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https://www.readbyqxmd.com/read/28936780/ct-based-spect-attenuation-correction-and-assessment-of-infarct-size-results-from-a-cardiac-phantom-study
#1
Alexander Stephan Kroiss, Stephan Gerhard Nekolla, Georg Dobrozemsky, Thomas Grubinger, Barry Lynn Shulkin, Markus Schwaiger
RATIONALE: Myocardial perfusion SPECT is a commonly performed, well established, clinically useful procedure for the management of patients with coronary artery disease. However, the attenuation of photons from myocardium impacts the quantification of infarct sizes. CT-Attenuation Correction (AC) potentially resolves this problem. This contention was investigated by analyzing various parameters for infarct size delineation in a cardiac phantom model. METHODS: A thorax phantom with a left ventricle (LV), fillable defects, lungs, spine and liver was used...
September 21, 2017: Annals of Nuclear Medicine
https://www.readbyqxmd.com/read/28935571/aggravated-post-infarct-heart-failure-in-type-2-diabetes-is-associated-with-impaired-mitophagy-and-exaggerated-inflammasome-activation
#2
Thota Durga Devi, Mohan Babu, Petri Mäkinen, Minna Kaikkonen, Merja Heinaniemi, Hanne Laakso, Elias Ylä-Herttuala, Lassi Rieppo, Timo Liimatainen, Nikolay Naumenko, Pasi Tavi, Seppo Ylä-Herttuala
Type 2 diabetes mellitus (T2DM) is a major risk factor for heart disease. Mortality rates following myocardial infarction (MI) are significantly increased in T2DM patients due to dysfunctional left ventricle (LV). However, molecular pathways underlying accelerated post-MI heart failure (HF) in T2DM remain unclear. We investigated the underlying mechanisms by inducing MI in a well-established model of T2DM and control mice. Cardiac imaging revealed a significantly decreased global LV ejection fraction (EF) in parallel with increased mortality post-MI in T2DM mice compared to controls...
September 18, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28932757/a-five-repeat-micro-dystrophin-gene-ameliorated-dystrophic-phenotype-in-the-severe-dba-2j-mdx-model-of-duchenne-muscular-dystrophy
#3
Chady H Hakim, Nalinda B Wasala, Xiufang Pan, Kasun Kodippili, Yongping Yue, Keqing Zhang, Gang Yao, Brittney Haffner, Sean X Duan, Julian Ramos, Joel S Schneider, N Nora Yang, Jeffrey S Chamberlain, Dongsheng Duan
Micro-dystrophins are highly promising candidates for treating Duchenne muscular dystrophy, a lethal muscle disease caused by dystrophin deficiency. Here, we report robust disease rescue in the severe DBA/2J-mdx model with a neuronal nitric oxide synthase (nNOS)-binding micro-dystrophin vector. 2 × 10(13) vector genome particles/mouse of the vector were delivered intravenously to 10-week-old mice and were evaluated at 6 months of age. Saturated micro-dystrophin expression was detected in all skeletal muscles and the heart and restored the dystrophin-associated glycoprotein complex and nNOS...
September 15, 2017: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/28932688/alg9-cdg-new-clinical-case-and-review-of-the-literature
#4
Kellie Davis, Duncan Webster, Chris Smith, Sheryl Jackson, David Sinasac, Lorne Seargeant, Xing-Chang Wei, Patrick Ferreira, Julian Midgley, Yolanda Foster, Xueli Li, Miao He, Walla Al-Hertani
Congenital disorders of glycosylation (CDG) are a group of metabolic diseases resulting from defects in glycan synthesis or processing. The number of subgroups and their phenotypic spectrums continue to expand with most related to deficiencies of N-glycosylation. ALG9-CDG (previously CDG-IL) is the result of a mutation in ALG9. This gene encodes the enzyme alpha-1,2-mannosyltransferase. To date, a total of 10 patients from 6 different families have been reported with one of four ALG9 mutations. Seven of these patients had a similar phenotype with failure to thrive, dysmorphic features, seizures, hepatic and/or renal cysts; the other three patients died in utero from a lethal skeletal dysplasia...
December 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28931652/responsive-monitoring-of-mitochondrial-redox-states-in-heart-muscle-predicts-impending-cardiac-arrest
#5
Dorothy A Perry, Joshua W Salvin, Padraic Romfh, Peili Chen, Kalyani Krishnamurthy, Lindsay M Thomson, Brian D Polizzotti, Francis X McGowan, Daryoosh Vakhshoori, John N Kheir
Assessing the adequacy of oxygen delivery to tissues is vital, particularly in the fields of intensive care medicine and surgery. As oxygen delivery to a cell becomes deficient, changes in mitochondrial redox state precede changes in cellular function. We describe a technique for the continuous monitoring of the mitochondrial redox state on the epicardial surface using resonance Raman spectroscopy. We quantify the reduced fraction of specific electron transport chain cytochromes, a metric we name the resonance Raman reduced mitochondrial ratio (3RMR)...
September 20, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28928613/clarifying-the-anatomy-and-physiology-of-totally-anomalous-systemic-venous-connection
#6
REVIEW
Saurabh Kumar Gupta, Rajnish Juneja, Robert H Anderson, Gurpreet S Gulati, Velayoudam Devagorou
The description of totally anomalous systemic venous connection is limited to case reports. In this review, we seek to clarify anatomic, physiologic, and hemodynamic aspects of this extremely rare anomaly. We also present findings of two patients in whom connection of all the systemic veins was anomalous. In the first patient, with usual atrial arrangement, all systemic veins, including the coronary sinus, were connected anomalously to the morphologically left atrium. Limited left-to-right shunt across an atrial septal defect provided the only source of blood flow to the lungs...
September 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28928610/does-heart-type-fatty-acid-binding-protein-predict-clinical-outcomes-after-pediatric-cardiac-surgery
#7
Egmond S Evers, Varsha Walavalkar, Suresh Pujar, Latha Balasubramanian, Frits W Prinzen, Tammo Delhaas, Ward Y Vanagt, Shreesha Maiya
INTRODUCTION: The early identification of vulnerable pediatric cardiac surgery patients can help clinicians provide them with timely support. Heart-type fatty acid-binding protein. (H-FABP) is an early biomarker of myocardial injury in acute myocardial infarction in adults. In this study, we evaluated the correlations between postoperative H-FABP, creatine kinase-myocardial band (CK-MB), troponin-I, total bypass time, and clinical outcomes. METHODS: In 32 pediatric patients that underwent ventricular septal defect...
September 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28924218/uncontrolled-angiogenic-precursor-expansion-causes-coronary-artery-anomalies-in-mice-lacking-pofut1
#8
Yidong Wang, Bingruo Wu, Pengfei Lu, Donghong Zhang, Brian Wu, Shweta Varshney, Gonzalo Del Monte-Nieto, Zhenwu Zhuang, Rabab Charafeddine, Adam H Kramer, Nicolas E Sibinga, Nikolaos G Frangogiannis, Richard N Kitsis, Ralf H Adams, Kari Alitalo, David J Sharp, Richard P Harvey, Pamela Stanley, Bin Zhou
Coronary artery anomalies may cause life-threatening cardiac complications; however, developmental mechanisms underpinning coronary artery formation remain ill-defined. Here we identify an angiogenic cell population for coronary artery formation in mice. Regulated by a DLL4/NOTCH1/VEGFA/VEGFR2 signaling axis, these angiogenic cells generate mature coronary arteries. The NOTCH modulator POFUT1 critically regulates this signaling axis. POFUT1 inactivation disrupts signaling events and results in excessive angiogenic cell proliferation and plexus formation, leading to anomalous coronary arteries, myocardial infarction and heart failure...
September 18, 2017: Nature Communications
https://www.readbyqxmd.com/read/28923324/regenerating-heart-using-a-novel-compound-and-human-wharton-jelly-mesenchymal-stem-cells
#9
Shahram Rabbani, Masoud Soleimani, Mohammad Imani, Mohammad Sahebjam, Ali Ghiaseddin, Seyed Mahdi Nassiri, Jalil Majd Ardakani, Maryam Tajik Rostami, Arash Jalali, Bahmanshir Mousanassab, Mahsa Kheradmandi, Seyed Hossein Ahmadi Tafti
BACKGROUND: Myocardial infarction is a major problem in health system and most conventional therapy is not led to restoration of the health. Stem cell therapy is a method to regenerate the heart but today appropriate cell source and scaffold selection as extracellular matrix to achieve the best effect is disputing. AIM OF THE STUDY: In this study a combination of human Wharton jelly mesenchymal stem cells (HWJMSCs) with a novel compound consisting polyethylene glycol (PEG), hyaluronic acid and chitosan is presented to heart regeneration...
April 2017: Archives of Medical Research
https://www.readbyqxmd.com/read/28919320/is-selective-echocardiography-in-duodenal-atresia-the-future-standard-of-care
#10
Abid Khan, Sharman Tan Tanny, Elizabeth J Perkins, Rodney W Hunt, John M Hutson, Sebastian K King, Bryn Jones, Warwick J Teague
BACKGROUND: Duodenal atresia (DA) is associated with cardiac defects that may have perioperative care implications. Standard preoperative care includes echocardiography to identify such cardiac defects, but this dogma has been challenged. We aimed to assess selective and selective strategies for preoperative echocardiography in DA patients. METHODS: Single-center retrospective review of neonates with DA over a 16-year period was performed. Data included preoperative cardiovascular and respiratory examination, chest x-ray, and echocardiography...
September 2, 2017: Journal of Pediatric Surgery
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
#11
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/28917066/effectiveness-of-the-critical-congenital-heart-disease-screening-program-for-early-diagnosis-of-cardiac-abnormalities-in-newborn-infants
#12
Abdulmajid M Almawazini, Hamdi K Hanafi, Hasan A Madkhali, Noura B Majrashi
To evaluate the effectiveness of critical congenital heart disease (CCHD) screening program for early diagnosis of cardiac anomalies in newborn infants.  Methods: This is a hospital-based prospective cross-sectional study conducted in the Pediatric and Neonatology Department, King Fahad Hospital at  Albaha, Saudi Arabia, between February 2016 and February 2017. Results: We screened 2961 (95.4%) of 3103 patients in a nursery unit; 142 (4.6%) patients were not screened. The test was positive in 114 (3.9%) patients and negative in 2847 (96...
October 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28915332/obesity-and-cardiometabolic-defects-in-heart-failure-pathology
#13
Ganesh V Halade, Vasundhara Kain
Obesity is a major global epidemic that sets the stage for diverse multiple pathologies, including cardiovascular disease. The obesity-related low-grade chronic inflamed milieu is more pronounced in aging and responsive to cardiac dysfunction in heart failure pathology. Metabolic dysregulation of obesity integrates with immune reservoir in spleen and kidney network. Therefore, an integrative systems biology approach is necessary to delay progressive cardiac alternations. The purpose of this comprehensive review is to largely discuss the impact of obesity on the cardiovascular pathobiology in the context of problems and challenges, with major emphasis on the diversified models, and to study cardiac remodeling in obesity...
September 12, 2017: Comprehensive Physiology
https://www.readbyqxmd.com/read/28912723/increased-hemodynamic-load-in-early-embryonic-stages-alters-myofibril-and-mitochondrial-organization-in-the-myocardium
#14
Madeline Midgett, Claudia S López, Larry David, Alina Maloyan, Sandra Rugonyi
Normal blood flow is essential for proper heart formation during embryonic development, as abnormal hemodynamic load (blood pressure and shear stress) results in cardiac defects seen in congenital heart disease (CHD). However, the detrimental remodeling processes that relate altered blood flow to cardiac malformation and defects remain unclear. Heart development is a finely orchestrated process with rapid transformations that occur at the tissue, cell, and subcellular levels. Myocardial cells play an essential role in cardiac tissue maturation by aligning in the direction of stretch and increasing the number of contractile units as hemodynamic load increases throughout development...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28912041/postoperative-b-type-natriuretic-peptide-monitoring-for-the-assessment-of-the-magnitude-of-shunting-through-blalock-taussig-anastomoses
#15
Cristian Mirabile, Alessandra Mazzola, Liana Valeanu, Vanessa Lopez-Lopez, Claudio Barbanti, Camilla Biselli, Carole Hennequin, Sophie Malekzadeh-Milani, Mirela Bojan
BACKGROUND: The Modified Blalock-Taussig shunt (MBTS) is the most common palliative operation performed in patients with complex cardiac defects. Postoperative morbidity and mortality rates are high, mainly due to shunt thrombosis and over-shunting. Over-shunting may be difficult to identify postoperatively based on conventional criteria. Since plasma B-type natriuretic peptide (BNP) concentrations correlate with the magnitude of shunting in various left-to-right shunt cardiac defects, we investigated its ability to identify postoperative MBTS over-shunting...
September 6, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28911200/homozygous-eef1a2-mutation-causes-dilated-cardiomyopathy-failure-to-thrive-global-developmental-delay-epilepsy-and-early-death
#16
Siqi Cao, Laura L Smith, Sergio R Padilla-Lopez, Brandon S Guida, Elizabeth Blume, Jiahai Shi, Sarah U Morton, Catherine A Brownstein, Alan H Beggs, Michael C Kruer, Pankaj B Agrawal
Eukaryotic elongation factor 1A (EEF1A), is encoded by two distinct isoforms, EEF1A1 and EEF1A2; whereas EEF1A1 is expressed almost ubiquitously, EEF1A2 expression is limited such that it is only detectable in skeletal muscle, heart, brain and spinal cord. Currently, the role of EEF1A2 in normal cardiac development and function is unclear. There have been several reports linking de novo dominant EEF1A2 mutations to neurological issues in humans. We report a pair of siblings carrying a homozygous missense mutation p...
September 15, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28904928/prenatal-sonographic-diagnosis-of-hypoplastic-left-heart-syndrome
#17
Raghu Teja Sadineni, B Santh Kumar, N B Chander, Durga Mahita Boppana
Hypoplastic left heart syndrome (HLHS) represents a variety of cardiac malformations that may result from errors in the early stages of cardiac development. HLHS includes a wide spectrum of cardiac malformations including hypoplasia of the left ventricle, ascending aorta, hypoplasia, or atresia of the aortic and mitral valves. Over the recent years, the improved resolution of advanced equipment with awareness and increased performance of second-trimester ultrasound examinations for the assessment of fetal anomalies have helped in understanding the spectrum and have expanded our knowledge of HLHS...
July 2017: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/28899882/chromosome-15-structural-abnormalities-effect-on-igf1r-gene-expression-and-function
#18
Rossella Cannarella, Teresa Mattina, Rosita A Condorelli, Laura M Mongioì, Giuseppe Pandini, Sandro La Vignera, Aldo E Calogero
Insulin-like growth factor 1 receptor (IGF1R), mapping on the 15q26.3 chromosome, is required for normal embryonic and postnatal growth. The aim of the present study was to evaluate the IGF1R gene expression and function in three unrelated patients with chromosome 15 structural abnormalities. We report two male patients with the smallest 15q26.3 chromosome duplication described so far, and a female patient with ring chromosome 15 syndrome. Patient one, with a 568 kb pure duplication, had overgrowth, developmental delay, mental and psychomotor retardation, obesity, cryptorchidism, borderline low testis volume, severe oligoasthenoteratozoospermia and gynecomastia...
October 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28899436/educational-level-and-employment-status-in-adults-with-congenital-heart-disease
#19
Constanze Pfitzer, Paul C Helm, Lisa-Maria Rosenthal, Christoph Walker, Hannah Ferentzi, Ulrike M M Bauer, Felix Berger, Katharina R L Schmitt
Purpose Through this study we aimed to assess the educational level and employment status of adults with CHD in Germany. METHODS: Data were acquired from an online survey carried out in 2015 by the German National Register for Congenital Heart Defects. A total of 1458 adults with CHD participated in the survey (response rate: 37.6%). For 1198 participants, detailed medical information, such as main cardiac diagnosis and information from medical reports, was available. RESULTS: Of the participants surveyed (n=1198), 54...
September 13, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28898459/transgastric-a-forgotten-view-for-atrial-septal-defect-device-closure-procedures
#20
Clifford L Cua, Amy L Cooper, Marc Dutro, Darren P Berman
Percutaneous closure of an atrial septal defect in the cardiac catheterization laboratory requires noninvasive imaging to assist in characterization of the atrial septal defect and deployment of the device. Transesophageal echocardiography is the modality most often used in these circumstances. Transesophageal echocardiographic guidelines exist for the assessment and guidance for atrial septal defect closures, but these guidelines do not mention transgastric views. In this case, we demonstrate that transgastric views are helpful in describing the atrial septal defect, especially as it concerns the inferior vena cava rim...
September 12, 2017: Echocardiography
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