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congenital critical aortic stenosis

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https://www.readbyqxmd.com/read/30128410/unicuspid-aortic-valve-presenting-with-decompensated-critical-aortic-stenosis
#1
Eunice Yang, Madeline Schiminger, Jose A Madrazo
•UAV is a rare congenital anomaly that leads to severe symptomatic stenosis.•Echocardiography plays a critical role in the evaluation of aortic stenosis.•Correctly distinguishing between UAV and BAV is relevant in determining intervention.
August 2018: CASE: Cardiovascular Imaging Case Reports
https://www.readbyqxmd.com/read/30022603/prenatal-detection-of-critical-cardiac-outflow-tract-anomalies-remains-suboptimal-despite-revised-obstetrical-imaging-guidelines
#2
Heather Y Sun, James A Proudfoot, Rachel T McCandless
BACKGROUND: Fetal echocardiography can accurately diagnose critical congenital heart disease prenatally, but relies on referrals from abnormalities identified on routine obstetrical ultrasounds. Critical congenital heart disease that is frequently missed due to inadequate outflow tract imaging includes anomalies such as truncus arteriosus, double outlet right ventricle, transposition of the great arteries, tetralogy of Fallot, pulmonary stenosis, and aortic stenosis. OBJECTIVE: This study evaluated the prenatal detection rate of critical outflow tract anomalies in a single urban pediatric hospital before and after "AIUM Practice Guideline for the Performance of Obstetric Ultrasound Examinations," which incorporated outflow tract imaging...
July 18, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29960994/discovery-of-an-experimental-model-of-unicuspid-aortic-valve
#3
Robert M Weiss, Yi Chu, Robert M Brooks, Donald D Lund, Justine Cheng, Kathy A Zimmerman, Melissa K Kafa, Phanicharan Sistla, Hardik Doshi, Jian Q Shao, Ramzi N El Accaoui, Catherine M Otto, Donald D Heistad
BACKGROUND: The epithelial growth factor receptor family of tyrosine kinases modulates embryonic formation of semilunar valves. We hypothesized that mice heterozygous for a dominant loss-of-function mutation in epithelial growth factor receptor, which are Egfr Vel/+ mice, would develop anomalous aortic valves, valve dysfunction, and valvular cardiomyopathy. METHODS AND RESULTS: Aortic valves from Egfr Vel/+ mice and control mice were examined by light microscopy at 2...
June 30, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29923134/comparison-of-balloon-dilatation-and-surgical-valvuloplasty-in-non-critical-congenital-aortic-valvular-stenosis-at-long-term-follow-up
#4
Sezen Ugan Atik, Ayşe Güler Eroğlu, Betül Çinar, Murat Tuğberk Bakar, İrfan Levent Saltik
The two main modalities used for congenital aortic valvular stenosis (AVS) treatment are balloon aortic valve dilatation (BAD) and surgical aortic valvuloplasty (SAV). This study evaluates residual and recurrent stenosis, aortic regurgitation (AR) development/progression, reintervention rates, and the risk factors associated with this end point in patients with non-critical congenital AVS who underwent BAD or SAV after up to 18 years of follow-up. From 1990 to 2017, 70 consecutive interventions were performed in patients with AVS, and 61 were included in this study (33 BADs and 28 SAVs)...
June 19, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29404717/pulse-oximetry-findings-in-newborns-with-antenatally-diagnosed-congenital-heart-disease
#5
Isabel E Mawson, Pratusha L Babu, John M Simpson, Grenville F Fox
A retrospective review of admission preductal oxygen saturations of neonates with antenatally diagnosed critical congenital heart disease (CCHD) was performed to investigate the differences in newborn pulse oximetry (Pulsox) by specific CCHD diagnosis. Saturations were recorded at median of < 1 h (range < 1-9 h) after delivery. Data was stratified by CCHD diagnosis and analysed according to the three different admission Pulsox thresholds, ≤ 90, ≤ 92 and ≤ 95%. Of the 276 neonates studied, 208 were clinically well at admission, with no co-morbidities, gestation > 34 weeks and birth weight > 1...
May 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29375228/balloon-aortic-valvuloplasty-in-a-premature-neonate-with-critical-aortic-valve-stenosis-weighing-1493-g
#6
Yin-Yu Lin, Ming-Ren Chen
The use of balloon aortic valvuloplasty for congenital aortic valve stenosis was well established in literatures. However, balloon aortic valvuloplasty performed in low body weight neonates had been infrequently reported. Here we report a 5-day-old premature neonate diagnosed critical aortic valve stenosis. Balloon aortic valvuloplasty was performed as first-line therapy while the patient weighed only 1493 g. Balloon aortic valvuloplasty went successfully with transvalvular pressure gradient decreased from 80 mmHg to 44 mmHg...
January 2018: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/28882282/acute-success-of-balloon-aortic-valvuloplasty-in-the-current-era-a-national-cardiovascular-data-registry-study
#7
MULTICENTER STUDY
Brian A Boe, Jeffrey D Zampi, Kevin F Kennedy, Natalie Jayaram, Diego Porras, Susan R Foerster, Aimee K Armstrong
OBJECTIVES: The aim of this study was to evaluate practice patterns and outcomes of a contemporary group of patients undergoing balloon aortic valvuloplasty (BAV) for congenital aortic stenosis (AS). BACKGROUND: BAV is the most common treatment for isolated congenital AS. METHODS: Within the IMPACT (Improving Pediatric and Adult Congenital Treatments) Registry, all BAV procedures performed between January 2011 and March 2015 were identified...
September 11, 2017: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/28776389/congenital-aortic-stenosis-treatment-outcomes-in-a-nationwide-survey
#8
Merja Kallio, Otto Rahkonen, Ilkka Mattila, Jaana Pihkala
OBJECTIVE: To evaluate treatment outcomes of pediatric valvar aortic stenosis (AS) in a nationwide follow-up. DESIGN: Balloon aortic valvuloplasty (BAV) has been the preferred treatment for congenital AS in Finland since the year 2000. All children treated due to isolated AS during 2000-2014 were included in this retrospective study. Treatment outcomes were categorized into Optimal: residual gradient  ≤35 mmHg and trivial or no aortic regurgitation (AR), Adequate: gradient ≤35 mmHg with mild AR, or Inadequate: gradient >35 mmHg and/or moderate to severe AR...
October 2017: Scandinavian Cardiovascular Journal: SCJ
https://www.readbyqxmd.com/read/28441821/-assessment-of-undiagnosed-critical-congenital-heart-disease-before-discharge-from-the-maternity-hospital
#9
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/28377475/diagnosis-imaging-and-clinical-management-of-aortic-coarctation
#10
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 C o A 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)...
August 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28040141/prenatal-diagnosis-of-smith-magenis-syndrome-in-two-fetuses-with-increased-nuchal-translucency-mild-lateral-ventriculomegaly-and-congenital-heart-defects
#11
Ting-Ying Lei, Ru Li, Fang Fu, Jun-Hui Wan, Yong-Ling Zhang, Xiang-Yi Jing, Can Liao
OBJECTIVE: Smith-Magenis syndrome (SMS) is a multiple congenital anomalies/mental retardation disorder characterized by an interstitial deletion involving chromosome 17p11.2 containing the retinoic acid-induced 1 (RAI1) gene or due to mutation of RAI1. Few cases have been reported in the medical literature regarding prenatal diagnosis of SMS. We report on the prenatal diagnosis of SMS in two fetuses with increased nuchal translucency (NT), mild lateral ventriculomegaly, and congenital heart defects by whole-genome and high-resolution chromosome microarray analysis (CMA)...
December 2016: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27665337/-balloon-valvuloplasty-for-aortic-stenosis-using-umbilical-vein-access-in-a-newborn-first-experience-in-turkey
#12
Kemal Nişli, Serra Karaca, Aygün Dindar
Balloon valvuloplasty is an effective therapy for severe congenital aortic valve stenosis, with mild aortic insufficiency and minimal intermediate-term restenosis. No consensus currently exists regarding optimal vascular approach for balloon dilatation in newborns with critical or severe aortic valve stenosis. Critical aortic valve stenosis in newborns must be treated promptly and effectively. Transcatheter therapy may offer marked advantages, as surgical therapy has been associated with significant rates of morbidity and mortality...
September 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/27452334/whole-exome-sequencing-familial-genomic-triangulation-and-systems-biology-converge-to-identify-a-novel-nonsense-mutation-in-tab2-encoded-tgf-beta-activated-kinase-1-in-a-child-with-polyvalvular-syndrome
#13
Jaeger P Ackerman, John A Smestad, David J Tester, Muhammad Y Qureshi, Beau A Crabb, Nancy J Mendelsohn, Michael J Ackerman
OBJECTIVE: To use whole exome sequencing (WES) of a family trio to identify a genetic cause for polyvalvular syndrome. METHODS AND RESULTS: A male child was born with mild pulmonary valve stenosis and mild aortic root dilatation, and an atrial septal defect, ventricular septal defect, and patent ductus arteriosus that were closed surgically. Subsequently, the phenotype of polyvalvular syndrome with involvement of both semilunar and both atrioventricular valves emerged...
September 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27279868/fetal-cardiac-interventions-clinical-and-experimental-research
#14
REVIEW
Shi-Min Yuan, Gulimila Humuruola
Fetal cardiac interventions for congenital heart diseases may alleviate heart dysfunction, prevent them evolving into hypoplastic left heart syndrome, achieve biventricular outcome and improve fetal survival. Candidates for clinical fetal cardiac interventions are now restricted to cases of critical aortic valve stenosis with evolving hypoplastic left heart syndrome, pulmonary atresia with an intact ventricular septum and evolving hypoplastic right heart syndrome, and hypoplastic left heart syndrome with an intact or highly restrictive atrial septum as well as fetal heart block...
2016: Postępy W Kardiologii Interwencyjnej, Advances in Interventional Cardiology
https://www.readbyqxmd.com/read/27275334/echocardiography-as-a-predicting-method-in-diagnosis-evaluation-and-assessment-of-children-with-subvalvar-aortic-stenosis
#15
Ramush Bejiqi, Hana Bejiqi, Ragip Retkoceri
BACKGROUND: Obstruction to the left ventricular outflow of the heart may be above the aortic valve (5%), at the valve (74%), or in the subvalvar region (23%). These anomalies represent 3 to 6% of all patients with congenital heart defects (CHD), and it occurs more often in males (male-female ratio of 4:1). AIM: The purpose of this study was to determine the sensitivity and specificity of transthoracic echocardiography in diagnosis of discrete subaortic membrane, to determine convenient time for surgical intervention, and for identifying involvement of the aortic valve by subaortic shelf...
March 15, 2016: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/26886784/fetal-interventions-for-congenital-heart-disease
#16
REVIEW
Lindsay R Freud, Wayne Tworetzky
PURPOSE OF REVIEW: This article discusses the rationale, patient selection, technical aspects, and outcomes of percutaneous, ultrasound-guided fetal cardiac intervention (FCI) for structural congenital heart disease. RECENT FINDINGS: FCI is most commonly performed for three forms of congenital heart disease: severe aortic stenosis with evolving hypoplastic left heart syndrome (HLHS), pulmonary atresia with intact ventricular septum and evolving hypoplastic right heart syndrome, and HLHS with intact or highly restrictive atrial septum...
April 2016: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/26491108/notch-tnf-signalling-is-required-for-development-and-homeostasis-of-arterial-valves
#17
Yidong Wang, Bingruo Wu, Emily Farrar, Wendy Lui, Pengfei Lu, Donghong Zhang, Christina M Alfieri, Kai Mao, Ming Chu, Di Yang, Di Xu, Michael Rauchman, Verdon Taylor, Simon J Conway, Katherine E Yutzey, Jonathan T Butcher, Bin Zhou
Aims: Congenital anomalies of arterial valves are common birth defects, leading to valvar stenosis. With no pharmaceutical treatment that can prevent the disease progression, prosthetic replacement is the only choice of treatment, incurring considerable morbidity and mortality. Animal models presenting localized anomalies and stenosis of congenital arterial valves similar to that of humans are critically needed research tools to uncover developmental molecular mechanisms underlying this devastating human condition...
March 1, 2017: European Heart Journal
https://www.readbyqxmd.com/read/26433522/hybrid-procedure-for-neonates-with-hypoplastic-left-heart-syndrome-at-high-risk-for-norwood-midterm-outcomes
#18
Michael O Murphy, Hannah Bellsham-Revell, Gareth J Morgan, Thomas Krasemann, Eric Rosenthal, Shakeel A Qureshi, Caner Salih, Conal B Austin, David R Anderson
BACKGROUND: Hybrid procedure offers patients with severe congenital heart disease an alternative initial procedure to conventional surgical reconstruction. We report the midterm outcomes of a cohort of neonates who had a hybrid procedure for variants of hypoplastic left heart syndrome because they were at high risk for the Norwood procedure. METHODS: Between December 2005 and January 2013, 41 neonates underwent bilateral pulmonary artery banding followed by ductal stenting by means of a sternotomy at a median age of 6 days (range, 2 to 18 days) and weight of 2...
December 2015: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/26219421/is-aortic-valve-leaflet-morphology-predictive-of-outcome-in-pediatric-aortic-valve-stenosis
#19
Rohit S Loomba, Jessica L Bowman, Yumei Cao, James Tweddell, Joseph A Dearani, Pippa M Simpson, Frank Cetta, Andrew N Pelech
BACKGROUND: Initial palliative treatment of congenital aortic valve stenosis consists of either surgical commissurotomy or transcatheter balloon valvuloplasty. The basis for selection of primary approach usually is ill defined. It was hypothesized that aortic valve characteristics may be helpful in directing choice of intervention. This study assesses the impact of valve morphology on time to reintervention after catheter- or surgical-based therapy. METHODS: A retrospective review of preprocedural echocardiographic aortic outflow characteristics was compared with outcomes of primary surgical or balloon valve interventions...
November 2015: Congenital Heart Disease
https://www.readbyqxmd.com/read/26204695/feto-maternal-outcomes-of-urgent-open-heart-surgery-during-pregnancy
#20
Saeid Hosseini, Fahimeh Kashfi, Niloufar Samiei, Amirjamshid Khamoushi, Alireza Alizadeh Ghavidel, Forouzan Yazdanian, Yalda Mirmesdagh, Carlos A Mestres
BACKGROUND AND AIM OF THE STUDY: Cardiac surgery during pregnancy is rarely required and potentially increases feto-maternal mortality. The study aim was to evaluate pregnancy outcomes in females who underwent open-heart surgery with cardiopulmonary bypass (CPB) during pregnancy. METHODS: Between 1999 and 2014, a total of 16 pregnant women (mean age 27 ± 7 years; mean gestational age 13 ± 7.7 weeks) underwent urgent cardiac surgery using CPB. The preoperative diagnosis included prosthetic valve dysfunction in 12 women (five aortic, seven mitral), native valve endocarditis and critical aortic stenosis each in one woman, and intracardiac masses in two women...
March 2015: Journal of Heart Valve Disease
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