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Congenital cardiac

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https://www.readbyqxmd.com/read/27928843/systemic-inflammatory-response-syndrome-after-pediatric-congenital-heart-surgery-incidence-risk-factors-and-clinical-outcome
#1
Martin Boehne, Michael Sasse, André Karch, Friederike Dziuba, Alexander Horke, Torsten Kaussen, Rafael Mikolajczyk, Philipp Beerbaum, Thomas Jack
BACKGROUND: Systemic inflammatory response syndrome (SIRS) is frequent after cardiac surgery, but data on its incidence and perioperative risk factors are scarce for children with congenital heart disease. METHODS: SIRS incidence within 72 hours following cardiac surgery was evaluated in a secondary analysis of children enrolled to a treatment-free control group of a randomized controlled trial. Intraoperative parameters were investigated for their association with SIRS using multivariable fractional polynomial logistic regression models...
December 7, 2016: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/27928836/atrial-septal-defect-repair-gone-wrong
#2
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/27926639/angiotensin-converting-enzyme-inhibitors-and-the-risk-of-congenital-malformations
#3
Brian T Bateman, Elisabetta Patorno, Rishi J Desai, Ellen W Seely, Helen Mogun, Sara Z Dejene, Michael A Fischer, Alexander M Friedman, Sonia Hernandez-Diaz, Krista F Huybrechts
OBJECTIVE: To examine the association between first-trimester angiotensin-converting enzyme (ACE) inhibitor exposure and the risk of overall major congenital, cardiac, and central nervous system malformations. METHODS: We used a cohort of completed pregnancies linked to liveborn neonates derived from Medicaid claims from 2000 to 2010. We examined the risk of malformations associated with first-trimester exposure to an ACE inhibitor. Propensity score-based methods were used to control for potential confounders including maternal demographics, medical conditions, exposure to other medications, and measures of health care utilization...
December 2, 2016: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27926615/coronary-artery-evaluation-by-screening-echocardiogram-in-intercollegiate-athletes
#4
Walter J Hoyt, Peter N Dean, Daniel S Schneider, Mark R Conaway, Christopher M Kramer, Robert W Battle
INTRODUCTION: Coronary artery anomalies are the second most common congenital cause of sudden cardiac death (SCD) in young athletes. Some centers have advocated for limited transthoracic echocardiogram (TTE) protocols in the screening of college athletes, which may miss important causes of SCD. PURPOSE: The purpose of this study is to evaluate the ability of screening TTE to determine origin and proximal course of the coronary arteries in intercollegiate athletes...
December 6, 2016: Medicine and Science in Sports and Exercise
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
#5
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/27923943/venovenous-ecmo-support-in-an-infant-with-single-ventricle-physiology-and-catastrophic-pulmonary-venous-abnormalities
#6
Richard P Fernandez, Don Hayes, Patrick I McConnell, Darren Berman
Extracorporeal membrane oxygenation (ECMO) is an accepted treatment modality for life support refractory to conventional efforts in neonates with complex congenital heart lesions. Cannulation for ECMO can be accomplished by venovenous (VV) access where patients receive primarily respiratory support or venoarterial (VA) access which provides complete cardiopulmonary support. VV ECMO delivered by a single-vessel cannulation with a dual-chamber venous cannula allows for respiratory support while the patient remains dependent upon intrinsic cardiac function to support hemodynamics...
December 6, 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27922989/totally-endoscopic-robotic-correction-of-cor-triatriatum-sinister-coexisting-with-atrial-septal-defect
#7
Changqing Gao, Ming Yang, Cangsong Xiao, Huajun Zhang, Gang Wang
Cor triatriatum sinister (CTS) is a rare congenital cardiac anomaly and is mainly corrected through conventional surgery through sternotomy. We described our successful novel surgical technique of totally robotic correction of CTS in one case of CTS with concomitant repair of atrial septal defect using da Vinci SI Surgical System (Intuitive Surgical, Inc, Sunnyvale, CA USA) with excellent surgical outcome. We conclude that robotic correction of CTS is a feasible and safe alternative to conventional surgical technique...
December 5, 2016: Innovations: Technology and Techniques in Cardiothoracic and Vascular Surgery
https://www.readbyqxmd.com/read/27922501/an-overview-of-congenital-myopathies
#8
Jean K Mah, Jeffrey T Joseph
PURPOSE OF REVIEW: This article uses a case-based approach to highlight the clinical features as well as recent advances in molecular genetics, muscle imaging, and pathophysiology of the congenital myopathies. RECENT FINDINGS: Congenital myopathies refer to a heterogeneous group of genetic neuromuscular disorders characterized by early-onset muscle weakness, hypotonia, and developmental delay. Congenital myopathies are further classified into core myopathies, centronuclear myopathies, nemaline myopathies, and congenital fiber-type disproportion based on the key pathologic features found in muscle biopsies...
December 2016: Continuum: Lifelong Learning in Neurology
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/27919715/perioperative-brain-natriuretic-peptide-in-pediatric-cardiac-surgery-patients-its-association-with-postoperative-outcomes
#10
Tomoyuki Kanazawa, Moritoki Egi, Yuichiro Toda, Kazuyoshi Shimizu, Kentaro Sugimoto, Tatsuo Iwasaki, Hiroshi Morimatsu
OBJECTIVES: The aim of this study was to investigate the relationship between perioperative brain natriuretic peptide levels and the incidence of postoperative serious adverse events (SAEs) in pediatric cardiac patients. DESIGN: A prospective observational study. SETTING: A single-institution university hospital. PARTICIPANTS: Children under 15 years old who underwent cardiac surgery that required cardiopulmonary bypass from December 21, 2012 to February 26, 2014...
September 13, 2016: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/27917693/identification-of-kcnq1-compound-heterozygous-mutations-in-three-chinese-families-with-jervell-and-lange-nielsen-syndrome
#11
Cuicui Wang, Yu Lu, Jing Cheng, Lei Zhang, Wei Liu, Weihua Peng, Di Zhang, Hong Duan, Dongyi Han, Huijun Yuan
CONCLUSION: Besides expanding the spectrum of KCNQ1 mutations causing Jervell and Lange-Nielsen Syndrome (JLNS), the results showed diversity of its phenotypes, and emphasized the importance of molecular genetic analysis in confirming clinical diagnosis and making diagnosis possible before the emergency symptoms for deaf individuals. OBJECTIVES: This study aimed to investigate four patients from three Chinese families with congenital hearing loss clinically and genetically...
December 5, 2016: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/27916860/novel-pigt-variant-in-two-brothers-expansion-of-the-multiple-congenital-anomalies-hypotonia-seizures-syndrome-3-phenotype
#12
Nadia Skauli, Sean Wallace, Samuel C C Chiang, Tuva Barøy, Asbjørn Holmgren, Asbjørg Stray-Pedersen, Yenan T Bryceson, Petter Strømme, Eirik Frengen, Doriana Misceo
Biallelic PIGT variants were previously reported in seven patients from three families with Multiple Congenital Anomalies-Hypotonia Seizures Syndrome 3 (MCAHS3), characterized by epileptic encephalopathy, hypotonia, global developmental delay/intellectual disability, cerebral and cerebellar atrophy, craniofacial dysmorphisms, and skeletal, ophthalmological, cardiac, and genitourinary abnormalities. We report a novel homozygous PIGT missense variant c.1079G>T (p.Gly360Val) in two brothers with several of the typical features of MCAHS3, but in addition, pyramidal tract neurological signs...
November 29, 2016: Genes
https://www.readbyqxmd.com/read/27914791/aberrant-developmental-titin-splicing-and-dysregulated-sarcomere-length-in-thymosin-%C3%AE-4-knockout-mice
#13
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/27913770/cor-triatriatum-a-review
#14
REVIEW
Ajay Kumar Jha, Neeti Makhija
Cor triatriatum is a rare congenital cardiac anomaly defined by an abnormal septation within the atrium (left or right) leading to inflow obstruction to the respective ventricles. It exists either in isolated classical form or may be associated with simple to complex congenital cardiac anomalies. Several anatomical variants exist even in the classical form, and therefore, it may require multimodal diagnostic modalities to characterize and differentiate for better percutaneous interventional or surgical planning...
December 2, 2016: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/27913750/congenital-heart-block-and-immune-mediated-sensorineural-hearing-loss-possible-cross-reactivity-of-immune-response
#15
C Bason, I Pagnini, A Brucato, S Maestroni, A Puccetti, C Lunardi, R Cimaz
Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27913050/congenital-high-airway-obstruction-syndrome-chaos-a-perinatal-autopsy-case-report
#16
Manasi Gosavi, Lalit Kumar, Ashwini Ratnakar, Hema Bannur
INTRODUCTION: Congenital High Airway Obstruction Syndrome (CHAOS) is a rare anomaly where a partial or complete obstruction of upper airway is seen. As a consequence, the foetus develops enlarged lungs, inverted or flattened diaphragm and ascites. The pathological findings in a case of CHAOS is presented. CASE REPORT: A 28-year-old primigravida presented with a history of 5 months of amenorrhoea. Ultrasonography revealed enlarged echogenic lungs, cardiac mid-position, inverted diaphragm, foetal ascites and talipes of left foot...
October 26, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27909479/atrial-fibrillation-ablation-in-adults-with-repaired-congenital-heart-disease
#17
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/27907850/automated-annotation-and-quantitative-description-of-ultrasound-videos-of-the-fetal-heart
#18
Christopher P Bridge, Christos Ioannou, J Alison Noble
Interpretation of ultrasound videos of the fetal heart is crucial for the antenatal diagnosis of congenital heart disease (CHD). We believe that automated image analysis techniques could make an important contribution towards improving CHD detection rates. However, to our knowledge, no previous work has been done in this area. With this goal in mind, this paper presents a framework for tracking the key variables that describe the content of each frame of freehand 2D ultrasound scanning videos of the healthy fetal heart...
November 19, 2016: Medical Image Analysis
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
#19
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/27904843/cardiac-surgery-in-africa-a-thirty-five-year-experience-on-open-heart-surgery-in-cote-d-ivoire
#20
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
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