/isolated-right-ventricular-apical-trabecular-hypoplasia-a-case-report
#8
Qi Tan, Jie Zi, Mei Zhu, Anbiao Wang
Right ventricular (RV) hypoplasia with isolated myocardial disease and complete absence of RV trabeculae is a rare congenital heart disease, the current treatment of which is ineffective. Here, a rare case is presented of a middle-aged female patient with RV hypoplasia complicated by tricuspid regurgitation, right atrial thrombus, and atrial fibrillation. The patient was treated with a one-and-a-half ventricular repair, and showed significant improvements in cardiac function and physical activity tolerance...
January 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28544787/analysis-of-adults-with-congenital-heart-disease-presenting-to-pediatric-emergency-departments-with-arrhythmias
#9
Shaun Mohan, Brady S Moffett, Wilson Lam, Caridad de la Uz, Christina Miyake, Santiago O Valdes, Jeffrey J Kim
OBJECTIVE: As survivors of congenital heart disease (CHD) continue to age, healthcare utilization by this population has increased. It is unknown how often these patients utilize the emergency department (ED) at children's hospitals and how arrhythmias play a role in their utilization of care. DESIGN: Using a retrospective cohort design, the Pediatric Hospital Information System (PHIS) database was investigated and we studied adults (≥18 years) with CHD (ACHD) who presented to pediatric EDs from 2004 to 2014...
May 22, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28544699/renal-anomalies-and-lymphedema-distichiasis-syndrome-a-rare-association
#10
Gabriela E Jones, Anna K Richmond, Osric Navti, Hatem A Mousa, Stephen Abbs, Edward Thompson, Sahar Mansour, Pradeep C Vasudevan
Lymphedema distichiasis syndrome (LDS) is a rare, autosomal dominant genetic condition, characterized by lower limb lymphedema and distichiasis. Other associated features that have been reported include varicose veins, cleft palate, congenital heart defects, and ptosis. We update a previously reported family with a pathogenic variant in FOXC2 (c.412-413insT) where five affected individuals from the youngest generation had congenital renal anomalies detected on prenatal ultrasound scan. These included four fetuses with hydronephrosis and one with bilateral renal agenesis...
May 23, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28544620/phenotyping-cardiac-and-structural-birth-defects-in-fetal-and-newborn-mice
#11
REVIEW
Xiaoqin Liu, Andrew J Kim, William Reynolds, Yijen Wu, Cecilia W Lo
Mouse models are invaluable for investigating the developmental etiology and molecular pathogenesis of structural birth defects. While this has been deployed for studying a wide spectrum of birth defects, mice are particularly valuable for modeling congenital heart disease, given they have the same four-chamber cardiac anatomy as in humans. We have developed the use of noninvasive fetal ultrasound together with micro-computed tomography (micro-CT) imaging for high throughput phenotyping of mice for congenital heart defects (CHD) and other developmental anomalies...
May 22, 2017: Birth Defects Res
https://www.readbyqxmd.com/read/28544539/intensive-care-unit-admissions-and-ventilation-support-in-infants-with-bronchiolitis
#12
Ed Oakley, Vi Chong, Meredith Borland, Jocelyn Neutze, Natalie Phillips, David Krieser, Stuart Dalziel, Andrew Davidson, Susan Donath, Kim Jachno, Mike South, Amanda Fry, Franz E Babl
OBJECTIVES: To describe the rate of intensive care unit (ICU) admission, type of ventilation support provided and risk factors for ICU admission in infants with bronchiolitis. DESIGN: Retrospective review of hospital records and Australia and New Zealand Paediatric Intensive Care (ANZPIC) registry data for infants 2-12 months old admitted with bronchiolitis. SETTING: Seven Australian and New Zealand hospitals. These infants were prospectively identified through the comparative rehydration in bronchiolitis (CRIB) study between 2009 and 2011...
May 19, 2017: Emergency Medicine Australasia: EMA
https://www.readbyqxmd.com/read/28544396/current-trends-in-racial-ethnic-and-healthcare-disparities-associated-with-pediatric-cardiac-surgery-outcomes
#13
Jennifer K Peterson, Yanjun Chen, Danh V Nguyen, Shaun P Setty
OBJECTIVE: Despite overall improvements in congenital heart disease outcomes, racial and ethnic disparities have continued. The purpose of this study is to examine the effect of race and ethnicity, as well as other risk factors on congenital heart surgery length of stay and in-hospital mortality. DESIGN: From the 2012 Healthcare Cost and Utilization Project Kids Inpatient Database (KID), we identified 13 130 records with Risk Adjustment in Congenital Heart Surgery complexity score-eligible procedures...
May 22, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28542743/neuroimaging-cardiovascular-physiology-and-functional-outcomes-in-infants-with-congenital-heart-disease
#14
REVIEW
Nathalie H P Claessens, Christopher J Kelly, Serena J Counsell, Manon J N L Benders
This review integrates data on brain dysmaturation and acquired brain injury using fetal and neonatal magnetic resonance imaging (MRI), including the contribution of cardiovascular physiology to differences in brain development, and the relationship between brain abnormalities and subsequent neurological impairments in infants with congenital heart disease (CHD). The antenatal and neonatal period are critical for optimal brain development; the developing brain is particularly vulnerable to haemodynamic disturbances during this time...
May 19, 2017: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/28542655/-risk-factors-associated-with-postoperative-acute-renal-failure-in-pediatric-patients-undergoing-cardiopulmonary-bypass-surgery
#15
Elsa Nohemí Reyes-Flandes, Alejandro Herrera-Landero, Pascual Bobadilla-González, Juan Carlos Núñez-Enríquez
Acute renal failure (ARF) is a complication associated with cardiac surgery with cardiopulmonary bypass (CPB) with an impact on morbidity and mortality. OBJECTIVE: To identify risk factors associated with postoperative IRA according to pediatric Risk, Injury, Failure, Loss, End-Stage Renal Disease scale in children undergoing cardiac surgery with CPB. PATIENTS AND METHOD: A nested case-control study was conducted. We included children under 16 years of age attended postoperative for CBP in a pediatric intensive care unit over a period of 18 months...
April 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/28542318/predicting-congenital-heart-defects-a-comparison-of-three-data-mining-methods
#16
Yanhong Luo, Zhi Li, Husheng Guo, Hongyan Cao, Chunying Song, Xingping Guo, Yanbo Zhang
Congenital heart defects (CHD) is one of the most common birth defects in China. Many studies have examined risk factors for CHD, but their predictive abilities have not been evaluated. In particular, few studies have attempted to predict risks of CHD from, necessarily unbalanced, population-based cross-sectional data. Therefore, we developed and validated machine learning models for predicting, before and during pregnancy, women's risks of bearing children with CHD. We compared the results of these models in a large-scale, comprehensive population-based retrospective cross-sectional epidemiological survey of birth defects in six counties in Shanxi Province, China, covering 2006 to 2008...
2017: PloS One
https://www.readbyqxmd.com/read/28542047/inferior-vena-cava-thrombus-in-a-postpartum-patient-with-fontan-physiology-a-case-report
#17
Jessica A Tashjian, Hannah Fraint, James DiNardo, Kathryn Rouine-Rapp
Women with complex congenital heart disease, such as those with single-ventricle physiology, are surviving into adulthood and becoming pregnant. Because of their complex physiology, common peripartum complications pose unique risks. We describe a patient with a single ventricle who underwent an external vascular conduit, nonfenestrated Fontan procedure in childhood and then presented during the postpartum period with extensive thrombosis in her lower extremity deep venous system and inferior vena cava. In this article, we will discuss single-ventricle physiology and the implications of pregnancy, anesthetic considerations, and data for maternal and fetal outcomes in this population...
May 23, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28541271/protein-altering-and-regulatory-genetic-variants-near-gata4-implicated-in-bicuspid-aortic-valve
#18
Bo Yang, Wei Zhou, Jiao Jiao, Jonas B Nielsen, Michael R Mathis, Mahyar Heydarpour, Guillaume Lettre, Lasse Folkersen, Siddharth Prakash, Claudia Schurmann, Lars Fritsche, Gregory A Farnum, Maoxuan Lin, Mohammad Othman, Whitney Hornsby, Anisa Driscoll, Alexandra Levasseur, Marc Thomas, Linda Farhat, Marie-Pierre Dubé, Eric M Isselbacher, Anders Franco-Cereceda, Dong-Chuan Guo, Erwin P Bottinger, G Michael Deeb, Anna Booher, Sachin Kheterpal, Y Eugene Chen, Hyun Min Kang, Jacob Kitzman, Heather J Cordell, Bernard D Keavney, Judith A Goodship, Santhi K Ganesh, Gonçalo Abecasis, Kim A Eagle, Alan P Boyle, Ruth J F Loos, Per Eriksson, Jean-Claude Tardif, Chad M Brummett, Dianna M Milewicz, Simon C Body, Cristen J Willer
Bicuspid aortic valve (BAV) is a heritable congenital heart defect and an important risk factor for valvulopathy and aortopathy. Here we report a genome-wide association scan of 466 BAV cases and 4,660 age, sex and ethnicity-matched controls with replication in up to 1,326 cases and 8,103 controls. We identify association with a noncoding variant 151 kb from the gene encoding the cardiac-specific transcription factor, GATA4, and near-significance for p.Ser377Gly in GATA4. GATA4 was interrupted by CRISPR-Cas9 in induced pluripotent stem cells from healthy donors...
May 25, 2017: Nature Communications
https://www.readbyqxmd.com/read/28541122/chance-of-surgery-in-adult-congenital-heart-disease
#19
Carianne L Verheugt, Cuno Spm Uiterwaal, Ilonca Vaartjes, Enno T van der Velde, A C Zomer, Folkert J Meijboom, Petronella G Pieper, Marco C Post, Hubert W Vliegen, Mark G Hazekamp, Diederick E Grobbee, Barbara Jm Mulder
Background Young patients with congenital heart disease reaching adulthood face mandatory transition to adult cardiology. Their new cardiologist needs to assess the chances of major future events such as surgery. Using a large national registry, we assessed if patient characteristics at the age of 18 years could predict the chance of congenital heart surgery in adulthood. Design and methods Of 10,300 patients from the CONCOR national registry, we used general patient characteristics at age 18 years, underlying congenital heart defect, history of complications, and interventions in childhood as potential predictors of congenital heart surgery occurring from age 18 years up to age 40 and 60 years...
January 1, 2017: European Journal of Preventive Cardiology
https://www.readbyqxmd.com/read/28540525/identification-of-22q11-2-deletion-syndrome-via-newborn-screening-for-severe-combined-immunodeficiency
#20
Jessica C Barry, Terrence Blaine Crowley, Soma Jyonouchi, Jennifer Heimall, Elaine H Zackai, Kathleen E Sullivan, Donna M McDonald-McGinn
PURPOSE: Chromosome 22q11.2 deletion syndrome (22q11.2DS), the most common cause of DiGeorge syndrome, is quite variable. Neonatal diagnosis traditionally relies on recognition of classic features and cytogenetic testing, but many patients come to attention only following identification of later onset conditions, such as hypernasal speech due to palatal insufficiency and developmental and behavioral differences including speech delay, autism, and learning disabilities that would benefit from early interventions...
May 24, 2017: Journal of Clinical Immunology
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