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https://www.readbyqxmd.com/read/28917495/complications-associated-with-implantable-cardioverter-defibrillators-in-adults-with-congenital-heart-disease-or-left-ventricular-noncompaction-cardiomyopathy-from-the-ncdr-%C3%A2-implantable-cardioverter-defibrillator-registry
#1
Marye J Gleva, Yongfei Wang, Jeptha P Curtis, Charles I Berul, Charles B Huddleston, Jeanne E Poole
Patients with childhood heart disease are living longer and entering adulthood, and may undergo implantable cardioverter-defibrillator (ICD) implantation to reduce the risk of sudden death. We evaluated the characteristics of adult patients with congenital heart disease or left ventricular noncompaction cardiomyopathy (LVNC) in the National Cardiovascular Disease Registry ICD Registry and determined ICD-related in-hospital complications. Patients with LVNC or transposition of the great arteries, tetralogy of Fallot, Ebstein's anomaly, atrial septal defect, ventricular septal defect, or common ventricle were identified in the registry...
August 8, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28912834/molecular-and-clinical-characterization-of-new-patient-with-1-08%C3%A2-mb-deletion-in-10p15-3-region
#2
Anna Poluha, Joanna Bernaciak, Ilona Jaszczuk, Marta Kędzior, Beata Anna Nowakowska
BACKGROUND: Three distinct contiguous gene deletion syndromes are located at 10p chromosomal region. The deletion, involving 10p15.3 region, has been characterized by (DeScipio et al., Am J Med Genet A 158A:2152-61, 2012). However, because of the variation in size of the described deletions and lack of knowledge about the involved genes, the correlation between genotypes and patients' phenotypes remains unknown. CASE PRESENTATION: We describe female patient with de novo 1,08 Mb deletion in 10p15...
2017: Molecular Cytogenetics
https://www.readbyqxmd.com/read/28904256/current-surgical-outcomes-of-congenital-heart-surgery-for-patients-with-down-syndrome-in-japan
#3
Takaya Hoashi, Norimichi Hirahara, Arata Murakami, Yasutaka Hirata, Hajime Ichikawa, Junjiro Kobayashi, Shinichi Takamoto
BACKGROUND: Current surgical outcomes of congenital heart surgery for patients with Down syndrome are unclear.Methods and Results:Of 29,087 operations between 2008 and 2012 registered in the Japan Congenital Cardiovascular Surgery Database (JCCVSD), 2,651 were carried out for patients with Down syndrome (9%). Of those, 5 major biventricular repair procedures [ventricular septal defect repair (n=752), atrioventricular septal defect repair (n=452), patent ductus arteriosus closure (n=184), atrial septal defect repair (n=167), tetralogy of Fallot (TOF) repair (n=108)], as well as 2 major single ventricular palliations [bidirectional Glenn (n=21) and Fontan operation (n=25)] were selected and their outcomes were compared...
September 12, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28902643/prevalence-and-characteristics-of-coronary-artery-anomalies-in-children-with-congenital-heart-disease-diagnosed-with-coronary-angiography
#4
Münevver Tuğba Temel, Mehmet Enes Coşkun, Osman Başpınar, Abdullah Tuncay Demiryürek
OBJECTIVE: Aim of the present study was to determine the prevalence of coronary artery anomalies in children with congenital heart disease. METHODS: Data of 1138 consecutive patients who were referred for cardiac catheterization and angiography for assessment of coronary anomaly between January 2005 and December 2009 were retrospectively analyzed. Total of 515 patients whose coronary arteries could be examined through left ventricle and aortic root injection were included in the study...
September 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28901230/clinical-progress-in-the-management-of-tetralogy-of-fallot-in-the-dominican-republic-a-case-series
#5
María T Perez, Marietta Vázquez, Michael F Canarie, Janet Toribio, Juan León-Wyss
BACKGROUND: Definitive surgical interventions for Dominican children with congenital heart disease, like those of other low- and middle-income countries, have been historically limited. METHODS: We undertook review of a case series focusing on the surgical correction of complex forms of tetralogy of Fallot at a single center, CEDIMAT Centro Cardiovascular, in the Dominican Republic, over a 30-month period. RESULTS: According to our criteria, 43 cases were determined to be complex tetralogy of Fallot repairs from the two-year period...
September 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28895497/turning-blue-babies-pink-alfred-blalock-s-shunt-for-fallot-s-tetralogy
#6
Tom Treasure
No abstract text is available yet for this article.
September 2017: Journal of the Royal Society of Medicine
https://www.readbyqxmd.com/read/28895301/early-leaflet-thrombosis-complicating-transcatheter-implantation-of-a-sapien-3-valve-in-a-native-right-ventricular-outflow-tract
#7
Mounir Riahi, Philipp Blanke, John Webb, Ronald G Carere
A 59-year-old female with Tetralogy of Fallot had a previous complete repair with RVOT patch enlargement. She developed subsequent severe symptomatic (NYHA III) pulmonary regurgitation with severe RV dilatation. She had a concomitant interstitial lung disease secondary to hypersensitivity pneumonitis that precluded her from cardiac surgery. After preprocedural assessment using computed tomography, echocardiography and invasive angiography we decided to implant a 29 mm Edwards Sapien 3 valve without pre-stenting...
September 12, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28890875/cyanotic-congenital-heart-diseases-among-nigerian-children
#8
Barakat Adeola Animasahun, Akpoembele Deborah Madise-Wobo, Olusola Yejide Kusimo
BACKGROUND: There are only few reports on cyanotic congenital heart diseases (CCHD) among Nigerian and African Children. The current report aim to provide the most recent hospital based data on the distribution of CCHD in children less than 14 years of age, the demographic characteristics and risk factors identified. METHODS: Prospective and cross-sectional involving consecutive cases of CCHD diagnosed with echocardiography at the Lagos State University Teaching Hospital between January 2007 and June 2016...
August 2017: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/28890082/catheter-palliation-or-early-surgery-for-tetralogy-of-fallot-options-when-timing-is-less-than-ideal
#9
EDITORIAL
James M Hammel
No abstract text is available yet for this article.
August 4, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28882285/stenting-of-the-right-ventricular-outflow-tract-promotes-better-pulmonary-arterial-growth-compared-with-modified-blalock-taussig-shunt-palliation-in-tetralogy-of-fallot-type-lesions
#10
Daniel Quandt, Bharat Ramchandani, John Stickley, Chetan Mehta, Vinay Bhole, David J Barron, Oliver Stumper
OBJECTIVES: This study sought to compare pulmonary arterial (PA) growth during palliation after right ventricular outflow tract (RVOT) stenting versus modified Blalock-Taussig shunt (mBTS) in patients coming forward for complete repair of tetralogy of Fallot-type lesions. BACKGROUND: RVOT stenting is a recent alternative to mBTS in the initial palliation of selected patients with Fallot-type lesions. METHODS: This was a retrospective, single-center study of nonrandomized, consecutive palliated Fallot patients over a 10-year period...
September 11, 2017: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/28882284/harmony-feasibility-trial-acute-and-short-term-outcomes-with-a-self-expanding-transcatheter-pulmonary-valve
#11
Lisa Bergersen, Lee N Benson, Matthew J Gillespie, Sharon L Cheatham, Andrew M Crean, Kan N Hor, Eric M Horlick, Te-Hsin Lung, Brian T McHenry, Mark D Osten, Andrew J Powell, John P Cheatham
OBJECTIVES: This study sought to obtain in vivo data to confirm assumptions on device loading conditions and assess procedural feasibility, safety, and valve performance. BACKGROUND: The Harmony transcatheter pulmonary valve (Medtronic, Minneapolis, Minnesota) was designed for patients with severe pulmonary regurgitation who require pulmonary valve replacement. METHODS: Three sites participated in this first Food and Drug Administration-approved early feasibility study using an innovative device design to accommodate the complex anatomy of the right ventricular outflow tract...
September 11, 2017: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/28881055/individual-healthcare-plan-for-allergic-children%C3%A2-at-school-lessons-from-a-2015-2016-school-year-survey
#12
Guillaume Pouessel, Stépanie Lejeune, Marie-Pierre Dupond, Annie Renard, Camille Fallot, Antoine Deschildre
The Individual Healthcare Plan (IHP) was implemented in schools in France in 2003 to improve management of allergic children. Our objectives were to assess the practical aspects of IHP (excluding asthma) and allergic reactions occurring at school. METHODS: Prospective study conducted in the North Department (France) during the 2015/2016 school-year. Two questionnaires were developed: one for the school doctors (n=67) and one for the school principals (n=2372). RESULTS: Data from 336 (25%) of the 1325 IHPs (0...
September 7, 2017: Pediatric Allergy and Immunology
https://www.readbyqxmd.com/read/28880457/development-of-quality-metrics-for-ambulatory-care-in-pediatric-patients-with-tetralogy-of-fallot
#13
Juan Villafane, Thomas C Edwards, Karim A Diab, Gary M Satou, Elizabeth Saarel, Wyman W Lai, Gerald A Serwer, Peter P Karpawich, Russell Cross, Russell Schiff, Devyani Chowdhury, Thomas J Hougen
OBJECTIVE: The objective of this study was to develop quality metrics (QMs) relating to the ambulatory care of children after complete repair of tetralogy of Fallot (TOF). DESIGN: A workgroup team (WT) of pediatric cardiologists with expertise in all aspects of ambulatory cardiac management was formed at the request of the American College of Cardiology (ACC) and the Adult Congenital and Pediatric Cardiology Council (ACPC), to review published guidelines and consensus data relating to the ambulatory care of repaired TOF patients under the age of 18 years...
September 7, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28878577/echocardiographic-classification-and-surgical-approaches-to-double-outlet-right-ventricle-for-great-arteries-arising-almost-exclusively-from-the-right-ventricle
#14
Kun-Jing Pang, Hong Meng, Sheng-Shou Hu, Hao Wang, David Hsi, Zhong-Dong Hua, Xiang-Bin Pan, Shou-Jun Li
Selecting an appropriate surgical approach for double-outlet right ventricle (DORV), a complex congenital cardiac malformation with many anatomic variations, is difficult. Therefore, we determined the feasibility of using an echocardiographic classification system, which describes the anatomic variations in more precise terms than the current system does, to determine whether it could help direct surgical plans. Our system includes 8 DORV subtypes, categorized according to 3 factors: the relative positions of the great arteries (normal or abnormal), the relationship between the great arteries and the ventricular septal defect (committed or noncommitted), and the presence or absence of right ventricular outflow tract obstruction (RVOTO)...
August 2017: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28878125/human-hepatic-organoids-for-the-analysis-of-human-genetic-diseases
#15
Yuan Guan, Dan Xu, Phillip M Garfin, Ursula Ehmer, Melissa Hurwitz, Greg Enns, Sara Michie, Manhong Wu, Ming Zheng, Toshihiko Nishimura, Julien Sage, Gary Peltz
We developed an in vitro model system where induced pluripotent stem cells (iPSCs) differentiate into 3-dimensional human hepatic organoids (HOs) through stages that resemble human liver during its embryonic development. The HOs consist of hepatocytes, and cholangiocytes, which are organized into epithelia that surround the lumina of bile duct-like structures. The organoids provide a potentially new model for liver regenerative processes, and were used to characterize the effect of different JAG1 mutations that cause: (a) Alagille syndrome (ALGS), a genetic disorder where NOTCH signaling pathway mutations impair bile duct formation, which has substantial variability in its associated clinical features; and (b) Tetralogy of Fallot (TOF), which is the most common form of a complex congenital heart disease, and is associated with several different heritable disorders...
September 7, 2017: JCI Insight
https://www.readbyqxmd.com/read/28878122/wnt11-regulates-cardiac-chamber-development-and-disease-during-perinatal-maturation
#16
Marlin Touma, Xuedong Kang, Fuying Gao, Yan Zhao, Ashley A Cass, Reshma Biniwale, Xinshu Xiao, Mansuoreh Eghbali, Giovanni Coppola, Brian Reemtsen, Yibin Wang
Ventricular chamber growth and development during perinatal circulatory transition is critical for functional adaptation of the heart. However, the chamber-specific programs of neonatal heart growth are poorly understood. We used integrated systems genomic and functional biology analyses of the perinatal chamber specific transcriptome and we identified Wnt11 as a prominent regulator of chamber-specific proliferation. Importantly, downregulation of Wnt11 expression was associated with cyanotic congenital heart defect (CHD) phenotypes and correlated with O2 saturation levels in hypoxemic infants with Tetralogy of Fallot (TOF)...
September 7, 2017: JCI Insight
https://www.readbyqxmd.com/read/28877886/pulmonary-right-ventricular-resynchronization-in-congenital-heart-disease-acute-improvement-in-right-ventricular-mechanics-and-contraction-efficiency
#17
Jan Janoušek, Jan Kovanda, Miroslav Ložek, Viktor Tomek, Pavel Vojtovič, Roman Gebauer, Peter Kubuš, Miroslav Krejčíř, Joost Lumens, Tammo Delhaas, Frits Prinzen
BACKGROUND: Electromechanical discoordination may contribute to long-term pulmonary right ventricular (RV) dysfunction in patients after surgery for congenital heart disease. We sought to evaluate changes in RV function after temporary RV cardiac resynchronization therapy. METHODS AND RESULTS: Twenty-five patients aged median 12.0 years after repair of tetralogy of Fallot and similar lesions were studied echocardiographically (n=23) and by cardiac catheterization (n=5) after primary repair (n=4) or after surgical RV revalvulation for significant pulmonary regurgitation (n=21)...
September 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28874283/mortality-in-adults-with-congenital-heart-disease
#18
Pavithra Naidu, Leeanne Grigg, Dominica Zentner
AIM: Retrospective ascertainment of the causes of mortality in the adult congenital heart disease (ACHD) cohort of the Royal Melbourne Hospital (RMH). METHODS: Deceased patients (n=73) of the 2519 ACHD patients in the Royal Melbourne Hospital registry (commenced in 1991) were identified. Retrospective analysis was undertaken. Age, gender of deceased individuals, and frequency and cause of death in different congenital diagnosis groups was explored. RESULTS: Between 1991 and 2015, death occurred in 3...
October 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28871660/clinical-echocardiographic-and-therapeutic-aspects-of-congenital-heart-diseases-of-children-at-douala-general-hospital-a-cross-sectional-study-in-sub-saharan-africa
#19
Félicité Kamdem, Danielle Kedy Koum, Ba Hamadou, Mélanie Yemdji, Henry Luma, Marie Solange Doualla, Diomède Noukeu, Esther Barla, Christophe Akazong, Anastase Dzudie, Henry Ngote, Yves Monkam, Sidiki Mouliom, Samuel Kingue
INTRODUCTION: Cardiovascular diseases in pediatric pathologies have emerged in the recent years in sub-Saharan Africa (SSA), with congenital heart diseases (CHDs) being the most frequent. Unfortunately, their diagnosis is usually delayed, thereby increasing childhood morbidity and mortality. OBJECTIVES: Describe the clinical, echocardiographic, and therapeutic aspects of CHDs of children at Douala General Hospital. METHODS: We carried out a cross-sectional descriptive study over a 10-year period, from January 2006 to December 2015...
September 4, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28870391/determinants-of-adverse-outcomes-after-systemic-to-pulmonary-shunts-in-biventricular-circulation
#20
Ben Hobbes, Yves d'Udekem, Diana Zannino, Igor E Konstantinov, Christian Brizard, Johann Brink
BACKGROUND: Systemic-to-pulmonary shunts are useful palliative procedures, although many teams have been deterred by high mortality and morbidity. We aimed to identify predictors of adverse outcomes after shunts in biventricular lesions. METHODS: From 2004 to 2014, 173 children had shunt procedures. Morphologies included: tetralogy of Fallot, pulmonary atresia with ventricular septal defect (VSD) with and without major aortopulmonary collaterals (MAPCAs), transposition of great arteries with pulmonary stenosis, and double outlet right ventricle...
September 1, 2017: Annals of Thoracic Surgery
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