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Congenital heart defect

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https://www.readbyqxmd.com/read/28086762/prevalence-and-risk-factors-of-congenital-heart-defects-among-live-births-a-population-based-cross-sectional-survey-in-shaanxi-province-northwestern-china
#1
Leilei Pei, Yijun Kang, Yaling Zhao, Hong Yan
BACKGROUND: Nearly half of the population of Northwest China live in Shaanxi province, but population-based data on the epidemiologic characteristics of congenital heart defects (CHD) in this population is limited. The study aimed to investigate the prevalence and epidemiologic characteristics of the CHD among infants born between 2010 and 2013 in Shaanxi province. METHODS: Infants born between 2010 and 2013 in Shaanxi province were surveyed using a stratified multi-stage sampling method...
January 13, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28076624/quadricuspid-aortic-valve-a-comprehensive-review
#2
Shi-Min Yuan
Quadricuspid aortic valve (QAV) is a rare congenital heart disease. The functional status of QAV is predominantly a pure aortic regurgitation. Clinical manifestations of patients with a QAV depend on the functional status of the QAV and the associated disorders. Significant valvular regurgitation and (or) stenosis is often present with subsequent operation performed at the fifth to sixth decade of life. The functional status of QAV is predominantly regurgitant; whereas pure stenotic QAV can be as few as in only 0...
November 2016: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/28075028/a-recognizable-type-of-syndromic-short-stature-with-arthrogryposis-caused-by-bi-allelic-sema3a-loss-of-function-variants
#3
Matthias Baumann, Elisabeth Steichen-Gersdorf, Birgit Krabichler, Thomas Müller, Andreas R Janecke
The semaphorins constitute a large family of secreted and membrane-associated proteins that regulate many developmental processes, including neural circuit assembly, bone formation and angiogenesis. Recently, bi-allelic loss-of-function variants in SEMA3A (semaphorin 3A) were identified in a single patient with a particular pattern of multiple congenital anomalies (MCA). Using homozygosity mapping combined with exome sequencing, we identified a homozygous SEMA3A variant causing a premature stop codon in an 8-year-old boy with the same pattern of MCA...
January 11, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28074821/acyanotic-congenital-heart-disease-and-transesophageal-echocardiography
#4
REVIEW
Rupa Sreedhar
The spectrum of congenital heart disease (CHD) seen in the adult varies widely. Malformations range from mild anomalies requiring no intervention to extremely complex pathologies characterized by the presence of multiple coexistent defects. Echocardiography represents the primary noninvasive imaging modality in the assessment of these lesions. The transesophageal approach expands the applications of echocardiography by allowing the acquisition of anatomic and functional information that may not be obtainable by transthoracic imaging...
January 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28072626/acquired-coronary-artery-disease-in-adult-patients-with-congenital-heart-disease-a-true-or-a-false-problem
#5
Alessandro Giamberti, Mauro Lo Rito, Erika Conforti, Alessandro Varrica, Mario Carminati, Alessandro Frigiola, Lorenzo Menicanti, Massimo Chessa
BACKGROUND: The population of adults with congenital heart disease (ACHD) is increasing and aging, and a large percentage of this population is now over 65 years of age. For this reason, it is probable that acquired coronary artery disease (CAD) will become an important issue that needs to be addressed also in these patients. We retrospectively analyzed all ACHD patients who underwent surgery in our Institution with the aim to investigate the incidence of associated CAD and the results of surgical treatment...
January 7, 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28070867/analytical-modeling-for-computing-lead-stress-in-a-novel-epicardial-micropacemaker
#6
Li Zhou, Yaniv Bar-Cohen, Raymond A Peck, Giorgio V Chirikian, Brett Harwin, Ramen H Chmait, Jay D Pruetz, Michael J Silka, Gerald E Loeb
Implantation and maintenance of a permanent cardiac pacing system in children remains challenging due to small patient size, congenital heart defects and somatic growth. We are developing a novel epicardial micropacemaker for children that can be implanted on the epicardium within the pericardial space via a minimally-invasive technique. The key design configurations include a novel open-coiled lead in which living tissue replaces the usual polymeric support for the coiled conductor. To better understand and be able to predict the behavior of the implanted lead, we performed a radiographic image-based modeling study on a chronic animal test...
January 9, 2017: Cardiovascular Engineering and Technology
https://www.readbyqxmd.com/read/28065906/incidence-risk-factors-and-predictors-of-infective-endocarditis-in-adult-congenital-heart-disease-focus-on-the-use-of-prosthetic-material
#7
Joey M Kuijpers, Dave R Koolbergen, Maarten Groenink, Kathinka C H Peels, Constant L A Reichert, Marco C Post, Hans A Bosker, Elly M C J Wajon, Aeilko H Zwinderman, Barbara J M Mulder, Berto J Bouma
AIMS: Adult congenital heart disease (ACHD) predisposes to infective endocarditis (IE). Surgical advancements have changed the ACHD population, whereas associated prosthetic material may constitute additional IE targets. We aimed to prospectively determine contemporary incidence, risk factors, and predictors of IE in a nationwide ACHD cohort, focusing on the presence of prosthetics. METHODS AND RESULTS: We identified 14 224 patients prospectively followed in the CONCOR ACHD registry (50...
January 8, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28063755/-congenital-heart-disease-recent-technical-advances-in-three-dimensional-echocardiography
#8
Clement Karsenty, Khaled Hadeed, Philippe Acar
The recent technical advances allow the use in practice of three-dimensional echocardiography in children especially through the new high frequency matrix probe. It is difficult or even impossible to hold breathing during children' acquisition so to avoid motion full volume artifact, one beat and live 3D modes are suitable. 3D echocardiography is more accurate than 2D to assess the size, location, and relationship with surrounding structures of atrial and ventricular septal defects and thus helps in the therapeutic decision...
January 4, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28062416/blood-flow-patterns-underlie-developmental-heart-defects
#9
Madeline Midgett, Kent L Thornburg, Sandra Rugonyi
Although cardiac malformations at birth are typically associated with genetic anomalies, blood flow dynamics also play a crucial role in heart formation. However, the relationship between blood flow patterns in the early embryo and later cardiovascular malformation has not been determined. We used the chicken embryo model to quantify the extent to which anomalous blood flow patterns predict cardiac defects that resemble those in humans, and found that restricting either the inflow to the heart or the outflow led to reproducible abnormalities with a dose-response type relationship between blood flow stimuli and the expression of cardiac phenotypes...
January 6, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28061423/correlation-between-fetal-autopsy-and-prenatal-diagnosis-by-ultrasound-a-systematic-review
#10
REVIEW
A Cristina Rossi, Federico Prefumo
: The objective of this study was to review literature about the correlation between fetal autopsy and ultrasound findings of fetal malformations. Search in PubMed, Medline, EMBASE, Clinicl trials.org, reference list was performed. Inclusion criteria for studies selection were: fetal autopsy performed after termination of pregnancy (TOP) or stillbirth, TOP for fetal anomalies, prenatal diagnosis of malformations, data reported as proportional rates. EXCLUSION CRITERIA: case reports, non English language, data reported in graphs or percentage...
December 29, 2016: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/28060152/an-elevated-low-cardiac-output-syndrome-score-is-associated-with-morbidity-in-infants-after-congenital-heart-surgery
#11
Kalia P Ulate, Ofer Yanay, Howard Jeffries, Harris Baden, Jane L Di Gennaro, Jerry Zimmerman
OBJECTIVES: To evaluate an empirically derived Low Cardiac Output Syndrome Score as a clinical assessment tool for the presence and severity of Low Cardiac Output Syndrome and to examine its association with clinical outcomes in infants who underwent surgical repair or palliation of congenital heart defects. DESIGN: Prospective observational cohort study. SETTING: Cardiac ICU at Seattle Children's Hospital. PATIENTS: Infants undergoing surgical repair or palliation of congenital heart defects...
January 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28058479/electrocardiographic-rr-and-qt-interval-variability-in-patients-with-atrial-septal-defect-and-healthy-children
#12
Yoshihiko Eryu, Tadayoshi Hata, Arisa Nagatani, Yuri Funamoto, Hidetoshi Uchida, Masayuki Fujino, Hiroko Boda, Masafumi Miyata, Tetsushi Yoshikawa
Atrial septal defect is a common congenital heart disease. In patients with atrial septal defect, left-to-right shunting increases the right atrial and right ventricular preload. This pathological change affects sinus node automaticity and myocardial depolarization and repolarization, and has the potential to evoke arrhythmogenic substrates. We examined the effect of atrial septal defect on sinus node automaticity and myocardial repolarization by investigating the variability in the repolarization interval, namely the QT variability index (QTVI) and variability ratio (VR)...
January 5, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28057753/a-novel-kleefstra-syndrome-associated-variant-that-affects-the-conserved-tplx-motif-within-the-ankyrin-repeat-of-ehmt1-leads-to-abnormal-protein-folding
#13
Patrick R Blackburn, Alexander Tischer, Michael T Zimmermann, Jennifer L Kemppainen, Sujatha Sastry, Amy E Knight Johnson, Margot A Cousin, Nicole J Boczek, Gavin Oliver, Vinod K Misra, Ralitza H Gavrilova, Gwen A Lomberk, Matthew Auton, Raul A Urrutia, Eric W Klee
Kleefstra syndrome (KS) (MIM# 610253), also known as 9q34 deletion syndrome, is an autosomal dominant disorder caused by haploinsufficiency of euchromatic histone methyltransferase-1 (EHMT1). The clinical phenotype of KS includes moderate to severe intellectual disability with absent speech, hypotonia, brachycephaly, congenital heart defects, and dysmorphic facial features with hypertelorism, synophrys, macroglossia, protruding tongue, and prognathism. Only a few cases of de novo missense mutations in EHMT1 giving rise to KS have been described...
January 5, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28057249/transposition-of-the-great-arteries-in-the-developing-world-surgery-and-outcomes
#14
David N Schidlow, Kathy J Jenkins, Kimberlee Gauvreau, Ulisses A Croti, Do Thi Cam Giang, Rama K Konda, William M Novick, Nestor F Sandoval, Aldo Castañeda
BACKGROUND: Little has been published regarding surgery for transposition of the great arteries (TGA) in the developing world. OBJECTIVES: This study sought to identify patient characteristics, surgical interventions, institutional characteristics, risk factors for mortality, and outcomes among patients undergoing surgery for TGA in this setting. METHODS: Developing world congenital heart surgical programs submitted de-identified data to a novel international collaborative database as part of a quality improvement project...
January 3, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28055942/hot-days-in-early-pregnancy-a-potential-risk-factor-for-congenital-heart-defects
#15
Lindsey Konkel
No abstract text is available yet for this article.
January 1, 2017: Environmental Health Perspectives
https://www.readbyqxmd.com/read/28054902/novel-goal-directed-hemodynamic-optimization-therapy-based-on-major-vasopressor-during-corrective-cardiac-surgery-in-patients-with-severe-pulmonary-arterial-hypertension-a-pilot-study
#16
Shuwen Li, Qing Ma, Yanwei Yang, Jiakai Lu, Zhiquan Zhang, Mu Jin, Weiping Cheng
INTRODUCTION: Pulmonary arterial hypertension (PAH) is a common and fatal complication of congenital heart disease (CHD). PAH-CHD increases the risk for postoperative complications. Recent evidence suggests that perioperative goal-directed hemodynamic optimization therapy (GDHOT) significantly improves outcomes in surgery patients. Standard GDHOT is based on major solution volume, vasodilators and inotropic therapy, while novel GDHOT is based on major vasopressor and inotropic therapy...
22, 2016: Heart Surgery Forum
https://www.readbyqxmd.com/read/28053308/evaluation-of-left-pulmonary-artery-sling-associated-cardiovascular-anomalies-and-surgical-outcomes-using-cardiovascular-computed-tomography-angiography
#17
Jiajun Xie, Yu-Hsiang Juan, Qiushi Wang, Jimei Chen, Jian Zhuang, Zhaofeng Xie, Changhong Liang, Yulei Zhu, Zhuliang Yu, Jinglei Li, Sachin S Saboo, Hui Liu
We evaluated the prevalence, image appearance, associated cardiovascular anomalies, and surgical outcomes of left pulmonary artery sling (LPAS) using cardiovascular computed tomography angiography (CCTA). A retrospective search of patients from our database between October 2007 and December 2014 identified 52,200 patients with congenital heart diseases (CHD) referred for CCTA, echocardiography, or magnetic resonance imaging. Clinical information, CCTA findings, associated cardiovascular anomalies, and surgical outcomes were analyzed...
January 5, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28052532/persistent-left-superior-vena-cava-accompanying-congenital-heart-disease-in-children-experience-of-a-tertiary-care-center
#18
Mehmet Emre Ari, Vehbi Doğan, Senem Özgür, Özben Ceylan, İlker Ertuğrul, Şeyma Kayalı, Tamer Yoldaş, Utku Arman Örün, Özkan Kaya, Selmin Karademir
BACKGROUND: This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD). METHODS: Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3.3%) of whom were diagnosed with persistent left SVC, were evaluated retrospectively. The demographic characteristics of patients, clinical and radiographic findings, echocardiography, cardiac catheterization, and angiography results obtained from the patients' records were reviewed...
January 3, 2017: Echocardiography
https://www.readbyqxmd.com/read/28049487/endothelial-microparticles-are-increased-in-congenital-heart-diseases-and-contribute-to-endothelial-dysfunction
#19
Ze-Bang Lin, Hong-Bo Ci, Yan Li, Tian-Pu Cheng, Dong-Hong Liu, Yan-Sheng Wang, Jun Xu, Hao-Xiang Yuan, Hua-Ming Li, Jing Chen, Li Zhou, Zhi-Ping Wang, Xi Zhang, Zhi-Jun Ou, Jing-Song Ou
BACKGROUND: We previously demonstrated that endothelial microparticles (EMPs) are increased in mitral valve diseases and impair valvular endothelial cell function. Perioperative systemic inflammation is an important risk factor and complication of cardiac surgery. In this study, we investigate whether EMPs increase in congenital heart diseases to promote inflammation and endothelial dysfunction. METHODS: The level of plasma EMPs in 20 patients with atrial septal defect (ASD), 23 patients with ventricular septal defect (VSD), and 30 healthy subjects were analyzed by flow cytometry...
January 4, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28045420/a-case-of-tracheal-bronchus-associated-with-right-aortic-arch-and-partial-anomalous-pulmonary-venous-connection
#20
Vehbi Doğan, İlker Ertuğrul, Yasemin Taşcı Yıldız, Utku Arman Örün, Selmin Karademir
Tracheal bronchus includes a variety of bronchial anomalies arising in the trachea or main bronchus and directed toward the upper-lobe territory. Reported incidence varies from 1-3% in the pediatric population. It is generally associated with other congenital malformations, including costovertebral anomalies, congenital airway and lung anomalies, vascular anomalies, and congenital heart defects. Presently described was the case of a 14-year-old female with tracheal right-upper-lobe bronchus, right aortic arch with mirror image, and abnormal left upper pulmonary venous return to innominate vein...
December 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
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