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Congenital valve disease

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https://www.readbyqxmd.com/read/27904846/complete-congenital-heart-block-in-a-neonate-with-a-complex-congenital-heart-defect-in-africa
#1
Clovis Nkoke, Edvine Yonta Wawo, Liliane Kuate Mfeukeu, Larissa Makamte, Sandrine Dikosso Edie, Flore Esiene Balana
Congenital heart block (CHB) is rare disorder that has a higher mortality when associated with structural congenital heart defects. Very few cases have been reported in Sub-Saharan Africa (SSA). We present a case of complete CHB associated with a complex congenital heart defect in a neonate in Cameroon. A 1-month-old neonate in Cameroon was referred for the evaluation of bradycardia. The obstetrical ultrasound done during pregnancy revealed fetal bradycardia without further evaluation. Clinical examination showed well a developed neonate with bradycardia at 62 beats/minute, and mild cyanosis with oxygen saturation at 93% at room air...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27899861/echocardiographic-follow-up-of-patent-foramen-ovale-and-the-factors-affecting-spontaneous-closure
#2
Ali Yildirim, Alperen Aydin, Tevfik Demir, Fatma Aydin, Birsen Ucar, Zubeyir Kilic
BACKGROUND: The aim of the present study was to evaluate the echocardiographic follow-up of patent foramen ovale, which is considered a potential etiological factor in various diseases, and to determine the factors affecting spontaneous closure. METHODS: Between January 2000 and June 2012, records of 918 patients with patent foramen ovale were retrospectively reviewed. Patency of less than 3 mm around the fossa ovalis is called patent foramen ovale. Patients with cyanotic congenital heart diseases, severe heart valve disorders and severe hemodynamic left to right shunts were excluded from the study...
November 2016: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/27899284/defective-lymphatic-valve-development-and-chylothorax-in-mice-with-a-lymphatic-specific-deletion-of-connexin43
#3
Stephanie J Munger, Michael J Davis, Alexander M Simon
Lymphatic valves (LVs) are cusped luminal structures that permit the movement of lymph in only one direction and are therefore critical for proper lymphatic vessel function. Congenital valve aplasia or agenesis can, in some cases, be a direct cause of lymphatic disease. Knowledge about the molecular mechanisms operating during the development and maintenance of LVs may thus aid in the establishment of novel therapeutic approaches to treat lymphatic disorders. In this study, we examined the role of Connexin43 (Cx43), a gap junction protein expressed in lymphatic endothelial cells (LECs), during valve development...
November 27, 2016: Developmental Biology
https://www.readbyqxmd.com/read/27895692/impact-of-postoperative-duration-of-aspirin-use-on-longevity-of-bioprosthetic-pulmonary-valve-in-patients-who-underwent-congenital-heart-disease-repair
#4
Tae-Woong Hwang, Sung-Ook Kim, Sang-Yun Lee, Seong-Ho Kim, Eun-Young Choi, So-Ick Jang, Su-Jin Park, Hye-Won Kwon, Hyo-Bin Lim, Chang-Ha Lee, Eun-Seok Choi
PURPOSE: Generally, aspirin is used as a protective agent against thrombogenic phenomenon after pulmonary valve replacement (PVR) using a bioprosthetic valve. However, the appropriate duration of aspirin use is unclear. We analyzed the impact of postoperative duration of aspirin use on the longevity of bioprosthetic pulmonary valves in patients who underwent repair for congenital heart diseases. METHODS: We retrospectively reviewed the clinical data of 137 patients who underwent PVR using a bioprosthetic valve between January 2000 and December 2003...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/27894865/the-genetic-component-of-bicuspid-aortic-valve-and-aortic-dilation-an-exome-wide-association-study
#5
Marina Gago-Díaz, María Brion, Pastora Gallego, Francisco Calvo, Juan Robledo-Carmona, Daniel Saura, Violeta Sánchez, Javier Bermejo, Teresa Sevilla, Christopher Newton-Cheh, Ángel Carracedo, J Daniel Muehlschlegel, David García-Dorado, Simon C Body, Artur Evangelista
BACKGROUND: Bicuspid aortic valve is the most common cardiovascular congenital malformation affecting 2% of the general population. The incidence of life-threatening complications, the high heritability, and familial clustering rates support the interest in identifying risk or protective genetic factors. The main objective of the present study was to identify population-based genetic variation associated with bicuspid aortic valve and concomitant ascending aortic dilation. MATERIALS AND METHODS: A cross-sectional exome-wide association study was conducted in 565 Spanish cases and 484 controls...
November 25, 2016: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/27893194/incidence-morphology-and-progression-of-bicuspid-aortic-valve-in-pediatric-and-young-adult-subjects-with-coexisting-congenital-heart-defects
#6
Talha Niaz, Joseph T Poterucha, Jonathan N Johnson, Cecilia Craviari, Thomas Nienaber, Jared Palfreeman, Frank Cetta, Donald J Hagler
BACKGROUND: Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV. METHODS: The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015...
November 28, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27879647/neuroprotective-strategies-during-cardiac-surgery-with-cardiopulmonary-bypass
#7
REVIEW
Aida Salameh, Stefan Dhein, Ingo Dähnert, Norbert Klein
Aortocoronary bypass or valve surgery usually require cardiac arrest using cardioplegic solutions. Although, in principle, in a number of cases beating heart surgery (so-called off-pump technique) is possible, aortic or valve surgery or correction of congenital heart diseases mostly require cardiopulmonary arrest. During this condition, the heart-lung machine also named cardiopulmonary bypass (CPB) has to take over the circulation. It is noteworthy that the invention of a machine bypassing the heart and lungs enabled complex cardiac operations, but possible negative effects of the CPB on other organs, especially the brain, cannot be neglected...
November 21, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27866077/the-concept-of-double-inlet-double-outlet-right-ventricle-a-distinct-congenital-heart-disease
#8
Veronica Spadotto, Carla Frescura, Siew Yen Ho, Gaetano Thiene
The aim of this study was to estimate the incidence and to analyze the anatomy of double inlet-double outlet right ventricle complex and its associated cardiac anomalies in our autopsy series. Among the 1640 hearts with congenital heart disease of our Anatomical Collection, we reviewed the specimens with double inlet-double outlet right ventricle, according to the sequential-segmental analysis, identifying associated cardiac anomalies and examining lung histology to assess the presence of pulmonary vascular disease...
September 25, 2016: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/27861628/characteristics-of-infective-endocarditis-in-a-tertiary-hospital-in-east-china
#9
Huimin Xu, Siyu Cai, Haibin Dai
The epidemiology, clinical presentation, and treatment of infective endocarditis (IE) has significantly changed over the past few years in developed countries. However, relevant data from developing countries are different and remain scarce. The objective of this study was to evaluate the clinical presentations, treatment and outcomes of IE patients in a tertiary hospital in East China over an 8-year period. This was a retrospective observational study of consecutive cases of definite or possible IE as per the modified Duke criteria between January 2008 and December 2015...
2016: PloS One
https://www.readbyqxmd.com/read/27845433/analysis-of-serum-metabolites-to-diagnose-bicuspid-aortic-valve
#10
Wenshuo Wang, Aikebaier Maimaiti, Yun Zhao, Lingfei Zhang, Hongyue Tao, Hui Nian, Limin Xia, Biao Kong, Chunsheng Wang, Mofang Liu, Lai Wei
Bicuspid aortic valve (BAV) is the most common congenital heart disease. The current study aims to construct a diagnostic model based on metabolic profiling as a non-invasive tool for BAV screening. Blood serum samples were prepared from an estimation group and a validation group, each consisting of 30 BAV patients and 20 healthy individuals, and analyzed by liquid chromatography-mass spectrometry (LC-MS). In total, 2213 metabolites were detected and 41 were considered different. A model for predicting BAV in the estimation group was constructed using the concentration levels of monoglyceride (MG) (18:2) and glycerophospho-N-oleoyl ethanolamine (GNOE)...
November 15, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27843769/a-case-of-infective-endocarditis-along-with-a-ruptured-valve-caused-by-streptococcus-agalactiae-in-an-immunocompetent-man
#11
Kiyozumi Suzuki, Yuji Hirai, Fujiko Morita, Yuki Uehara, Hiroko Oshima, Kazunori Mitsuhashi, Atsushi Amano, Toshio Naito
Streptococcus agalactiae (S. agalactiae) is a major cause of invasive disease in neonates and pregnant women, but has also recently been observed among non-pregnant adults, especially elderly persons or persons with underlying chronic disease. S. agalactiae is also a rare cause of infective endocarditis, and most cases require early surgery. We report the case of a 43-year-old previously healthy man who experienced rapid progressive culture-negative infective endocarditis with aortic valve vegetation and severe aortic regurgitation, which was complicated by lumbar spondylodiscitis...
2016: IDCases
https://www.readbyqxmd.com/read/27833985/common-and-unusual-dental-development-abnormalities-in-a-patient-with-bicuspid-aortic-valve
#12
Anca Maria Răducanu, Ion Victor Feraru, Ioana Suciu, Elina Teodorescu, Andreea Cristiana Didilescu, Ileana Ionescu, Ecaterina Ionescu
Bicuspid aortic valve (BAV) is the most common congenital abnormality of the heart. In this condition, instead of three cusps, the aortic valve has two cusps. Children with congenital heart diseases are at increased risk of developing oral diseases, such as: higher number of decayed teeth, developmental anomalies, periodontal disease, malocclusion, dental crowding, as well as susceptibility to develop infective endocarditis from bacteremia caused by chronic poor oral health. However, little information is available regarding oral manifestations and their management in patients with congenital heart defects, despite the importance of these diseases...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/27828833/percutaneous-management-of-failed-bioprosthetic-pulmonary-valves-in-patients-with-congenital-heart-defects
#13
Pierpaolo Tarzia, Erika Conforti, Alessandro Giamberti, Alessandro Varrica, Luca Giugno, Angelo Micheletti, Diana Negura, Luciane Piazza, Antonio Saracino, Mario Carminati, Massimo Chessa
AIMS: We reviewed our center experience in the field of transcatheter pulmonary valve-in-valve implantation (TPViV), that is emerging as a treatment option for patients with pulmonary bioprosthetic valve (BPV) dysfunction. METHODS: Between April 2008 and September 2015, a total of six patients with congenital heart disease (four men) underwent TPViV due to stenosis of preexisting BPV. Four patients received a Melody Medtronic Transcatheter Pulmonary Valve and two an Edward Sapien Valve...
November 8, 2016: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/27807680/genetics-of-congenital-heart-disease-past-and-present
#14
REVIEW
Iolanda Muntean, Rodica Togănel, Theodora Benedek
Congenital heart disease is the most common congenital anomaly, representing an important cause of infant morbidity and mortality. Congenital heart disease represents a group of heart anomalies that include septal defects, valve defects, and outflow tract anomalies. The exact genetic, epigenetic, or environmental basis of congenital heart disease remains poorly understood, although the exact mechanism is likely multifactorial. However, the development of new technologies including copy number variants, single-nucleotide polymorphism, next-generation sequencing are accelerating the detection of genetic causes of heart anomalies...
November 2, 2016: Biochemical Genetics
https://www.readbyqxmd.com/read/27791437/urinary-biomarkers-for-renal-tract-malformations
#15
Pedro Magalhães, Joost P Schanstra, Emma Carrick, Harald Mischak, Petra Zürbig
Renal tract malformations (RTMs) are congenital anomalies of the kidneys and urinary tract, which are the major cause of end-stage renal disease in children. Using immunoassay-based approaches (ELISA, western blot), individual urinary proteins including transforming growth factor β, tumor necrosis factor and monocyte attractant proteins 1 were found to be associated to RTMs. However, only mass spectrometry (MS) based methods leading to the identification of panels of protein-based markers composed of fragments of the extracellular matrix allowed the prediction of progression of RTMs and its complications...
November 15, 2016: Expert Review of Proteomics
https://www.readbyqxmd.com/read/27788187/genetic-variants-in-isolated-ebstein-anomaly-implicated-in-myocardial-development-pathways
#16
Robert J Sicko, Marilyn L Browne, Shannon L Rigler, Charlotte M Druschel, Gang Liu, Ruzong Fan, Paul A Romitti, Michele Caggana, Denise M Kay, Lawrence C Brody, James L Mills
Ebstein anomaly (EA) is a rare heart defect in which the tricuspid valve is malformed and displaced. The tricuspid valve abnormalities can lead to backflow of blood from the right ventricle to the right atrium, preventing proper circulation of blood to the lungs. Although the etiology of EA is largely unresolved, increased prevalence of EA in those with a family history of congenital heart disease suggests EA has a genetic component. Copy number variants (CNVs) are a major source of genetic variation and have been implicated in a range of congenital heart defect phenotypes...
2016: PloS One
https://www.readbyqxmd.com/read/27773402/spectrum-of-congenital-mitral-valve-abnormalities-associated-with-solitary-undifferentiated-papillary-muscle-in-adults
#17
Jagdish C Mohan, Madhu Shukla, Vishwas Mohan, Arvind Sethi
BACKGROUND: Congenital anomaly wherein the mitral valve leaflets are directly attached to the papillary muscle(s) (PM) with or without short under-developed chords is rarely reported in adults. Patients with two PMs with an intervening fibrous bridge have also been included under this head in previous studies. METHODS: Echocardiography enables accurate evaluation of the morphology and function of valve leaflets, chordae tendineae, and PM. This report describes a series of six patients aged 56-84 years who had abnormal mitral valve with a large solitary and anomalously inserted PM seen over a period of 3 years...
September 2016: Indian Heart Journal
https://www.readbyqxmd.com/read/27766996/non-fluoroscopic-cardiac-ablation-of-neonates-with-chd
#18
Amee M Bigelow, Brandon S Arnold, Gregory C Padrutt, John M Clark
In current practice, children with anatomically normal hearts routinely undergo fluoroscopy-free ablations. Infants and children with congenital heart disease (CHD) represent the most difficult population to perform catheter ablation without fluoroscopy. We report two neonatal patients with CHD in whom cardiac ablations were performed without fluoroscopy. The first infant had pulmonary atresia with intact ventricular septum with refractory supraventricular tachycardia, and the second infant presented with Ebstein's anomaly of the tricuspid valve along with persistent supraventricular tachycardia...
October 21, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27751299/hypoplastic-right-heart-syndrome-absent-pulmonary-valve-and-non-compacted-left-ventricle-in-an-adult
#19
Jagdish C Mohan, Vishwas Mohan, Madhu Shukla, Arvind Sethi
Hypoplastic right heart syndrome is a rare cyanotic congenital heart disease with under-development of the right ventricle, tricuspid, and pulmonary valves leading to right-to-left shunting of the blood through inter-atrial septal defect. Perinatal mortality is high with very few patients surviving to adulthood without corrective surgery. This report describes a 26-year-old young woman, who had recurrent abortions and stillbirths and detected to have marked cyanosis with hypoplastic right heart, sub-arterial ventricular septal defect, absent pulmonary valve, non-compaction of the left ventricle, and bicuspid aortic valve with aortic regurgitation...
September 2016: Indian Heart Journal
https://www.readbyqxmd.com/read/27742239/the-significance-of-the-interleaflet-triangles-in-determining-the-morphology-of-congenitally-abnormal-aortic-valves-implications-for-noninvasive-imaging-and-surgical-management
#20
REVIEW
Justin T Tretter, Diane E Spicer, Shumpei Mori, Sathish Chikkabyrappa, Andrew N Redington, Robert H Anderson
A comprehensive understanding of the normal and abnormal aortic root is paramount if we are to improve not only our assessment of the aortic root and its components but also the surgical approach to reconstructing this complex structure when congenitally malformed. Most anatomic and imaging-based classifications of the normal root recognize and describe the basic components, which include the shape and size of the three aortic sinuses and their three valvar leaflets, as well as the sinutubular junction and proximal ascending aorta...
December 2016: Journal of the American Society of Echocardiography
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