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https://www.readbyqxmd.com/read/28719142/changing-prevalence-of-severe-congenital-heart-disease-results-from-the-national-register-for-congenital-heart-defects-in-germany
#1
Constanze Pfitzer, Paul C Helm, Hannah Ferentzi, Lisa-Maria Rosenthal, Ulrike M M Bauer, Felix Berger, Katharina R L Schmitt
OBJECTIVE: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender. DESIGN: Cross-sectional registry study. SETTING: We analyzed data from patients with CHD born between 1996 and 2015. PATIENTS: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic capability for earlier identifying minor lesions)...
July 18, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28714851/pdgfr%C3%AE-functions-in-endothelial-derived-cells-to-regulate-neural-crest-cells-and-development-of-the-great-arteries
#2
Haig Aghajanian, Young Kuk Cho, Nicholas W Rizer, Qiaohong Wang, Li Li, Karl Degenhardt, Rajan Jain
Originating as a single vessel emerging from the embryonic heart, the truncus arteriosus must septate and remodel into the aorta and pulmonary artery to support postnatal life. Defective remodeling or septation leads to abnormalities collectively known as conotruncal defects, which are associated with significant mortality and morbidity. Multiple populations of cells must interact to coordinate outflow tract remodeling, and cardiac neural crest has emerged as particularly important during this process. Abnormalities in cardiac neural crest have been implicated in the pathogenesis of multiple conotruncal defects, including persistent truncus arteriosus, double outlet right ventricle, and tetralogy of Fallot...
July 14, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28713939/microarray-analysis-reveals-key-genes-and-pathways-in-tetralogy-of-fallot
#3
Yue-E He, Hui-Xian Qiu, Jian-Bing Jiang, Rong-Zhou Wu, Ru-Lian Xiang, Yuan-Hai Zhang
The aim of the present study was to identify key genes that may be involved in the pathogenesis of Tetralogy of Fallot (TOF) using bioinformatics methods. The GSE26125 microarray dataset, which includes cardiovascular tissue samples derived from 16 children with TOF and five healthy age‑matched control infants, was downloaded from the Gene Expression Omnibus database. Differential expression analysis was performed between TOF and control samples to identify differentially expressed genes (DEGs) using Student's t‑test, and the R/limma package, with a log2 fold‑change of >2 and a false discovery rate of <0...
July 6, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28712562/abnormal-right-atrial-performance-in-repaired-tetralogy-of-fallot-a-cmr-feature-tracking-analysis
#4
Shelby Kutty, Quanliang Shang, Navya Joseph, Johannes T Kowallick, Andreas Schuster, Michael Steinmetz, David A Danford, Phillip Beerbaum, Samir Sarikouch
BACKGROUND: We hypothesized that right atrial (RA) performance is abnormal in repaired tetralogy of Fallot (TOF). METHODS: TOF patients were prospectively enrolled for cardiovascular magnetic resonance (CMR), echocardiography and exercise stress following a standardized 14-center protocol. Peak RA longitudinal strain (RALS) and right ventricular longitudinal strain (RVLS) were measured using CMR feature tracking (FT) and correlated to RA and RV end diastolic volumes (EDVi) and ejection fraction (EF)...
July 8, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28704916/three-dimensional-echocardiography-in-adult-congenital-heart-disease
#5
REVIEW
Hyun Suk Yang
Congenital heart disease (CHD) is now more common in adults than in children due to improvements in fetal echo, neonatal and pediatric care, and surgical techniques leading to dramatically increased survivability into adulthood. Adult patients with CHD, regardless of prior cardiac surgery, experience further cardiac problems or therapeutic challenges; therefore, a non-invasive, easily accessible echocardiographic examination is an essential follow-up tool. Among echocardiographic modalities, three-dimensional (3D) echocardiography provides better delineation of spatial relationships in complex cardiac geometries and more accurate volumetric information without geometric assumptions...
July 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28702146/are-polymorphisms-in-mtrr-a66g-and-mthfr-c677t-genes-associated-with-congenital-heart-diseases-in-iranian-population
#6
Noormohammad Noori, Ebrahim Miri-Moghaddam, Asieh Dejkam, Yasman Garmie, Ali Bazi
BACKGROUND: The 5, 10-methyleneterahydrofolate reductase (MTHFR) and methionine synthase reductase (MTRR) are two essential enzymes involved in folate metabolism. The relationship between genetic polymorphisms and congenital heart defects (CHDs) is inconsistent. Our aim was to investigate the association between two well-known polymorphisms of MTHFR and MTRR genes, C677T and A66G, respectively, and CHDs in Iranian patients. METHODS: We enrolled 74 patients with ventricular septal defect (VSD) and 79 with tetralogy of fallot (TOF) along with 147 healthy controls...
2017: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/28701598/the-blalock-and-taussig-shunt-revisited
#7
REVIEW
Usha Kiran, Shivani Aggarwal, Arin Choudhary, B Uma, Poonam Malhotra Kapoor
The systemic to pulmonary artery shunts are done as palliative procedures for cyanotic congenital heart diseases ranging from simple tetralogy of Fallots (TOFs)/pulmonary atresia (PA) to complex univentricular hearts. They allow growth of pulmonary arteries and maintain regulated blood flow to the lungs till a proper age and body weight suitable for definitive corrective repair is reached. We have reviewed the BT shunt with its anaesthtic considerations and management of associated complications.
July 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28698799/adults-with-repaired-tetralogy-low-mortality-but-high-morbidity-up-to-middle-age
#8
Mark Dennis, Ben Moore, Irina Kotchetkova, Lynne Pressley, Rachael Cordina, David S Celermajer
OBJECTIVE: Survival of patients with repaired tetralogy of Fallot (rToF) into young adulthood is very good. Concerns exist, however, over long-term morbidity and mortality as these subjects reach middle age. We aimed to assess survival and the prevalence of complications in patients with rToF seen in our Adult Congenital Heart Disease (ACHD) service. METHODS: One hundred and sixty-eight consecutive patients with 'simple rToF', aged over 16 years, followed up at our tertiary-level ACHD service in Sydney, Australia since 2000, were included...
2017: Open Heart
https://www.readbyqxmd.com/read/28697893/mechanical-valves-in-the-pulmonary-position-an-international-retrospective-analysis
#9
Hanna Pragt, Joost P van Melle, Hoda Javadikasgari, Dong Man Seo, John M Stulak, Igor Knez, Jürgen Hörer, Christian Muñoz-Guijosa, Mahyar G Dehaki, Hong Ju Shin, Joseph A Dearani, Maziar G Dehaki, Petronella G Pieper, Christine Eulenburg, Laura Dos, Tjark Ebels
OBJECTIVE: Life expectancy of patients with congenital heart disease has improved over the past decades, increasing the need for a durable pulmonary prosthetic valve. Biological valves in various forms have become the valve of choice for pulmonary valve replacement (PVR), but structural valve deterioration is unavoidable in the long term. Use of a mechanical valve could be an alternative, but data on long-term outcomes are sparse. METHODS: We retrospectively collected and analyzed data on 364 patients with mechanical valves implanted in the pulmonary position between 1965 and 2014...
May 20, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28693530/analysis-of-circulating-micrornas-in-patients-with-repaired-tetralogy-of-fallot-with-and-without-heart-failure
#10
Masood Abu-Halima, Eckart Meese, Andreas Keller, Hashim Abdul-Khaliq, Tanja Rädle-Hurst
BACKGROUND: MicroRNAs (miRNAs) are a class of regulatory RNAs that regulate gene expression post-transcriptionally. Little, however, is known on the expression profile of circulating miRNAs in Tetralogy of Fallot (TOF) patients late after surgical repair. In this study, we aimed to identify the specific patterns of circulating miRNAs in blood of patients with repaired, non-syndromic TOF and to assess whether these specific miRNAs may be useful to differentiate patients with and without heart failure...
July 10, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28685697/does-pharmacological-therapy-still-play-a-role-in-preventing-sudden-death-in-surgically-treated-tetralogy-of-fallot
#11
Bronzetti Gabriele, Brighenti Maurizio, Bonvicini Marco
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, with a familial recurrence risk of 3%. Despite the performing of an optimal surgical repair, TOF patients may feature a poor medium and long-term survival rate: atrial re-entrant tachycardia will develop in more than 30% of patients and high-grade ventricular arrhythmias will be seen in about 10% of patients. These life-threatening arrhythmias and consequent sudden death continue to represent serious complications following TOF repair...
July 7, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28677054/left-ventricular-regional-myocardial-motion-and-twist-function-in-repaired-tetralogy-of-fallot-evaluated-by-magnetic-resonance-tissue-phase-mapping
#12
Meng-Chu Chang, Ming-Ting Wu, Ken-Pen Weng, Mao-Yuan Su, Marius Menza, Hung-Chieh Huang, Hsu-Hsia Peng
OBJECTIVES: We aimed to characterise regional myocardial motion and twist function in the left ventricles (LV) in patients with repaired tetralogy of Fallot (rTOF) and preserved LV global function. METHODS: We recruited 47 rTOF patients and 38 age-matched normal volunteers. Tissue phase mapping (TPM) was performed for evaluating the LV myocardial velocity in longitudinal, radial, and circumferential (Vz, Vr, and VØ) directions in basal, middle, and apical slices...
July 4, 2017: European Radiology
https://www.readbyqxmd.com/read/28675906/early-evaluation-of-the-fetal-heart
#13
Edgar Hernandez-Andrade, Manasi Patwardhan, Mónica Cruz-Lemini, Suchaya Luewan
Evaluation of the fetal heart at 11-13 + 6 weeks of gestation is indicated for women with a family history of congenital heart defects (CHD), a previous child with CDH, or an ultrasound finding associated with cardiac anomalies. The accuracy for early detection of CHD is highly related to the experience of the operator. The 4-chamber view and outflow tracts are the most important planes for identification of an abnormal heart, and can be obtained in the majority of fetuses from 11 weeks of gestation onward...
July 5, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28673366/significance-of-lung-anomalies-in-fetuses-affected-by-tetralogy-of-fallot-with-absent-pulmonary-valve-syndrome
#14
Estelle Tenisch, Marie-Josée Raboisson, Françoise Rypens, Julie Déry, Andrée Grignon, Chantale Lapierre
OBJECTIVES: Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of tetralogy of Fallot with dilatation of large pulmonary arteries. Prognosis is related to the severity of the cardiac malformation and to bronchial tree compression by dilated pulmonary arteries. This study analyses the prenatal echographic lung appearance in fetuses with tetralogy of Fallot with absent pulmonary valve and discusses its significance. METHODS: We carried out a retrospective review of fetal and postnatal files of nine fetuses diagnosed with tetralogy of Fallot with absent pulmonary valve syndrome in our institution...
July 4, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28673109/takotsubo-cardiomyopathy-complicating-percutaneous-pulmonary-valve-implantation-in-a-child
#15
Robert Dalla Pozza, Anja Lehner, Sarah Ulrich, Michael Näbauer, Nikolaus A Haas, B Heineking
Takotsubo cardiomyopathy describes a sudden onset cardiomyopathy with acute impairment of left ventricular function and spontaneous resolution over time. Only a few cases of Takotsubo cardiomyopathy in childhood have been described. We report the case of a 12-year-old girl with repaired tetralogy of Fallot who experienced acute onset of left ventricular dysfunction without coronary arterial involvement, suggesting Takotsubo cardiomyopathy, during an interventional catheterization procedure. Cardiogenic shock necessitated mechanical circulatory support with extracorporeal membrane oxygenator for ten days and mechanical ventilation for 12 days...
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28668430/pixel-wise-derivation-of-pulmonary-regurgitation-index-could-influence-clinical-decision-a-phase-contrast-mr-imaging-study-on-patients-with-repaired-tetralogy-of-fallot
#16
Pei-Hsin Wu, Hsiao-Wen Chung, Ming-Ting Wu, Cheng-Wen Ko
PURPOSE: Regurgitant fraction (RF) measured from 2D phase-contrast MRI has been used as a standard to quantitate the degree of pulmonary regurgitation and serves as a determinant indicator of prognosis for tetralogy of Fallot after surgical repair. This study demonstrated the potential underestimate of RF using the conventional definition and its impact on clinical decision when backward flow occurred during systolic periods. METHODS: Quantitative flow parameters, including forward flow volume (FFV), backward flow volume (BFV), and RF were estimated by two approaches: One derived from conventional ROI-averaged curve (bulk quantity) and the other in a pixel-wise manner to spatially reflect inhomogeneous flow profile (pixel-wise quantity)...
August 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28666664/young-infants-with-severe-tetralogy-of-fallot-early-primary-surgery-versus-transcatheter-palliation
#17
Travis J Wilder, Glen S Van Arsdell, Lee Benson, Eric Pham-Hung, Michael Gritti, Alexandra Page, Christopher A Caldarone, Edward J Hickey
BACKGROUND: Infants with severe tetralogy of Fallot may undergo (1) early primary surgical repair (EARLY) or (2) early transcatheter palliation (CATH) before delayed surgical repair. We compared these strategies with (3) elective single-stage tetralogy of Fallot repair (IDEAL). METHODS: From 2000 to 2012, 453 children underwent tetralogy of Fallot repair (excluding systemic-pulmonary shunts), including 383 in the IDEAL (75%), 42 in the EARLY (9%), and 28 in the CATH (6%) groups...
May 19, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28664568/circulating-microrna-in-patients-with-repaired-tetralogy-of-fallot
#18
Clare Tm Lai, Enders Ko Ng, Pak-Cheong Chow, Ava Kwong, Yiu-Fai Cheung
BACKGROUND: Emerging data suggest that heart-related microRNAs (miRs) may serve as circulating biomarkers of myocardial injury. We aimed to determine the circulating profile of miRs in patients with volume-overloaded right ventricles after repair of tetralogy (TOF). METHODS: A total of 104 TOF patients and 70 controls were recruited. The study was conducted into two phases: I) determination of circulating heart-related miRs described in left heart diseases (miR-1, miR-133a, miR208a, miR-208b and miR423-5p) by quantitative real time-PCR in 49 patients and 30 controls and followed by validation in an independent cohort of 55 patients and 40 controls; II) expression profiling of serum samples from 8 patients and 8 controls, followed by validation...
June 29, 2017: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28662918/reproducibility-of-cardiac-magnetic-resonance-imaging-cmri-derived-right-ventricular-parameters-in-repaired-tetralogy-of-fallot-tof
#19
Ganesh Kumar Gnanappa, Imran Rashid, David Celermajer, Julian Ayer, Rajesh Puranik
BACKGROUND: Quantification of right ventricular (RV) volumes is challenging owing to variable reproducibility and is especially so in congenital heart disease. Cardiac magnetic resonance (CMR) has the ability to more comprehensively survey the entire right ventricle and is currently considered the gold standard. AIMS: We aimed to determine the inter-observer reproducibility of CMR-derived RV volumes generated by two independent and experienced (SCMR Level III) observers in Tetralogy of Fallot (ToF) patients with varying degrees of RV dilatation...
June 3, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28657453/tetralogy-of-fallot-with-fifth-aortic-arch
#20
Ming-Chun Yang, Shu-Chien Huang, Ming-Tai Lin, Jou-Kou Wang
No abstract text is available yet for this article.
June 28, 2017: Acta Cardiologica
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