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https://www.readbyqxmd.com/read/29468314/recent-advances-and-trends-in-pediatric-cardiac-imaging
#1
REVIEW
Wadi Mawad, Luc L Mertens
Cardiac imaging is central to today's pediatric cardiology practice not only to diagnose structural congenital defects and delineate cardiac and extracardiac anatomy but also for determining the hemodynamic impact of the structural defects and acquired pediatric diseases. Not so long ago, clinicians had to heavily rely on angiography as the main cardiac imaging modality to visualize the heart. Particularly, the development of echocardiography in the 1970s and 1980s together with the development of magnetic resonance imaging (MRI) and computed tomography (CT) resulted in a non-invasive diagnostic revolution with diagnostic catheterization becoming obsolete apart for very specific indications...
February 21, 2018: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29461882/genetic-analyses-identified-a-sall4-gene-mutation-associated-with-holt-oram-syndrome
#2
Bojian Li, Sun Chen, Kun Sun, Rang Xu, Yurong Wu
Holt-Oram syndrome (HOS) is an autosomal dominant disorder, which is characterized by deformities of upper limbs and congenital heart defects. Alterations of TBX5 gene have been identified to be the leading cause of HOS, while some cases could not be explained by TBX5 mutations. In our study, we preliminarily diagnosed a newborn baby, who had Tetralogy of Fallot, thumb agenesis, facial dysplasia, and right ear canal malformation, as HOS. Chromosome microarray analyses showed no pathological deletions or replications of chromosome segments; whole exome sequencing screened out six candidate genes that were involved in cardiac diseases or syndromes among which SALL4 has been reported as HOS related gene...
February 20, 2018: DNA and Cell Biology
https://www.readbyqxmd.com/read/29460287/chromosomal-aberrations-and-cnvs-in-twin-fetuses-with-cardiovascular-anomalies-comparison-between-monochorionic-diamniotic-and-dichorionic-diamniotic-twins
#3
Yi Zhang, Linhuan Huang, Xuan Huang, Zhiming He, Shaobin Lin, Ye Wang, Lin Li, Yanmin Luo, Qun Fang
OBJECTIVE: To investigate the types of cardiovascular anomalies and the results of invasive prenatal diagnosis in twin fetuses. METHODS: A total of 298 fetuses in 149 twin pairs were enrolled, in which 1 or 2 fetuses of a twin pair had cardiovascular anomalies. Prenatal diagnosis was performed on 290 fetuses of 149 twin pairs, including 150 monochorionic diamniotic (MCDA) fetuses (79 pairs) and 140 dichorionic diamniotic (DCDA) fetuses (70 pairs). G-banding karyotyping and/or chromosomal microarray analysis (CMA) were performed...
February 19, 2018: Prenatal Diagnosis
https://www.readbyqxmd.com/read/29455964/polytetrafluoroethylene-conduits-versus-homografts-for-right-ventricular-outflow-tract-reconstruction-in-infants-and-young-children-an-institutional-experience
#4
Christopher W Mercer, Shawn C West, Mahesh S Sharma, Masahiro Yoshida, Victor O Morell
OBJECTIVE: Our institution uses a valved polytetrafluoroethylene conduit as an alternative to homografts. The objective of this study was to investigate the performance of bicuspid valved polytetrafluoroethylene conduits used for right ventricular outflow tract reconstruction in children aged less than 2 years and to evaluate risk factors for earlier conduit explant. METHODS: We performed an Institutional Review Board-approved retrospective chart review of all patients aged less than 2 years who underwent surgical right ventricular outflow tract reconstruction with a bicuspid valved polytetrafluoroethylene conduit or homograft conduit from July 2004 to December 2014...
January 31, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29455823/sequential-melody-valve-insertion-after-repair-of-tetralogy-of-fallot-atrioventricular-septal-defect
#5
Damien Kenny, Terence Prendiville, Kevin P Walsh, Jonathan McGuinness
Repair of tetralogy of Fallot with atrioventricular septal defect may be associated with hemodynamic challenges in the postoperative period particularly as left atrial hypertension secondary to left atrioventricular valve dysfunction may exacerbate pulmonary regurgitation and augment low cardiac output. We present a case describing hybrid strategies to treat severe left atrioventricular and pulmonary valve regurgitation with modified balloon expandable stent valves to counter low cardiac output secondary to valve dysfunction...
March 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29455794/effect-of-ventricular-function-and-volumes-on-exercise-capacity-in-adults-with-repaired-tetralogy-of-fallot
#6
Natalia Dłużniewska, Piotr Podolec, Tomasz Miszalski-Jamka, Maciej Krupiński, Paweł Banyś, Małgorzata Urbańczyk, Bogdan Suder, Grzegorz Kopeć, Maria Olszowska, Lidia Tomkiewicz-Pająk
OBJECTIVES: Investigate the effects of left and right ventricular function and severity of pulmonary valve regurgitation, quantified by cardiac magnetic resonance (CMR), on exercise tolerance in adult patients who underwent ToF repair at a young age. METHODS: This is a retrospective cohort study of 52 patients after ToF surgery and 33 age- and sex-matched healthy volunteers. CMR and cardiopulmonary exercise testing (CPET) were performed on all patients; CPET was performed on control subjects...
January 2018: Indian Heart Journal
https://www.readbyqxmd.com/read/29453683/flow-energy-loss-as-a-predictive-parameter-for-right-ventricular-deterioration-caused-by-pulmonary-regurgitation-after-tetralogy-of-fallot-repair
#7
Miyuki Shibata, Keiichi Itatani, Taiyu Hayashi, Takashi Honda, Atsushi Kitagawa, Kagami Miyaji, Minoru Ono
The optimal timing for pulmonary valve replacement after Tetralogy of Fallot (TOF) repair remains controversial. In this study, we estimated the feasibility of using flow energy loss (FEL) to predict right ventricular (RV) deterioration due to pulmonary regurgitation after TOF repair. We examined RV outflow tract (RVOT) flow in nine patients who underwent TOF or double-outlet right ventricle repair in the intervention group (Group I) and compared them with three healthy children in the control group (Group C)...
February 16, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29444724/early-postoperative-remodelling-following-repair-of-tetralogy-of-fallot-utilising-unsedated-cardiac-magnetic-resonance-a-pilot-study
#8
Michael P DiLorenzo, Elizabeth Goldmuntz, Susan C Nicolson, Mark A Fogel, Laura Mercer-Rosa
Introduction The right ventricular adaptations early after surgery in infants with tetralogy of Fallot are important to understand the changes that occur later on in life; this physiology has not been fully delineated. We sought to assess early postoperative right ventricular remodelling in patients with tetralogy of Fallot by cardiac MRI. Materials and method Subjects with tetralogy of Fallot under 1 year of age were recruited following complete surgical repair for tetralogy of Fallot. Protocol-based cardiac MRI to assess anatomy, function, and flows was performed before hospital discharge using the feed and sleep technique, an unsedated imaging technique...
February 15, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29434740/a-pilot-study-of-a-cardiovascular-virtual-endoscopy-system-based-on-multi-detector-computed-tomography-in-diagnosing-tetralogy-of-fallot-in-pediatric-patients
#9
Li-Ping Yao, Li Zhang, Ju Mei, Fang-Bao Ding, Hui-Ming Li, Ming Ding, Xin Yang, Xiao-Ming Li, Kun Sun
The present study aimed to investigate the capabilities of the cardiovascular virtual endoscopy (VE) system in diagnosing tetralogy of Fallot (TOF) and performing measurements. A total of 37 patients underwent two-dimensional echocardiography (2-DE) and multi-detector computed tomography (MDCT) examinations. The obtained MDCT images were applied to a cardiovascular VE system. Diagnostic time by VE was first studied and compared with MDCT. Subsequently, with surgical findings as the ground truth, the capabilities of VE, 2-DE and MDCT in diagnosing TOF and its complications were investigated...
February 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29433731/relation-of-increased-epicardial-fat-after-fontan-palliation-to-cardiac-output-and-systemic-ventricular-ejection-fraction
#10
Adam M Lubert, Jimmy C Lu, Albert P Rocchini, Mark D Norris, Sunkyung Yu, Prachi P Agarwal, Maryam Ghadimi Mahani, Adam L Dorfman
Epicardial fat produces multiple proinflammatory cytokines and is associated with adverse cardiovascular events. Inflammation and resultant endothelial dysfunction may play a role in progressive myocardial dysfunction among adults with single ventricle physiology after Fontan palliation, but the potential impact of increased epicardial fat volume (EFV) has not been studied. This study sought to determine if there is greater EFV in Fontan patients compared with a group of repaired tetralogy of Fallot (rTOF) patients...
January 9, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29433351/transcatheter-pulmonary-valve-implantation-will-it-replace-surgical-pulmonary-valve-replacement
#11
Ahmed Kheiwa, Punag Divanji, Vaikom S Mahadevan
Right ventricular outflow tract (RVOT) dysfunction is a common hemodynamic challenge for adults with congenital heart disease (ACHD), including patients with repaired tetralogy of Fallot (TOF), truncus arteriosus (TA), and those who have undergone the Ross procedure for congenital aortic stenosis and the Rastelli repair for transposition of great vessels. Pulmonary valve replacement (PVR) has become one of the most common procedures performed for ACHD patients. Areas covered: Given the advances in transcatheter technology, we conducted a detailed review of the available studies addressing the indications for PVR, historical background, evolving technology, procedural aspects, and the future direction, with an emphasis on ACHD patients...
February 12, 2018: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/29432830/functional-polymorphisms-of-the-neuropilin-1-gene-are-associated-with-the-risk-of-tetralogy-of-fallot-in-a-chinese-han-population
#12
Sai-Hou Fan, Zhen-Ya Shen, Yi-Min Xiao
Tetralogy of Fallot (TOF) is one of the most severe forms of cyanotic congenital heart disease (CHD) and is also the most common. Previous genome-wide association study (GWAS) and replication studies have suggested that a polymorphism in the neuropilin 1 (NRP1) gene is significantly associated with the risk of TOF. To further confirm the association between the NRP1 polymorphism and the risk of TOF and to identify additional positive functional single-nucleotide polymorphisms (SNPs) for TOF risk, we systematically screened for functional polymorphisms throughout the regulatory and coding regions of the NRP1 gene...
February 9, 2018: Gene
https://www.readbyqxmd.com/read/29425528/comprehensive-management-of-major-aortopulmonary-collaterals-in-the-repair-of-tetralogy-of-fallot
#13
REVIEW
Michael Ma, Richard D Mainwaring, Frank L Hanley
The heterogenous anatomy of Tetralogy of Fallot with major aortopulmonary collateral arteries has engendered a similar degree of diversity in its management and, ultimately, outcome. We summarize our comprehensive treatment paradigm for this lesion evolved over 15 years of experience through 458 patients and the results obtained. An updated analysis of 307 patients treated primarily at our institution is included. A review of recent literature, comparison of management strategies, and discussion of ongoing controversies are provided...
March 2018: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://www.readbyqxmd.com/read/29416223/pregnacy-and-delivery-in-a-patient-after-tetralogy-of-fallot-surgery-being-undergone
#14
Anis Cerovac, Dzenita Ljuca, Bedreldin Khodary, Gordana Grgic
Aim: The aim of the paper is to present the risk of pregnancy for mother and her child in a young patient who had a surgery to repair Tetralogy of Fallot (ToF), who gave a birth to her firstborn by having a cesarean section. Case report: 23 years old patient, in 28 weeks of pregnancy was admitted to the clinic due to her medical record. She did not have any subjective complaints. She had two surgeries to repair ToF. After the surgery, she had residual ventricular septal defect (VSD)...
February 2018: Medical Archives
https://www.readbyqxmd.com/read/29408326/the-role-of-ablation-therapy-for-ventricular-tachycardia-in-patients-with-tetralogy-of-fallot
#15
EDITORIAL
Edward P Walsh
No abstract text is available yet for this article.
February 2, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29397465/3d-printing-provides-a-precise-approach-in-the-treatment-of-tetralogy-of-fallot-pulmonary-atresia-with-major-aortopulmonary-collateral-arteries
#16
REVIEW
Shafkat Anwar, Toby Rockefeller, Demetrios A Raptis, Pamela K Woodard, Pirooz Eghtesady
Patients with tetralogy of Fallot, pulmonary atresia, and multiple aortopulmonary collateral arteries (Tet PA MAPCAs) have a wide spectrum of anatomy and disease severity. Management of these patients can be challenging and often require multiple high-risk surgical and interventional catheterization procedures. These interventions are made challenging by complex anatomy that require the proceduralist to mentally reconstruct three-dimensional anatomic relationships from two-dimensional images. Three-dimensional (3D) printing is an emerging medical technology that provides added benefits in the management of patients with Tet PA MAPCAs...
February 3, 2018: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29395214/transcatheter-pulmonary-valve-replacement-using-the-melody-valve-for-treatment-of-dysfunctional-surgical-bioprostheses-a-multicenter-study
#17
Allison K Cabalka, Jeremy D Asnes, David T Balzer, John P Cheatham, Matthew J Gillespie, Thomas K Jones, Henri Justino, Dennis W Kim, Te-Hsin Lung, Daniel R Turner, Doff B McElhinney
BACKGROUND: Stented bioprosthetic valves (BPVs) are commonly used for surgical pulmonary valve (PV) replacement in postoperative congenital heart disease, but develop structural failure in a time-related fashion. The Melody transcatheter PV (TPV) (Medtronic, Minneapolis, Minn) has been used to treat BPV dysfunction, but there have been few studies in this population. METHODS: We performed a retrospective, multicenter study to evaluate Melody valve function in patients who underwent TPV replacement (TPVR) into a dysfunctional pulmonary BPV...
December 6, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29377646/stillborn-infants-associated-malformations
#18
Lewis B Holmes, Hanah Nasri, Rebecca Beroukhim, Anne-Therese Hunt, Drucilla J Roberts, M Hassan Toufaily, Marie-Noel Westgate
BACKGROUND: Stillbirth, defined as death of a fetus in utero after 20 weeks of gestation, occurs in 1 to 2% of pregnancies in the United States. Many of these stillborn infants have associated malformations, including chromosome abnormalities, neural tube defects, and malformation syndromes. Other causes are abnormalities of the placenta and maternal conditions, such as pre-eclampsia and obesity. A consecutive sample of malformed stillborn infants can establish the relative frequency and severity of the associated malformations...
January 2018: Birth Defects Research
https://www.readbyqxmd.com/read/29365090/long-term-results-of-pulmonary-valve-annular-enlargement-with-valve-repair-in-tetralogy-of-fallot
#19
Hyungtae Kim, Si Chan Sung, Kwang Ho Choi, Hyoung Doo Lee, Geena Kim, Hoon Ko, Young Seok Lee
OBJECTIVES: We adopted an operative technique of pulmonary valve (PV) annular enlargement with valve repair in tetralogy of Fallot (TOF) correction to reduce postoperative pulmonary regurgitation (PR) 16 years ago. Here, we have evaluated the long-term results. METHODS: Between April 2000 and August 2005, 43 patients (26 men) with tetralogy of Fallot with pulmonary stenosis underwent PV annular enlargement with valve repair. The median age and body weight at the time of surgery were 14 months and 10...
January 22, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29363855/nrp1-haploinsufficiency-predisposes-to-the-development-of-tetralogy-of-fallot
#20
Ivan Duran, Jessica Tenney, Carmen M Warren, Anna Sarukhanov, Fabiana Csukasi, Mark Skalansky, Maria L Iruela-Arispe, Deborah Krakow
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. It involves anatomical abnormalities that change the normal flow of blood through the heart resulting in low oxygenation. Although not all of the underlying causes of TOF are completely understood, the disease has been associated with varying genetic etiologies including chromosomal abnormalities and Mendelian disorders, but can also occur as an isolated defect. In this report, we describe a familial case of TOF associated with a 1...
January 24, 2018: American Journal of Medical Genetics. Part A
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