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Congenital aortic stenosis

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https://www.readbyqxmd.com/read/28441821/-assessment-of-undiagnosed-critical-congenital-heart-disease-before-discharge-from-the-maternity-hospital
#1
Q M Zhao, F Liu, L Wu, M Ye, B Jia, X J Ma, G Y Huang
Objective: Undiagnosed critical congenital heart disease (CCHD) was assessed before discharge from maternity hospital.Basic information was provided for screening CCHD in the early neonatal stage.Chi-squared test was used for comparison of categorical variables(detection rate of different types of CCHD). Method: A retrospective cohort study was conducted in neonates with CCHD who were admitted to Children's Hospital of Fudan University between 1 January 2012 and 31 December 2015. For comparing with the previously reported undiagnosed rate of CCHD at discharge, CCHD was defined as all duct dependent congenital heart disease (DDCHD) and any cyanotic CHD that required early surgery...
April 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28424443/case-report-of-a-rarely-seen-long-segment-middle-aortic-syndrome
#2
Kahraman Yakut, İlkay Erdoğan
Middle aortic syndrome (MAS) follows a course with distal thoracic and abdominal aorta stenosis. It is a rare disease that is usually diagnosed after the first decade of life. Clinical reflection of MAS is often in the form of hypertension and claudication in the lower extremities. Its etiology is unclear, but is known to be associated with congenital or acquired diseases. This pathology, which is accompanied by malignant hypertension, often does not respond to medical treatment. In patients with MAS, surgical treatment is first line recommendation to prevent complications such as hypertension, heart failure, intracranial bleeding, or aortic rupture...
March 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28420337/right-aortic-arch-analysis-anatomical-variant-or-serious-vascular-defect
#3
Agata Arazińska, Michał Polguj, Konrad Szymczyk, Magdalena Kaczmarska, Łukasz Trębiński, Ludomir Stefańczyk
BACKGROUND: The right-sided aortic arch (RAA) is a rare congenital defect of the aorta. The aim of the study was to assess the occurrence of RAA in diagnoses performed by the University Radiology Department and analyze the frequency of concomitant vascular abnormalities. METHODS: The database of the Radiology Department was retrospectively analyzed between January 2008 and May 2016 with the keyword "right aortic arch". Twenty patients with this diagnosis were identified from a total of 11,690 CT examinations of the chest area, 19,623 CT examinations of brain-supplying vessels, and 1863 MRI examinations of the heart and aortic arch or brain-supplying arteries...
April 19, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28394008/large-thoracic-tumor-without-superior-vena-cava-syndrome
#4
Nikolaos Garmpis, Christos Damaskos, Nikolaos Patelis, Dimitrios Dimitroulis, Eleftherios Spartalis, Ioannis Tomos, Anna Garmpi, Michael Spartalis, Efstathios A Antoniou, Konstantinos Kontzoglou, Periklis Tomos
A 62 year-old male with long-standing smoking history presented with hemoptysis. Plain chest x-ray showed abnormal findings proximate to the right pulmonary hilum. Bronchoscopy revealed a fragile exophytic tumor of the right wall of the lower third of the trachea, infiltrating the right main bronchus (75% stenosis) and the right upper lobar bronchus (near total occlusion). Contrast-enhanced chest CT demonstrated a 7.2x4.9 cm tumor contiguous to the above-mentioned structures, mediastinal lymph node pathology, and a vessel coursing inferiorly to the left of the aortic arch and anterior to the left hilum...
April 10, 2017: Folia Morphologica (Warsz)
https://www.readbyqxmd.com/read/28381879/tfap2b-mutation-and-dental-anomalies
#5
Natchaya Tanasubsinn, Rekwan Sittiwangkul, Yupada Pongprot, Katsushige Kawasaki, Atsushi Ohazama, Thanapat Sastraruji, Massupa Kaewgahya, Piranit Nik Kantaputra
Mutations inTFAP2B has been reported in patients with isolated patent ductus arteriosus (PDA) and Char syndrome. We performed mutation analysis of TFAP2B in 43 patients with isolated PDA, 7 patients with PDA with other congenital heart defects and 286 patients with isolated tooth agenesis with or without other dental anomalies. The heterozygous c.1006G>A mutation was identified in 20 individuals. Those mutation carriers consisted of 1 patient with term PDA (1/43), 16 patients with isolated tooth agenesis with or without other dental anomalies (16/286; 5...
April 6, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/28377475/diagnosis-imaging-and-clinical-management-of-aortic-coarctation
#6
REVIEW
Elles J Dijkema, Tim Leiner, Heynric B Grotenhuis
Coarctation of the aorta (CoA) is a well-known congenital heart disease (CHD), which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV), ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. Despite echocardiographic screening, prenatal diagnosis of CoA remains difficult. Most patients with CoA present in infancy with absent, delayed or reduced femoral pulses, a supine arm-leg blood pressure gradient (>20 mm Hg), or a murmur due to rapid blood flow across the CoA or associated lesions (BAV)...
April 4, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28367436/severe-aortic-stenosis-and-severe-coarctation-of-the-aorta-a-hybrid-approach-to-treatment
#7
Daniel McLennan, Massimo Caputo, Demetris Taliotis
Hybrid surgery is becoming more popular in the treatment of children with congenital heart disease, particularly small infants and neonates. We report a case of a patient with aortic stenosis (AS) and coarctation of the aorta (CoA). CASE: a 1-month-old baby presented with severe AS and CoA. The decision was made to perform a hybrid surgical procedure. The patient underwent a lateral thoracotomy for repair of the CoA and carotid cutdown for aortic balloon valvuloplasty (AoVP).
2017: Frontiers in Surgery
https://www.readbyqxmd.com/read/28363949/incidental-finding-of-anomalous-circumflex-coronary-artery-from-right-coronary-sinus-prior-to-aortic-valve-surgery
#8
Amer Harky, Alexander Hof, Mohammad U Ahmad, Rakesh Uppal
Anomalous origin of the left circumflex (Cx) artery is a common and mostly benign coronary artery anomaly. We report the case of a man aged 52 years who presented to his local hospital with progressive breathlessness on exertion and syncopal episodes. His admission transthoracic echocardiography (TTE) showed bicuspid aortic valve, severe aortic stenosis with a valve area of 0.5 cm(2) and his left ventricular ejection fraction (LVEF) was 27%. His coronary angiogram showed normal coronary arteries but anomalous origin of the Cx artery from the right coronary...
March 31, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28363226/frontiers-of-valvular-heart-disease-from-aortic-stenosis-to-the-tricuspid-valve-and-congenital-anomalies
#9
Thomas F Lüscher
No abstract text is available yet for this article.
March 1, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28337429/investigating-cardiac-motion-patterns-using-synthetic-high-resolution-3d-cardiovascular-magnetic-resonance-images-and-statistical-shape-analysis
#10
Benedetta Biffi, Jan L Bruse, Maria A Zuluaga, Hopewell N Ntsinjana, Andrew M Taylor, Silvia Schievano
Diagnosis of ventricular dysfunction in congenital heart disease is more and more based on medical imaging, which allows investigation of abnormal cardiac morphology and correlated abnormal function. Although analysis of 2D images represents the clinical standard, novel tools performing automatic processing of 3D images are becoming available, providing more detailed and comprehensive information than simple 2D morphometry. Among these, statistical shape analysis (SSA) allows a consistent and quantitative description of a population of complex shapes, as a way to detect novel biomarkers, ultimately improving diagnosis and pathology understanding...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28335833/association-between-cardiovascular-risk%C3%A2-factors-and-aortic-stenosis-the-canheart-aortic-stenosis-study
#11
Andrew T Yan, Maria Koh, Kelvin K Chan, Helen Guo, David A Alter, Peter C Austin, Jack V Tu, Harindra C Wijeysundera, Dennis T Ko
BACKGROUND: Few longitudinal studies have delineated the association between traditional cardiovascular risk factors and development of aortic stenosis (AS). OBJECTIVES: The authors examined the association between traditional cardiovascular risk factors and incident severe AS in a large, unselected elderly population. METHODS: This observational cohort study used multiple linked health care population-based databases of individuals older than 65 years on April 1, 2002, without prior valvular disease, coronary artery disease, heart failure, cardiac arrhythmia, cerebrovascular disease, congenital heart disease, or admissions with cardiac symptoms...
March 28, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28290851/-degenerative-aortic-stenosis-modern-view-on-development-course-and-management
#12
A L Burdeinaya, M S Safarova, M V Ezhov, V V Kukharchuk
Degenerative aortic stenosis is an acquired heart defect manifesting as progressive thickening and calcification of leaflets of originally normal tricuspid or congenital bicuspid aortic valve with development of orifice narrowing, left ventricular hypertrophy, and high risk of cardiovascular complications. In this review we present modern concepts of formation and progression of degenerative aortic stenosis and discuss optimal methods of management of this disease.
June 2016: Kardiologiia
https://www.readbyqxmd.com/read/28287063/fatal-severe-coronary-artery-stenosis-in-williams-syndrome-decision-making-using-late-gadolinium-enhancement-cardiovascular-mri
#13
Inga Voges, Rodney C Franklin, Ricardo Wage, Sonya V Babu-Narayan
Williams syndrome is a well-recognised congenital disorder characterised by cardiovascular, connective tissue, and central nervous system abnormalities. Coronary artery abnormalities are seen in patients with supravalvar aortic stenosis, but end-stage ischaemic heart disease is rare. We report a case of end-stage ischaemic heart disease due to severe coronary arterial stenosis, highlighting how cardiovascular MRI contributed to the management.
March 13, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28259930/high-resolution-single-nucleotide-polymorphism-arrays-identified-an-atypical-microdeletion-of-the-williams-beuren-syndrome-interval-in-a-patient-presenting-with-a-different-phenotype
#14
Shijun Hu, Yifeng Yang, Lin Liu, Zhiping Tan, Tianli Zhao
The present study aimed to identify the mutation causing an atypical syndrome. High-resolution single nucleotide polymorphism (SNP) arrays are considered to be a major detection method for submicroscopic chromosomal rearrangements smaller than 5 Mb in size. Genomic DNA samples of the patient and his parents were converted to a final concentration of 50 ng/ml. The Illumina BeadScan genotyping system and the HumanOmni1‑Quad Chip were employed to obtain the signal intensities of SNP probes. The patient presented with congenital heart disease, autism, mental retardation, growth retardation, hypercalcemia, nephroliths and cleft palate...
May 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28253361/respiratory-syncytial-virus-hospitalization-risk-in-the-second-year-of-life-by-specific-congenital-heart-disease-diagnoses
#15
Deborah Friedman, Jon Fryzek, Xiaohui Jiang, Adam Bloomfield, Christopher S Ambrose, Pierre C Wong
Children with hemodynamically significant congenital heart disease (CHD) are at elevated risk of morbidity and mortality due to respiratory syncytial virus (RSV) disease compared to their healthy peers. Previous studies have demonstrated lower RSV hospitalization risk among all children with CHD at 12-23 months of age versus 0-11 months of age. However, RSV hospitalization risk at 12-23 months of age by specific CHD diagnosis has not been characterized. Both case-control and cohort studies were conducted using data from the US National Inpatient Sample from 1997 to 2013 to characterize relative risk of RSV hospitalization among children 12-23 months of age with CHD...
2017: PloS One
https://www.readbyqxmd.com/read/28238238/bicuspid-aortic-valve-a-review-of-its-genetics-and-clinical-significance
#16
Lei Wang, Li Ming Wang, Wen Chen, Xin Chen
The aim of this review was to describe recent advancements in the understanding of bicuspid aortic valve (BAV). BAV is the most common congenital cardiac anomaly, and affects between 0.46% and 1.37% of the population. There is a male predominance of approximately 3:1.While isolated BAV is found in certain patients, it is often associated with other congenital cardiac lesions, including dilatation of the thoracic aorta, coarctation of the aorta and abnormalities of the coronary anatomy. In most cases, it remains undetected until the patient contracts infective endocarditis, or calcification occurs...
September 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28212480/-reintervention-with-percutaneous-balloon-angioplasty-in-patients-with-congenital-heart-disease-with-left-sided-obstructions
#17
Horacio Márquez-González, Diana López-Gallegos, Nataly Alejandra Pérez-Velázquez, Lucelli Yáñez-Gutiérrez
BACKGROUND: Left-sided cardiac obstructions represent 15% of congenital heart disease (CHD). The treatment in adults is surgical; however, balloon dilation by interventional catheterization can alleviate the symptoms in pediatric patients to allow them to reach the target height. The aim was to determine the survival and the factors associated with reintervention in patients with CHD with left-sided obstruction treated with balloon angioplasty. METHODS: A cohort study was conducted in patients aged 4 to 17 years with left-sided heart obstruction (valvular stenosis [VS], supravalvular aortic stenosis [SAS], coarctation of the aorta [CA]) successfully treated with balloon angioplasty...
2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28185629/the-role-of-adjunctive-procedures-in-reducing-postoperative-tracheobronchial-obstruction-in-single-lung-patients-with-congenital-tracheal-stenosis-undergoing-slide-tracheoplasty
#18
Akiko Yokoi, Yoshihiro Oshima, Eiji Nishijima
PURPOSE: Congenital tracheal stenosis (CTS) and a single right lung treated with slide tracheoplasty (ST) has relatively high rates of mortality and morbidity. We report a single institution's experience with adjunctive procedures at the time of ST to reduce postoperative tracheal obstruction in patients with a single right lung and CTS. METHODS: With IRB approval, 8 patients with a single right lung and CTS who underwent ST in our institution between 2008 and 2016 were reviewed...
January 27, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28182589/bicuspid-aortic-valve-aortopathy-mechanistic-and-clinical-insights-from-recent-studies
#19
David G Guzzardi, Subodh Verma, Paul W M Fedak
PURPOSE OF REVIEW: This focused review summarizes key insights from the past 12 months of basic science and clinical research on bicuspid aortic valve (BAV)-associated aortopathy. RECENT FINDINGS: Recent studies in BAV-associated aortopathy support a heterogeneous spectrum of disease with distinct phenotypes. Basic science studies provide further support for the concept of regional differences in the severity of aortopathy within the aorta of BAV patients. Clinical studies compared outcomes of BAV patients after isolated aortic valve replacement and showed that those with primarily valvular insufficiency as compared with stenosis may be at greater risk for important aortic events over time...
March 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28163921/bioprosthetic-aortic-valve-replacement-12-years-after-percutaneous-aortic-valvuloplasty-in-a-young-female-adult-with-hope-of-pregnancy
#20
Hirohiko Akutsu, Yuichiro Kaminishi, Soki Kurumisawa, Yoshio Misawa
CASE: A 26-year-old woman who had congenital aortic valve stenosis presented with exertional dyspnea. She had undergone percutaneous balloon aortic valvuloplasty 12 years previously at the age of 14. When she was 20 years old, she delivered a neonate by elective cesarean section at the 31st week of gestation because the mean pressure between the left ventricle and the ascending aorta was 52 mmHg. OUTCOME: She successfully underwent aortic valve replacement with a bioprosthetic valve combined with replacement of the ascending aorta in order to make the next pregnancy possible...
October 2016: Acute Medicine & Surgery
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