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congenital cardiac disease

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https://www.readbyqxmd.com/read/28436940/disruption-of-spatiotemporal-hypoxic-signaling-causes-congenital-heart-disease-in-mice
#1
Xuejun Yuan, Hui Qi, Xiang Li, Fan Wu, Jian Fang, Eva Bober, Gergana Dobreva, Yonggang Zhou, Thomas Braun
Congenital heart disease (CHD) represents the most prevalent inborn anomaly. Only a minority of CHD cases are attributed to genetic causes, suggesting a major role of environmental factors. Nonphysiological hypoxia during early pregnancy induces CHD, but the underlying reasons are unknown. Here, we have demonstrated that cells in the mouse heart tube are hypoxic, while cardiac progenitor cells (CPCs) expressing islet 1 (ISL1) in the secondary heart field (SHF) are normoxic. In ISL1+ CPCs, induction of hypoxic responses caused CHD by repressing Isl1 and activating NK2 homeobox 5 (Nkx2...
April 24, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28436859/in-vivo-intracardiac-vector-velocity-imaging-using-phased-array-transducers-for-pediatric-cardiology
#2
Solveig Fadnes, Morten Wigen, Siri Ann Nyrnes, Lasse Lovstakken
Two-dimensional blood speckle tracking has shown promise for measuring complex flow patterns in neonatal hearts using linear arrays and high-frame-rate plane wave imaging. For general pediatric applications, however, the need for phased array probes emerges due to the limited intercostal acoustic window available. In this work, a clinically approved real-time duplex imaging setup with phased array probes was used to investigate the potential of blood speckle tracking for 2-D vector velocity imaging of children with congenital heart disease...
April 24, 2017: IEEE Transactions on Ultrasonics, Ferroelectrics, and Frequency Control
https://www.readbyqxmd.com/read/28435986/asian-consortium-on-radiation-dose-of-pediatric-cardiac-ct-asci-redcard
#3
Peter K T Hui, Hyun Woo Goo, Jing Du, Janice J K Ip, Suzu Kanzaki, Young Jin Kim, Supika Kritsaneepaiboon, Oktavia Lilyasari, Suvipaporn Siripornpitak
BACKGROUND: With incremental utilization of pediatric cardiac CT in congenital heart disease, it is imperative to define its current radiation dose levels in clinical practice in order to help imagers optimize CT protocols, particularly in Asia and other developing countries where CT physicists are not readily available. OBJECTIVE: To evaluate current radiation dose levels and influencing factors in cardiac CT in children with congenital heart disease in Asia by conducting a retrospective multi-center, multi-vendor study...
April 24, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28434921/identification-of-a-novel-and-functional-mutation-in-the-tbx5-gene-in-a-patient-by-screening-from-354-patients-with-isolated-ventricular-septal-defect
#4
Huan-Xin Chen, Xi Zhang, Hai-Tao Hou, Jun Wang, Qin Yang, Xiu-Li Wang, Guo-Wei He
Ventricular septal defect (VSD) is the most frequently occurring congenital heart disease (CHD) in newborns. A number of genetic studies have linked TBX5 mutations to cardiac abnormalities. We aimed to identify potential pathogenic mutations in TBX5 and to provide insights into the etiology of sporadic and isolated VSD. Case-control mutational and functional analyses were performed in 354 sporadic patients with isolated VSD and 341 controls. All the coding exons and intron-exon boundaries of TBX5 were first sequenced in a group of VSD patients and controls...
April 18, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28434627/-sports-in-children-with-congenital-heart-diseases
#5
Gilles Bosser, Anne Moulin-Zinsch, Reem Fischer-Atalla
The practice of physical activity is one of the essential elements for health in general but also for the well-being and the quality of life. It is highly desirable to encourage physical activities in children with congenital heart diseases, taking into account all the benefits associated with this practice (quality of life, life expectancy) and this especially since these children often have limited capacity (due to their heart disease but also often by relative deconditioning). While there is a transient increase in risk of cardiac complications during intense activity, it would nevertheless be inappropriate to contra-indicate physical activities considering the well-known benefits in the medium and long term...
April 21, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28430913/prediction-of-adverse-events-after-catheter-based-procedures-in-adolescents-and-adults-with-congenital-heart-disease-in-the-impact-registry
#6
Ada C Stefanescu Schmidt, Aimee Armstrong, Kevin F Kennedy, David Nykanen, Jamil Aboulhosn, Ami B Bhatt
Aims: We sought to identify factors associated with major adverse events (MAE) after cardiac catheterization in adolescents and adults with congenital heart disease (CHD), and create the first model to individualize risk discussions in this growing population. Methods and results: Improving Pediatric and Adult Congenital Treatment (IMPACT), a National Cardiovascular Data Registry, contains congenital catheterization data from over 87 hospitals in the United States...
April 18, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28430115/a-systematic-review-of-3-d-printing-in-cardiovascular-and-cerebrovascular-diseases
#7
Zhonghua Sun, Shen Yuan Lee
OBJECTIVE: The application of 3-D printing has been increasingly used in medicine, with research showing many applications in cardiovascular disease. This systematic review analyzes those studies published about the applications of 3-D printed, patient-specific models in cardiovascular and cerebrovascular diseases. METHODS: A search of PubMed/Medline and Scopus databases was performed to identify studies investigating the 3-D printing in cardiovascular and cerebrovascular diseases...
April 10, 2017: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/28427801/-multimodality-imaging-in-the-cardiac-catheterization-laboratory
#8
Karine Warin-Fresse, Sébastien Hascoet, Patrice Guérin
Cardiac catheterization has greatly contributed to the progress made in the management of congenital heart diseases (CHD). Initially used in diagnosis, it allowed the understanding of heart diseases, their anatomy and hemodynamics. Gradually, the development of interventional cardiology has played a major role in the management of these malformations (Patent ductus arteriosus [PDA] and atrial septal defect [ASD] closure, pulmonary dilatation, percutaneous pulmonary valve implantation…). The development of such technology was made possible through the concomitant development of imaging techniques: fluoroscopy, ultrasound, MRI and CT...
April 17, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28423752/a-decision-support-system-for-cardiac-disease-diagnosis-based-on-machine-learning-methods
#9
Arash Gharehbaghi, Maria Lindén, Ankica Babic
This paper proposes a decision support system for screening pediatric cardiac disease in primary healthcare centres relying on the heart sound time series analysis. The proposed system employs our processing method which is based on the hidden Markov model for extracting appropriate information from the time series. The binary output resulting from the method is discriminative for the two classes of time series existing in our databank, corresponding to the children with heart disease and the healthy ones. A total 90 children referrals to a university hospital, constituting of 55 healthy and 35 children with congenital heart disease, were enrolled into the study after obtaining the informed consent...
2017: Studies in Health Technology and Informatics
https://www.readbyqxmd.com/read/28419616/the-provision-of-pediatric-cardiac-anesthesia-services-in-germany-current-status-of-structural-and-personnel-organization
#10
Torsten Baehner, Oliver Dewald, Ingo Heinze, Matthias Mueller, Ehrenfried Schindler, Uwe Schirmer, Georg Baumgarten, Andreas Hoeft, Richard K Ellerkmann
BACKGROUND: Anesthesia for pediatric cardiac surgery requires a high level of expert knowledge. There are currently no recommendations and standards for anesthetic management for congenital cardiac surgery in Germany. AIM: The aim of the present study was to assess the current status of structural and personnel anesthetic standards at pediatric cardiac surgery centers in Germany. METHOD: All cardiac surgical centers in Germany were reviewed for an active program for congenital heart surgery...
April 17, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28419229/non-invasive-assessment-of-cerebral-oxygen-metabolism-following-surgery-of-congenital-heart-disease
#11
Felix Neunhoeffer, Katharina Sandner, Milena Wiest, Christoph Haller, Hanna Renk, Matthias Kumpf, Christian Schlensak, Michael Hofbeck
OBJECTIVES: Cerebral protection is a major issue in the treatment of infants with complex congenital heart disease. We tested a new device combining tissue spectrometry and laser Doppler flowmetry for non-invasive determination of cerebral oxygen metabolism following cardiac surgery in infants. METHODS: We prospectively measured regional cerebral oxygen saturation cSO 2 and microperfusion (rcFlow) in 43 infants 12-24 h following corrective ( n  = 30) or palliative surgery ( n  = 13) of congenital heart defects...
April 16, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28416028/attitudes-and-perceptions-of-pregnant-women-with-chd-results-of-a-single-site-survey
#12
Aarthi Sabanayagam, David Briston, Ali N Zaidi
Introduction CHD occurs in about 1% of the United States population, and is now the most common cardiac condition affecting women during pregnancy. METHODS: An anonymous, single-site, cross-sectional, 50-question survey was performed at a national Adult Congenital Heart Disease conference to assess the level of knowledge, attitudes, and perceptions regarding cardiac care during pregnancy in women with CHD. RESULTS: A total of 77 women completed the survey. Among them, 50% (n=39) had moderate and 38% (n=29) had severely complex disease; 30% (n=23) of women were told that pregnancy was contraindicated given their underlying cardiac condition...
April 18, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28415932/the-effects-of-corrective-surgery-on-endothelial-biomarkers-and-anthropometric-data-in-children-with-congenital-heart-disease
#13
Hung-Tao Chung, Yu-Sheng Chang, Sui-Ling Liao, Shen-Hao Lai
Objective To investigate the influence of surgical correction on biomarkers of endothelial dysfunction in children with congenital heart disease and to evaluate anthropometric data. Methods Children with pulmonary hypertension (PH) or Tetralogy of Fallot (TOF) who were scheduled for corrective surgery were enrolled in this prospective study. Age-matched healthy children were included as controls. Demographic, haemodynamic and cardiac ultrasonography data were collected. Blood samples were taken pre-surgery, 24-48 hours post-surgery and again 3-6 months later...
April 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28413915/oxidative-stress-status-in-congenital-hypogonadism-an-appraisal
#14
C Haymana, A Aydoğdu, B Soykut, O Erdem, T Ibrahimov, M Dinc, C Meric, Y Basaran, A Sonmez, O Azal
Patients with hypogonadism are at increased risk of cardiac and metabolic diseases. However, the pathogenesis of increased cardiometabolic risk in patients with hypogonadism is not clear. Oxidative stress plays an important role in the pathogenesis of cardiometabolic diseases. This study aimed to investigate possible differences in oxidative stress conditions between patients with hypogonadism and healthy controls. In this study, 38 male patients with congenital hypogonadotrophic hypogonadism (CHH) (mean age: 21...
April 16, 2017: Toxicology Mechanisms and Methods
https://www.readbyqxmd.com/read/28412993/paediatric-onset-coronary-artery-anomalies-in-pregnancy-a-single-centre-experience-and-systematic-literature-review
#15
Michelle Keir, Catriona Bhagra, Debra Vatenmakher, Francisca Arancibia-Galilea, Katrijn Jansen, Norihisa Toh, Candice K Silversides, Jack Colman, Samuel C Siu, Mathew Sermer, Andrew M Crean, Rachel M Wald
OBJECTIVES: Individuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population. METHODS: We performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases...
April 17, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28412020/cardiac-surgery-in-patients-with-congenital-heart-disease-is-associated-with-acute-kidney-injury-and-the-risk-of-chronic-kidney-disease
#16
Nicolas L Madsen, Stuart L Goldstein, Trine Frøslev, Christian F Christiansen, Morten Olsen
Cardiac surgery associated-acute kidney injury (CS-AKI) occurs in 30-50% of patients undergoing surgery for congenital heart disease. Here we determine if CS-AKI is associated with chronic kidney disease (CKD) in patients with congenital heart disease. Using Danish regional population-based registries, our cohort study included patients with congenital heart disease born between 1990-2010 with first cardiac surgery between 2005 and 2010 (under 15 years of age). Utilizing in- and out-patient laboratory serum creatinine data, we identified individuals fulfilling KDIGO stages of AKI within 5 days of cardiac surgery...
April 12, 2017: Kidney International
https://www.readbyqxmd.com/read/28410842/evidence-of-impaired-microvascular-dilatation-in-preterms-with-acute-respiratory-distress-syndrome
#17
Stefania Troiani, Andrea Cardona, Maddalena Milioni, Debora Monacelli, Alberto Verrotti, Marta Gehring, Giuseppe Ambrosio
BACKGROUND: Evaluating microcirculatory function in severely ill neonates is a relevant, unmet clinical need. Inappropriate peripheral microvascular vasodilatation is thought to contribute to cardiovascular alterations in preterm infants with acute respiratory distress syndrome (ARDS). We directly evaluated microcirculatory function in preterms with ARDS. METHODS: Peripheral microvascular function was assessed in 50 newborns, divided in three groups: preterms with ARDS; at-term newborns with mild-moderate congenital cardiac disease (Cardio group); healthy controls...
April 6, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28408715/postoperative-infection-in-developing-world-congenital-heart-surgery-programs-data-from-the-international-quality-improvement-collaborative
#18
Amitabh Chanchal Sen, Debra Forbes Morrow, Rakhi Balachandran, Xinwei Du, Kimberlee Gauvreau, Byalal R Jagannath, Raman Krishna Kumar, Jennifer Koch Kupiec, Monica L Melgar, Nguyen Tran Chau, Gail Potter-Bynoe, Orlando Tamariz-Cruz, Kathy J Jenkins
BACKGROUND: Postoperative infections contribute substantially to morbidity and mortality after congenital heart disease surgery and are often preventable. We sought to identify risk factors for postoperative infection and the impact on outcomes after congenital heart surgery, using data from the International Quality Improvement Collaborative for Congenital Heart Surgery in Developing World Countries. METHODS AND RESULTS: Pediatric cardiac surgical cases performed between 2010 and 2012 at 27 participating sites in 16 developing countries were included...
April 2017: Circulation. Cardiovascular Quality and Outcomes
https://www.readbyqxmd.com/read/28408455/cell-therapy-trials-in-congenital-heart-disease
#19
REVIEW
Hidemasa Oh
Dramatic evolution in medical and catheter interventions and complex surgeries to treat children with congenital heart disease (CHD) has led to a growing number of patients with a multitude of long-term complications associated with morbidity and mortality. Heart failure in patients with hypoplastic left heart syndrome predicated by functional single ventricle lesions is associated with an increase in CHD prevalence and remains a significant challenge. Pathophysiological mechanisms contributing to the progression of CHD, including single ventricle lesions and dilated cardiomyopathy, and adult heart disease may inevitably differ...
April 14, 2017: Circulation Research
https://www.readbyqxmd.com/read/28407839/-effect-of-histone-acetylation-deacetylation-imbalances-on-key-gene-of-planar-cell-polarity-pathway
#20
Hong-Yu Duan, Yi Zhang, Kai-Yu Zhou, Chuan Wang, DA-Jian Qiu, Yi-Min Hua
OBJECTIVE: To investigate the effect of histone acetylation/deacetylation imbalances on embryonic hearts of mice and its effect on key genes of planar cell polarity (PCP) pathway-Vangl2, Scrib and Rac1 in H9C2 cells. METHODS: Forty pregnant C57/B6 mice were randomly assigned into three groups: blank group (n=10), vehicle group (n=10), and valproic acid (VPA)-treated group (n=20). In the VPA-treated group, VPA, a histone deacetylase (HDAC) inhibitor, was administered to each individual dam intraperitoneally at a single dose of 700 mg/kg on embryonic day 10...
April 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
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