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Cardiac defects

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https://www.readbyqxmd.com/read/29138508/fetal-cardiac-cine-magnetic-resonance-imaging-in-utero
#1
Jerome Chaptinel, Jerome Yerly, Yvan Mivelaz, Milan Prsa, Leonor Alamo, Yvan Vial, Gregoire Berchier, Chantal Rohner, François Gudinchet, Matthias Stuber
Fast magnetic resonance imaging (MRI) led to the emergence of 'cine MRI' techniques, which enable the visualization of the beating heart and the assessment of cardiac morphology and dynamics. However, established cine MRI methods are not suitable for fetal heart imaging in utero, where anatomical structures are considerably smaller and recording an electrocardiogram signal for synchronizing MRI data acquisition is difficult. Here we present a framework to overcome these challenges. We use methods for image acquisition and reconstruction that robustly produce images with sufficient spatial and temporal resolution to detect the heart contractions of the fetus, enabling a retrospective gating of the images and thus the generation of images of the beating heart...
November 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29137093/an-incidental-encounter-of-a-rare-high-take-off-right-coronary-artery-a-case-report
#2
Xicheng Deng, Peng Huang, Wenjuan Chen, Xiaohui Yang, Qianjun Liu, Yunbin Xiao, Cheng He
RATIONALE: High take-off of the coronary arteries is a rare cardiac anatomic anomaly, which may occur independently or with other congenital heart defects. In the clinical setting, it is noteworthy as a cause of sudden cardiac death. Further, it is vital to identify such anomalies to avoid intraoperative catastrophes in surgeries for congenital heart defects. PATIENT CONCERNS: A II/6 systolic heart murmur on physical examination was incidentally found in a 9-year-old boy; he was confirmed to have a secundum-type atrial septal defect on echocardiography...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29133341/the-trafficking-protein-ehd2-positively-regulates-cardiac-sarcolemmal-katp-channel-surface-expression-role-in-cardioprotection
#3
Hua Qian Yang, Kundan Jana, Michael J Rindler, William A Coetzee
ATP-sensitive K(+) (KATP) channels uniquely link cellular energy metabolism to membrane excitability and are expressed in diverse cell types that range from the endocrine pancreas to neurons and smooth, skeletal, and cardiac muscle. A decrease in the surface expression of KATP channels has been linked to various disorders, including dysregulated insulin secretion, abnormal blood pressure, and impaired resistance to cardiac injury. In contrast, up-regulation of KATP channel surface expression may be protective, for example, by mediating the beneficial effect of ischemic preconditioning...
November 13, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29129255/regional-variation-in-quality-of-life-in-patients-with-a-fontan-circulation-a-multinational-perspective
#4
Nicholas D Fogleman, Silke Apers, Philip Moons, Stacey Morrison, Samuel G Wittekind, Martha Tomlin, Kathy Gosney, Maayke A Sluman, Bengt Johansson, Junko Enomoto, Mikael Dellborg, Chun-Wei Lu, Raghavan Subramanyan, Koen Luyckx, Werner Budts, Jamie Jackson, Adrienne Kovacs, Alexandra Soufi, Katrine Eriksen, Corina Thomet, Malin Berghammer, Edward Callus, Susan M Fernandes, Maryanne Caruana, Stephen C Cook, Andrew S Mackie, Kamila S White, Paul Khairy, Shelby Kutty, Gruschen Veldtman
BACKGROUND: Impaired quality of life (QOL) is associated with congenital heart disease (CHD) and country of residence; however, few studies have compared QOL in patients with differing complexities of CHD across regional populations. The current study examined regional variation in QOL outcomes in a large multinational sample of patients with a Fontan relative to patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs). METHODS: From the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study (APPROACH-IS), 405 patients (163 Fontan and 242 ASD/VSD) across Asia, Europe, and North America provided consent for access to their medical records and completed a survey evaluating QOL (0 to 100 linear analog scale)...
November 2017: American Heart Journal
https://www.readbyqxmd.com/read/29127187/apobec2-deficiency-causes-mitochondrial-defects-and-mitophagy-in-skeletal-muscle
#5
Yusuke Sato, Hideaki Ohtsubo, Naohiro Nihei, Takane Kaneko, Yoriko Sato, Shin-Ichi Adachi, Shinji Kondo, Mako Nakamura, Wataru Mizunoya, Hiroshi Iida, Ryuichi Tatsumi, Cristina Rada, Fumiaki Yoshizawa
Apobec2 is a member of the activation-induced deaminase/apolipoprotein B mRNA editing enzyme catalytic polypeptide cytidine deaminase family expressed in differentiated skeletal and cardiac muscle. We previously reported that Apobec2 deficiency in mice leads to a shift in muscle fiber type, myopathy, and diminished muscle mass. However, the mechanisms of myopathy caused by Apobec2 deficiency and its physiologic functions are unclear. Here we show that, although Apobec2 localizes to the sarcomeric Z-lines in mouse tissue and cultured myotubes, the sarcomeric structure is not affected in Apobec2-deficient muscle...
November 10, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29126655/incidence-of-infective-endocarditis-and-its-thromboembolic-complications-in-a-pediatric-population-over-30years
#6
K Thom, A Hanslik, J L Russell, S Williams, P Sivaprakasam, U Allen, C Male, L R Brandão
BACKGROUND: Pediatric infective endocarditis (IE) has been associated with high morbidity and mortality, mostly related to thromboembolic complications (TEC). The objective of our study was to describe the experience in children with IE and to review the changes over a thirty-year period, regarding origin of IE, incidence of vegetations, TEC and their respective morbidity and mortality rates. METHODS: A retrospective chart review of children aged 0-18years with IE defined by the Duke Criteria and admitted to The Hospital for Sick Children, was conducted...
November 7, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29125824/intraluminal-valves-development-function-and-disease
#7
REVIEW
Xin Geng, Boksik Cha, Md Riaj Mahamud, R Sathish Srinivasan
The circulatory system consists of the heart, blood vessels and lymphatic vessels, which function in parallel to provide nutrients and remove waste from the body. Vascular function depends on valves, which regulate unidirectional fluid flow against gravitational and pressure gradients. Severe valve disorders can cause mortality and some are associated with severe morbidity. Although cardiac valve defects can be treated by valve replacement surgery, no treatment is currently available for valve disorders of the veins and lymphatics...
November 1, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29125447/multiple-echocardiography-abnormalities-associated-with-endoscopic-third-ventriculostomy-failure
#8
Ashish H Shah, George M Ibrahim, Jun Sasaki, John Ragheb, Sanjiv Bhatia, Toba N Niazi
OBJECTIVE Although endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has gained increasing prominence in the management of hydrocephalus caused by intraventricular hemorrhage of prematurity, the rates of long-term shunt independence remain low. Furthermore, limited evidence is available to identify infants who might benefit from the procedure. The authors tested the hypothesis that elevated venous pressure that results from comorbid cardiac disease might predispose patients to ETV/CPC failure and shunt dependence...
November 10, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29124685/mitochondrial-trifunctional-protein-deficiency-severe-cardiomyopathy-and-cardiac-transplantation
#9
C Bursle, R Weintraub, C Ward, R Justo, J Cardinal, D Coman
We describe mitochondrial trifunctional protein deficiency (MTPD) in two male siblings who presented with severe cardiomyopathy in infancy. The first sibling presented in severe cardiac failure at 6 months of age and succumbed soon after. The second sibling came to attention after newborn screening identified a possible fatty acid oxidation defect. Dietary therapy and carnitine supplementation commenced in the neonatal period. Despite this the second child required cardiac transplantation at 3 years of age after a sudden and rapid decline in cardiac function...
November 10, 2017: JIMD Reports
https://www.readbyqxmd.com/read/29120500/oral-healthcare-in-fraser-syndrome
#10
Y Hassona, H Kharoub, C Scully
Fraser syndrome is a rare autosomal recessive disorder characterized by renal agenesis, cryptophthalmos, syndactyly, and laryngeal stenosis. Oral manifestations are sparsely described in the literature, and patients with this syndrome might require special dental management considerations because of the associated cardiac, renal, and cognitive defects. In this report, we describe the oral/dental findings in a 22-year-old girl with Fraser syndrome, and discuss the dental management.
November 9, 2017: Special Care in Dentistry
https://www.readbyqxmd.com/read/29119710/role-of-sdf-1-cxcr4-in-impaired-post-myocardial-infarction-cardiac-repair-in-diabetes
#11
Maritza E Mayorga, Matthew Kiedrowski, Patricia McCallinhart, Farhad Forudi, Jeremiah Ockunzzi, Kristal Weber, William Chilian, Marc S Penn, Feng Dong
Diabetes is a risk factor for worse outcomes following acute myocardial infarction (AMI). In this study, we tested the hypothesis that SDF-1:CXCR4 expression is compromised in post-AMI in diabetes, and that reversal of this defect can reverse the adverse effects of diabetes. Mesenchymal stem cells (MSC) isolated from green fluorescent protein (GFP) transgenic mice (control MSC) were induced to overexpress stromal cell-derived factor-1 (SDF-1). SDF-1 expression in control MSC and SDF-1-overexpressing MSC (SDF-1:MSC) were quantified using enzyme-linked immunosorbent assay (ELISA)...
November 9, 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/29119274/quantification-of-pulmonary-regurgitation-and-prediction-of-pulmonary-valve-replacement-by-echocardiography-in-patients-with-congenital-heart-defects-in-comparison-to-cardiac-magnetic-resonance-imaging
#12
Claudia Dellas, Laura Kammerer, Verena Gravenhorst, Joachim Lotz, Thomas Paul, Michael Steinmetz
Pulmonary regurgitation (PR) is common in patients with congenital heart defects (CHD) and contributes to morbidity and mortality in the long-term. We investigated in this retrospective analysis whether readily accessible echocardiographic parameters are useful for quantification of PR and for predicting pulmonary valve replacement (PVR) in comparison to the gold-standard phase contrast (PC) flow measurements from cardiovascular magnetic resonance (CMR). Continuous wave (CW) Doppler and colour flow images in echocardiograms from 53 patients with CHD were analysed...
November 8, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/29119262/role-of-cardiac-pet-in-clinical-practice
#13
REVIEW
Brian M Salata, Parmanand Singh
Early identification of atherosclerosis and at-risk lesions plays a critical role in reducing the burden of cardiovascular disease. While invasive coronary angiography serves as the gold standard for diagnosing coronary artery disease, non-invasive imaging techniques provide visualization of both anatomical and functional atherosclerotic processes prior to clinical presentation. The development of cardiac positron emission tomography (PET) has greatly enhanced our capability to diagnose and treat patients with early stages of atherosclerosis...
November 9, 2017: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29118953/the-role-of-preoperative-sildenafil-therapy-in-controlling-of-postoperative-pulmonary-hypertension-in-children-with-ventricular-septal-defects
#14
Hamid Bigdelian, Mohsen Sedighi
Introduction: Most of the ventricular septal defects (VSD) are complicated with pulmonary arterial hypertension (PAH) which is the major cause of pulmonary hypertensive crisis and right ventricular failure. Methods: We reviewed clinical outcomes of 63 infants who underwent cardiac surgery and were divided into three groups. Control group (n=20) did not received sildenafil while group A (n = 22) received drug (0.3 mg/kg) before and after surgery. Group B (n=21) received drug at the initiation of surgery. Demographic data, preoperative and postoperative variables were compared among the patients...
2017: Journal of Cardiovascular and Thoracic Research
https://www.readbyqxmd.com/read/29118365/impaired-development-of-the-cerebral-cortex-in-infants-with-congenital-heart-disease-is-correlated-to-reduced-cerebral-oxygen-delivery
#15
Christopher J Kelly, Antonios Makropoulos, Lucilio Cordero-Grande, Jana Hutter, Anthony Price, Emer Hughes, Maria Murgasova, Rui Pedro A G Teixeira, Johannes K Steinweg, Sagar Kulkarni, Loay Rahman, Hui Zhang, Daniel C Alexander, Kuberan Pushparajah, Daniel Rueckert, Joseph V Hajnal, John Simpson, A David Edwards, Mary A Rutherford, Serena J Counsell
Neurodevelopmental impairment is the most common comorbidity associated with complex congenital heart disease (CHD), while the underlying biological mechanism remains unclear. We hypothesised that impaired cerebral oxygen delivery in infants with CHD is a cause of impaired cortical development, and predicted that cardiac lesions most associated with reduced cerebral oxygen delivery would demonstrate the greatest impairment of cortical development. We compared 30 newborns with complex CHD prior to surgery and 30 age-matched healthy controls using brain MRI...
November 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29117223/sensitivity-analysis-revealing-the-effect-of-modulating-ionic-mechanisms-on-calcium-dynamics-in-simulated-human-heart-failure
#16
Maria T Mora, Jose M Ferrero, Lucia Romero, Beatriz Trenor
Abnormal intracellular Ca2+ handling is the major contributor to the depressed cardiac contractility observed in heart failure. The electrophysiological remodeling associated with this pathology alters both the action potential and the Ca2+ dynamics, leading to a defective excitation-contraction coupling that ends in mechanical dysfunction. The importance of maintaining a correct intracellular Ca2+ concentration requires a better understanding of its regulation by ionic mechanisms. To study the electrical activity and ionic homeostasis of failing myocytes, a modified version of the O'Hara et al...
2017: PloS One
https://www.readbyqxmd.com/read/29116562/prediction-of-long-term-%C3%A2-10%C3%A2-year-cardiovascular-outcomes-in-heart-transplant-recipients-value-of-stress-technetium-99m-tetrofosmin-myocardial-perfusion-imaging
#17
Jesse F Veenis, Hendrik J Boiten, Jan C van den Berge, Kadir Caliskan, Alex P W M Maat, Roelf Valkema, Alina A Constantinescu, Olivier C Manintveld, Felix Zijlstra, Ron T van Domburg, Arend F L Schinkel
BACKGROUND: Myocardial perfusion imaging (MPI) using single-photon emission computed tomography (SPECT) is useful in the evaluation of cardiac allograft vasculopathy (CAV) in heart transplant (HTx) recipients. The current study evaluated the long-term prognostic value of stress SPECT MPI for predicting all-cause mortality and cardiac events in HTx recipients. METHODS: The study population consisted of 166 HTx recipients (mean age 54 ± 10 years, 84% male) who underwent exercise or dobutamine stress (99m)Tc-tetrofosmin SPECT MPI for the assessment of CAV...
November 7, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/29115376/ccdc134-serves-a-crucial-role-in-embryonic-development
#18
Biaoyi Yu, Tianzhuo Zhang, Peng Xia, Xiaoting Gong, Xiaoyan Qiu, Jing Huang
Coiled-coil domain containing 134 (CCDC134), a characterized secreted protein, may serve as an immune cytokine and illustrates its potent antitumor effects by augmenting CD8+ T-cell-mediated immunity. Additionally, CCDC134 may also act as a novel regulator of human alteration/deficiency in activation 2a, and be involved in the p300-CBP-associated factor complex and affect its acetyltransferase activity. To clarify the biological and pathological function of CCDC134, the present study generated a viable and fertile Ccdc134fl/fl mouse strain that allowed temporal and spatial control of gene ablation...
October 19, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29114300/an-adult-with-atrial-septal-defect-presenting-with-a-brain-abscess
#19
Bijesh Ravindran Nair, Edmond Jonathan, Ranjith Krishna Moorthy, Vedantam Rajshekhar, Oommen George
The common heart diseases resulting in a brain abscess are associated with a right to left shunt and include tetralogy of Fallot and transposition of great vessels. Atrial septal defect (ASD) is almost always associated with the left to right shunt and therefore is not a commonly considered risk factor for brain abscess. We report the case of a 29-year-old male, with no symptoms of cardiac disease, who presented with the left posterior frontal pyogenic abscess which led to the detection of a silent ASD. Our case emphasizes the need for a careful evaluation of the source of infection in patients with a brain abscess...
October 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/29112643/assessment-of-fetal-congenital-heart-diseases-by-4-dimensional-ultrasound-using-spatiotemporal-image-correlation-pictorial-review
#20
Edward Araujo Júnior, Gabriele Tonni, Nathalie Jeanne Bravo-Valenzuela, Fabricio Da Silva Costa, Simon Meagher
The aim of this pictorial review is to describe the technical advances achieved through the application of 4-dimensional (4D) ultrasound using spatiotemporal image correlation (STIC) over conventional 2-dimensional ultrasound in the prenatal detection of congenital heart disease (CHD). Spatiotemporal image correlation is a volume imaging technique that simplifies fetal heart studies while providing more diagnostic information than is typically available from traditional 2-dimensional studies. Four-dimensional software allows the study of cardiac anatomy and function during a single cardiac cycle and has greatly contributed to diagnostic enhancement of CHD...
November 6, 2017: Ultrasound Quarterly
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