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https://www.readbyqxmd.com/read/28654552/severe-upper-airway-obstruction-after-intraoperative-transesophageal-echocardiography-in-pediatric-cardiac-surgery-a-retrospective-analysis
#1
Jörg Michel, Michael Hofbeck, Christian Schineis, Matthias Kumpf, Ellen Heimberg, Harry Magunia, Eckhard Schmid, Christian Schlensak, Gunnar Blumenstock, Felix Neunhoeffer
OBJECTIVES: The aim of this study was to evaluate if there is a correlation between the use of intraoperative transesophageal echocardiography and an increased rate of extubation failure and to find other risk factors for severe upper airway obstructions after pediatric cardiac surgery. DESIGN: Retrospective analysis. SETTING: Cardiac PICU. PATIENTS: Patients 24 months old or younger who underwent surgery for congenital heart disease with cardiopulmonary bypass were retrospectively enrolled and divided into two groups depending on whether they received an intraoperative transesophageal echocardiography or not...
June 24, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28653939/report-cerebral-electrical-impedance-value-reflects-brain-edema-caused-by-cardiopulmonary-bypass-in-infants
#2
Mingqing Peng, Chunbao Guo, Fang Gong, Min Li, Yuan Li, Qiang Peng, Lin Bo
The study aimed to investigate if the dynamic changes in cerebral electrical impedance (CEI) values could be used to monitor brain edema during cardiopulmonary bypass (CPB) in infants. Forty infants (mean age: 1.4±0.38y) with acyanotic congenital heart disease who underwent CPB open-heart surgery between September 2009 and March 2010 were prospectively enrolled, and divided into 2 groups based on aortic cross-clamping (ACC) time: CPB-A (ACC<50 min) and CPB-B (ACC<50 min). During the same period, twenty infants (aged 1-3y) who underwent surgery for indirect inguinal hernias were selected as controls...
May 2017: Pakistan Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28653809/a-prenatal-diagnosis-of-mosaic-trisomy-5-reveals-a-postnatal-complete-uniparental-disomy-of-chromosome-5-with-multiple-congenital-anomalies
#3
Andrew M Reittinger, Benjamin M Helm, Debra J Boles, Inder K Gadi, Samantha A Schrier Vergano
Mosaic trisomy 5 is a very rare condition in liveborns, with few cases reported in the last four decades. There are some reports of prenatally diagnosed mosaic trisomy 5 resulting in phenotypically normal offspring, suggesting a low level of mosaicism, but there are also reports associated with multiple congenital anomalies, cardiovascular malformations, and intrauterine growth restriction. We report an infant male diagnosed with mosaic trisomy 5 (5/15 cells) via amniocentesis. The patient was subsequently found to have uniparental disomy 5 (UPD5) by postnatal chromosome microarray, but high-resolution chromosome analysis on peripheral blood did not identify trisomy 5...
June 27, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28653806/assessment-of-large-copy-number-variants-in-patients-with-apparently-isolated-congenital-left-sided-cardiac-lesions-reveals-clinically-relevant-genomic-events
#4
Neil A Hanchard, Luis A Umana, Lisa D'Alessandro, Mahshid Azamian, Mojisola Poopola, Shaine A Morris, Susan Fernbach, Seema R Lalani, Jeffrey A Towbin, Gloria A Zender, Sara Fitzgerald-Butt, Vidu Garg, Jessica Bowman, Gladys Zapata, Patricia Hernandez, Cammon B Arrington, Dieter Furthner, Siddharth K Prakash, Neil E Bowles, Kim L McBride, John W Belmont
Congenital left-sided cardiac lesions (LSLs) are a significant contributor to the mortality and morbidity of congenital heart disease (CHD). Structural copy number variants (CNVs) have been implicated in LSL without extra-cardiac features; however, non-penetrance and variable expressivity have created uncertainty over the use of CNV analyses in such patients. High-density SNP microarray genotyping data were used to infer large, likely-pathogenic, autosomal CNVs in a cohort of 1,139 probands with LSL and their families...
June 27, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28653469/development-of-quality-metrics-for-ambulatory-pediatric-cardiology-chest-pain
#5
Jimmy C Lu, Manish Bansal, Sarina K Behera, Jeffrey R Boris, Brian Cardis, John S Hokanson, Bahram Kakavand, Roy Jedeikin
OBJECTIVE: As part of the American College of Cardiology Adult Congenital and Pediatric Cardiology Section effort to develop quality metrics (QMs) for ambulatory pediatric practice, the chest pain subcommittee aimed to develop QMs for evaluation of chest pain. DESIGN: A group of 8 pediatric cardiologists formulated candidate QMs in the areas of history, physical examination, and testing. Consensus candidate QMs were submitted to an expert panel for scoring by the RAND-UCLA modified Delphi process...
June 27, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28653367/significance-of-l-carnitine-for-human-health
#6
REVIEW
María M Adeva-Andany, Isabel Calvo-Castro, Carlos Fernández-Fernández, Cristóbal Donapetry-García, Ana María Pedre-Piñeiro
Carnitine acyltransferases catalyze the reversible transfer of acyl groups from acyl-coenzyme A esters to l-carnitine, forming acyl-carnitine esters that may be transported across cell membranes. l-Carnitine is a wáter-soluble compound that humans may obtain both by food ingestion and endogenous synthesis from trimethyl-lysine. Most l-carnitine is intracellular, being present predominantly in liver, skeletal muscle, heart and kidney. The organic cation transporter-2 facilitates l-carnitine uptake inside cells...
June 26, 2017: IUBMB Life
https://www.readbyqxmd.com/read/28653340/aortic-perfusion-score-for-pulmonary-atresia-with-intact-ventricular-septum-an-antegrade-coronary-perfusion-scoring-system-that-is-predictive-of-need-for-transplant-and-mortality
#7
Rohit S Loomba, Andrew N Pelech
BACKGROUND: Pulmonary atresia with intact ventricular septum is a unique congenital malformation of the heart in which patients may undergo single-, two- or 1½- ventricle repair. Size of the tricuspid valve annulus, morphology of the right ventricle and presence of ventriculocoronary connections can all impact the selection of appropriate palliative strategy. We developed the aortic perfusion score, a novel scoring system based on anterograde coronary perfusion with the aim of being able to identify patients at risk for death or transplant...
June 27, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28653300/defining-the-incidence-and-associated-morbidity-and-mortality-of-severe-respiratory-syncytial-virus-infection-among-children-with-chronic-diseases
#8
REVIEW
Paolo Manzoni, Josep Figueras-Aloy, Eric A F Simões, Paul A Checchia, Brigitte Fauroux, Louis Bont, Bosco Paes, Xavier Carbonell-Estrany
INTRODUCTION: REGAL (RSV Evidence-a Geographical Archive of the Literature) has provided a comprehensive review of the published evidence in the field of respiratory syncytial virus (RSV) in Western countries over the last 20 years. This review covers the risk and burden of RSV infection in children with underlying medical conditions or chronic diseases (excluding prematurity and congenital heart disease). METHODS: A systematic review of publications between January 1, 1995 and December 31, 2015 across PubMed, Embase, The Cochrane Library, and Clinicaltrials...
June 26, 2017: Infectious Diseases and Therapy
https://www.readbyqxmd.com/read/28652674/evolution-of-ventricular-outpouching-through-the-fetal-and-postnatal-periods-unabating-dilemma-of-serial-observation-or-surgical-correction
#9
REVIEW
Niraj Kumar Dipak, Sumitra Venkatesh, Shakuntala Prabhu, Sudha Rao
Ventricular outpouching is a rare finding in prenatal sonography and the main differential diagnoses are diverticulum, aneurysm, and pseudoaneurysm in addition to congenital cysts and clefts. The various modes of fetal presentation of congenital ventricular outpouching include an abnormal four-chamber view on fetal two-dimensional echocardiogram, fetal arrhythmia, fetal hydrops, and pericardial effusion. Left ventricular aneurysm (LVA)/nonapical diverticula are usually isolated defects. Apical diverticula are always associated with midline thoracoabdominal defects (epigastric pulsating diverticulum or large omphalocele) and other structural malformations of the heart...
July 2017: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/28652574/clinical-features-of-congenital-and-developmental-cataract-in-east-china-a-five-year-retrospective-review
#10
Xiangjia Zhu, Yu Du, Wenwen He, Ting Sun, Yinglei Zhang, Ruiqi Chang, Keke Zhang, Yi Lu
Congenital/developmental cataract is a significant cause of blindness in children worldwide. Full knowledge of clinical features is essential for early diagnosis and proper treatment to prevent irreversible visual impairment. We conducted a retrospective chart review on 520 congenital/developmental cataract cases based on a five-year clinical data from Eye and ENT Hospital of Fudan University, Shanghai, China. Clinical features including age at the surgery, chief complaints, interval between initial identification of cataract-related manifestations and surgery, etc...
June 26, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28651938/elevated-oxidative-stress-in-the-aortic-media-of-patients-with-bicuspid-aortic-valve
#11
Marie Billaud, Julie A Phillippi, Mary P Kotlarczyk, Jennifer C Hill, Bradley W Ellis, Claudette M St Croix, Nadiezhda Cantu-Medéllin, Eric E Kelley, Thomas G Gleason
OBJECTIVE: Congenital bicuspid aortic valve (BAV) is distinctly associated with the development of ascending aortopathy in adulthood, portending risk of both ascending aortic aneurysm and dissection. Our previous work implicated deficiency in oxidative stress response as a mediator of the BAV-associated aortopathy. We hypothesize that reactive oxygen species generation invokes elevated local oxidative tissue damage in ascending aorta of patients with BAV. METHODS: Ascending aortic specimens were obtained from patients undergoing elective aortic replacement and/or aortic valve replacement and during heart transplant operations...
May 25, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28651900/potential-clinical-applications-of-placental-stem-cells-for-use-in-fetal-therapy-of-birth-defects
#12
Melissa Vanover, Aijun Wang, Diana Farmer
Placental stem cells are of growing interest for a variety of clinical applications due to their multipotency and ready availability from otherwise frequently discarded biomaterial. Stem cells derived from the placenta have been investigated in a number of disease processes, including wound healing, ischemic heart disease, autoimmune disorders, and chronic lung or liver injury. Fetal intervention for structural congenital defects, such as spina bifida, has rapidly progressed as a field due to advances in maternal-fetal medicine and improving surgical techniques...
May 18, 2017: Placenta
https://www.readbyqxmd.com/read/28651679/determinants-of-quality-of-life-in-adults-with-chd-an-australian-cohort
#13
Sarah L Eaton, QiFeng Wang, Samuel Menahem
BACKGROUND: Following improved survival rates in children with CHD, their quality of life and its determinants have become increasingly important. As part of a multicentre study entitled "Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart Disease - International Study", this article reviews the relationships among quality of life, anxiety and depression, sense of coherence, and severity of disease in an Australian cohort of adults with CHD. Methods and results Adults with CHD were recruited from a single, community-based cardiology practice...
June 27, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28651677/influence-of-weight-at-the-time-of-first-palliation-on-survival-in-patients-with-a-single-ventricle
#14
Sushitha Surendran, T K Susheel Kumar, Ben Tansey, Jerry Allen, David Zurakowski, Umar Boston, Samir H Shah, Christopher J Knott-Craig
BACKGROUND: Numerous advances in surgical techniques and understanding of single-ventricle physiology have resulted in improved survival. We sought to determine the influence of various demographic, perioperative, and patient-specific factors on the survival of single-ventricle patients following stage 1 palliation at our institution. METHODS: We conducted a retrospective study of all single-ventricle patients who had undergone staged palliation at our institution over an 8-year period...
June 27, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28651676/anomalous-origin-of-the-left-coronary-artery-from-the-right-pulmonary-artery-in-a-univentricular-heart
#15
Ziyad M Binsalamah, Diego A Lara, Emmett D McKenzie
Anomalous origin of the left coronary artery from the right pulmonary artery in single ventricles is a very rare congenital anomaly. Failure to recognise it preoperatively may lead to adverse outcomes, including death. We report the case of a neonate with a univentricular heart in the form of a double-outlet right ventricle, mitral atresia with discrete coarctation of the aorta, and an incidental intraoperative finding of an anomalous origin of the left coronary artery from the right pulmonary artery. The patient underwent a successful repair with an uneventful postoperative course...
June 27, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28651276/white-paper-interventional-mri-current-status-and-potential-for-development-considering-economic-perspectives-part-1-general-application
#16
Jörg Barkhausen, Thomas Kahn, Gabriele A Krombach, Christiane K Kuhl, Joachim Lotz, David Maintz, Jens Ricke, Stefan O Schönberg, Thomas J Vogl, Frank K Wacker
Background MRI is attractive for the guiding and monitoring of interventional procedures due to its high intrinsic soft tissue contrast and the possibility to measure physiologic parameters like flow and cardiac function. Method The current status of interventional MRI for the clinical routine was analyzed. Results The effort needed for the development of MR-safe monitoring systems and instruments initially resulted in the application of interventional MRI only for procedures that could not be performed by other means...
July 2017: RöFo: Fortschritte Auf Dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
https://www.readbyqxmd.com/read/28649564/primary-ciliary-dyskinesia-an-update-on-clinical-aspects-genetics-diagnosis-and-future-treatment-strategies
#17
REVIEW
Virginia Mirra, Claudius Werner, Francesca Santamaria
Primary ciliary dyskinesia (PCD) is an orphan disease (MIM 244400), autosomal recessive inherited, characterized by motile ciliary dysfunction. The estimated prevalence of PCD is 1:10,000 to 1:20,000 live-born children, but true prevalence could be even higher. PCD is characterized by chronic upper and lower respiratory tract disease, infertility/ectopic pregnancy, and situs anomalies, that occur in ≈50% of PCD patients (Kartagener syndrome), and these may be associated with congenital heart abnormalities...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28649500/complete-congenital-heart-block-a-case-of-multilevel-block
#18
Ian W Hovis, David N Schidlow, Jeffrey P Moak
No abstract text is available yet for this article.
June 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28648597/genotype-phenotype-correlation-for-congenital-heart-disease-in-down-syndrome-through-analysis-of-partial-trisomy-21-cases
#19
Maria Chiara Pelleri, Elena Gennari, Chiara Locatelli, Allison Piovesan, Maria Caracausi, Francesca Antonaros, Alessandro Rocca, Costanza Maria Donati, Letizia Conti, Pierluigi Strippoli, Marco Seri, Lorenza Vitale, Guido Cocchi
Among Down syndrome (DS) children, 40-50% have congenital heart disease (CHD). Although trisomy 21 is not sufficient to cause CHD, three copies of at least part of chromosome 21 (Hsa21) increases the risk for CHD. In order to establish a genotype-phenotype correlation for CHD in DS, we built an integrated Hsa21 map of all described partial trisomy 21 (PT21) cases with sufficient indications regarding presence or absence of CHD (n=107), focusing on DS PT21 cases. We suggest a DS CHD candidate region on 21q22...
June 22, 2017: Genomics
https://www.readbyqxmd.com/read/28648533/recurrent-laryngeal-nerve-injury-and-swallowing-dysfunction-in-neonatal-aortic-arch-repair
#20
Kamal K Pourmoghadam, William M DeCampli, Mark Ruzmetov, James Kosko, Sami Kishawi, Michael O'Brien, Amanda Cowden, Kurt Piggott, Harun Fakioglu
BACKGROUND: We evaluated the incidence, clinical effect, and recovery rate of vocal cord dysfunction (VCD) and swallowing dysfunction in neonates undergoing aortic arch repair. METHODS: We retrospectively evaluated 101 neonates who underwent aortic arch reconstruction from 2008 to 2015. Direct flexible laryngoscopy was performed in 89 patients before initiation of postoperative oral feeding after Norwood (n = 63) and non-Norwood (n = 26) arch reconstruction. We defined VCD as immobility of vocal cords or their lack of coaptation and poor mobility...
June 22, 2017: Annals of Thoracic Surgery
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