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Congenital aortic stenosis

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https://www.readbyqxmd.com/read/29455791/criss-cross-heart-transthoracic-echocardiographic-features
#1
Devi Manuel, Gopal Ghosh, George Joseph, Anandaroop Lahiri, Paul V George
OBJECTIVE: To study the echocardiographic features of criss-cross heart (CCH), a congenital cardiac anomaly characterized by crossed ventricular inflow streams, in Indian patients. METHODS: In this retrospective observational study, all pediatric echocardiograms performed in a single tertiary care institution in South India over a three-year period were scrutinized for a diagnosis of CCH. Demographic, clinical and echocardiographic data were collected from patients' medical records and echocardiographic database...
January 2018: Indian Heart Journal
https://www.readbyqxmd.com/read/29455772/prenatal-diagnosis-of-congenital-heart-disease-a-review-of-current-knowledge
#2
REVIEW
Nathalie Jeanne Bravo-Valenzuela, Alberto Borges Peixoto, Edward Araujo Júnior
This article reviews important features to improve the diagnosis of congenital heart disease (CHD) by applying ultrasound in prenatal cardiac screening. As low and high-risk pregnancies for CHD are subject to routine obstetric ultrasound, the diagnosis of structural heart defects represents a challenge that involves a team of specialists and subspecialists on fetal ultrasonography. In this review, the images highlight normal anatomy of the heart as well as pathologic cases consistent with cardiac malposition and isomerism, septal defects, pulmonary stenosis/atresia, aortic malformations, hypoplastic left ventricle, conotruncal anomalies, tricuspid dysplasia, and Ebstein's anomaly, and univentricular heart, among other congenital cardiovascular defects...
January 2018: Indian Heart Journal
https://www.readbyqxmd.com/read/29448010/improved-outcomes-in-management-of-hypoplastic-left-heart-syndrome-associated-with-congenital-diaphragmatic-hernia-an-algorithmic-approach
#3
Kaitlin Balduf, T K Susheel Kumar, Umar Boston, Shyam Sathanandam, Marc V Lee, Tim Jancelewicz, Christopher J Knott-Craig
BACKGROUND: Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival of neonates with CDH and HLHS is only 1-5%. We review our experience with CDH and HLHS and compare our outcomes to published literature. METHODS: Retrospective review of all neonates with CDH and HLHS at our institution over a 10 year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies and outcomes were reviewed and an algorithmic approach is proposed...
February 12, 2018: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29433351/transcatheter-pulmonary-valve-implantation-will-it-replace-surgical-pulmonary-valve-replacement
#4
Ahmed Kheiwa, Punag Divanji, Vaikom S Mahadevan
Right ventricular outflow tract (RVOT) dysfunction is a common hemodynamic challenge for adults with congenital heart disease (ACHD), including patients with repaired tetralogy of Fallot (TOF), truncus arteriosus (TA), and those who have undergone the Ross procedure for congenital aortic stenosis and the Rastelli repair for transposition of great vessels. Pulmonary valve replacement (PVR) has become one of the most common procedures performed for ACHD patients. Areas covered: Given the advances in transcatheter technology, we conducted a detailed review of the available studies addressing the indications for PVR, historical background, evolving technology, procedural aspects, and the future direction, with an emphasis on ACHD patients...
February 12, 2018: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/29415779/severe-bicuspid-aortic-stenosis-in-pregnancy-balancing-the-risk-of-prematurity-and-maternal-mortality
#5
Chinedu Nwabuobi, Megan McDowell, Sarah Običan
We report a case of combined severe aortic stenosis and regurgitation in a pregnant patient with a history of congenital bicuspid aortic valve. The patient presented at 22 weeks of gestation with angina and pre-syncopal symptoms. During her admission, she experienced intermittent episodes of non-sustained ventricular tachycardia and hypotension. A multi-disciplinary healthcare team was assembled to decide on the appropriate medical and surgical treatment options. At 28 weeks of gestation, the patient underwent a caesarean delivery immediately followed by a mechanical aortic valve replacement...
February 8, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29414430/surgical-pathology-of-subaortic-septal-myectomy-histology-skips-over-clinical-diagnosis
#6
João Abecasis, Rosa Gouveia, Mariana Castro, Maria João Andrade, Regina Ribeiras, Sância Ramos, Miguel Abecasis, Nuno Cardim, Victor Gil
BACKGROUND: Subaortic septal myectomy is usually performed to mitigate obstruction in patients with the obstructive form of hypertrophic cardiomyopathy (HCM) or in those with congenital subaortic stenosis. Moreover, it is combined with aortic valve replacement in patients with severe aortic valve stenosis (SAS) and asymmetrical septal hypertrophy causing concomitant left ventricular outflow tract obstruction. When both conditions coexist, it is conceptually difficult to identify a cardiomyopathy beyond an adaptive myocardial hypertrophy, strictly related to pressure overload...
January 3, 2018: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/29410185/evaluation-of-the-adult-congenital-heart-surgery-mortality-score-at-two-european-centers
#7
Jürgen Hörer, Emre Belli, Régine Roussin, Emanuel LeBret, Mohamed Ly, Jarrah Abdullah, Raffaella Marzullo, Martina Strbad, Julie Cleuziou, Jelena Pabst von Ohain, Rüdiger Lange
BACKGROUND: The Adult Congenital Heart Surgery (ACHS) score was derived from the Society of Thoracic Surgeons Congenital Heart Surgery Database. The score was validated with data of 1,603 operations and reached a good predictive power. We sought to evaluate its predictive power on 1,654 operations performed in 2 European centers. METHODS: Data of all consecutive patients aged 18 years or more, who underwent surgery for congenital heart disease between 2004 and 2013 at Center 1 (n=830), and between 2005 and 2016 at Center 2 (n=824), were collected...
February 1, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29404717/pulse-oximetry-findings-in-newborns-with-antenatally-diagnosed-congenital-heart-disease
#8
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...
February 5, 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29391147/aortic-valve-repair-of-a-stenotic-unicuspid-aortic-valve-in-young-patients
#9
Adrian Kolesar, Tomas Toporcer, Milan Bajmoczi, Jan Luczy, Peter Candik, Frantisek Sabol
BACKGROUND: The unicuspid aortic valve (UAV) is a well-described pediatric congenital abnormality, with incidence of 0.02% in the general population. Bicuspidization has been described as a potential surgical option to repair this defect. METHODS: Seventeen symptomatic young patients with a unicuspid valve combined with either valve insufficiency or valve stenosis underwent aortic valve bicuspidization procedure using either an equine pericardium. In addition to bicuspidization, eight patients underwent aortic ring implantation and five patients underwent supracoronary replacement of the aorta...
January 29, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29377232/subvalvular-aortic-stenosis-a-review-of-current-literature
#10
REVIEW
Subodh R Devabhaktuni, Eyas Chakfeh, Ali O Malik, Joshua A Pengson, Jibran Rana, Chowdhury H Ahsan
Subvalvular aortic stenosis (SAS) is one of the common adult congenital heart diseases, with a prevalence of 6.5%. It is usually diagnosed in the first decade of life. Echocardiography is the test of choice to diagnose SAS. Surgical correction is the best treatment modality, and the prognosis is usually excellent. In this review, we describe the pathophysiology, diagnosis, prognosis, and management of SAS with a focus on different pathophysiologic mechanisms, diagnostic approach, and prognosis of the disease by reviewing the current literature...
January 29, 2018: Clinical Cardiology
https://www.readbyqxmd.com/read/29375228/balloon-aortic-valvuloplasty-in-a-premature-neonate-with-critical-aortic-valve-stenosis-weighing-1493-g
#11
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/29346529/echocardiographic-criteria-to-detect-unicuspid-aortic-valve-morphology
#12
Sebastian Ewen, Irem Karliova, Petra Weber, Stephan H Schirmer, Hashim Abdul-Khaliq, Jakob Schöpe, Felix Mahfoud, Hans-Joachim Schäfers
Aims: Unicuspid aortic valve (UAV) is a rare congenital malformation associated with severe aortic stenosis or regurgitation. This study aimed to systematically determine echocardiographic criteria to identify UAV. Methods and results: All patients underwent a preoperative baseline examination, including echocardiography. A total of 69 patients with intraoperatively confirmed UAV underwent an aortic valve repair procedure between August 2001 and May 2011. To compare the findings of UAV cases with those of other valve morphologies, we examined 99 consecutive patients with a bicuspid aortic valve (BAV) and 103 consecutive patients with a tricuspid aortic valve (TAV) undergoing isolated aortic valve surgery before May 2016...
January 15, 2018: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/29332374/transcatheter-aortic-valve-replacement-in-bicuspid-aortic-valve-stenosis-where-do-we-stand
#13
Sung-Han Yoon, Rahul Sharma, Tarun Chakravarty, Masaki Miyasaka, Tomoki Ochiai, Takahiro Nomura, Norman Gellada, Shadi Nemanpour, Mamoo Nakamura, Wen Chen, Raj Makkar
Bicuspid aortic valve is the most common congenital cardiac defect in adults, and symptom typically develops in adulthood. In the majority of cases, bicuspid aortic valve disease progress with ages and surgical aortic valve replacement is performed with excellent operative outcomes. However, with the relatively slow progression of disease, surgical aortic valve replacement is required in elderly patients but the surgical risk often deemed extremely high due to old age and multiple comorbidities. Transcatheter aortic valve replacement (TAVR) has evolved from a novel technology to an established therapy for intermediate- and high-risk patients with symptomatic severe aortic valve stenosis (AS)...
January 11, 2018: Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/29327146/mid-term-outcome-of-100-consecutive-ross-procedures-excellent-survival-but-yet-to-be-a-cure
#14
Corina Zimmermann, Christine Attenhofer Jost, René Prêtre, Christoph Mueller, Matthias Greutmann, Burkhardt Seifert, Emanuela Valsangiacomo Büchel, Oliver Kretschmar, Hitendu Hasmukhlal Dave, Roland Weber
The Ross procedure offers excellent short-term outcome but the long-term durability is under debate. Reinterventions and follow-up of 100 consecutive patients undergoing Ross Procedure at our centre (1993-2011) were analysed. Follow-up was available for 96 patients (97%) with a median duration of 5.3 (0.1-17.1) years. Median age of the patient cohort was 15.2 (0.04-58.4) years with 76 males. 93% had underlying congenital aortic stenosis. Root replacement technique was applied in all. The most common valved conduits used for reconstruction of the right ventricular outflow tract were homografts (66 patients) and bovine jugular vein (ContegraR) graft (31 patients)...
January 12, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29302949/simultaneous-transcatheter-intervention-for-coarctation-of-the-aorta-and-bicuspid-aortic-valve
#15
Yukiko Mizutani, Norio Tada, Takahiko Masuda, Masaki Hata
Coarctation of the aorta (CoA) is a relatively common congenital heart anomaly, and bicuspid aortic valve (BAV) is a common congenital heart disease that coexists with CoA. In larger children and adults with CoA, transcatheter intervention has gained acceptance, but for surgical high-risk patients with aortic stenosis, the use of transcatheter aortic valve implantation (TAVI) has been established. Recently, although favorable data have been reported for TAVI when treating BAV, simultaneous transcatheter intervention for CoA and BAV will prove to be a challenge because of the unique anatomy involved requires multiple procedural steps and also has problems of site access...
July 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/29300374/clinical-history-and-management-recommendations-of-the-smooth-muscle-dysfunction-syndrome-due-to-acta2-arginine-179-alterations
#16
Ellen S Regalado, Lauren Mellor-Crummey, Julie De Backer, Alan C Braverman, Lesley Ades, Susan Benedict, Timothy J Bradley, M Elizabeth Brickner, Kathryn C Chatfield, Anne Child, Cori Feist, Kathryn W Holmes, Glen Iannucci, Birgit Lorenz, Paul Mark, Takayuki Morisaki, Hiroko Morisaki, Shaine A Morris, Anna L Mitchell, John R Ostergaard, Julie Richer, Denver Sallee, Sherene Shalhub, Mustafa Tekin, Anthony Estrera, Patricia Musolino, Anji Yetman, Reed Pyeritz, Dianna M Milewicz
PurposeSmooth muscle dysfunction syndrome (SMDS) due to heterozygous ACTA2 arginine 179 alterations is characterized by patent ductus arteriosus, vasculopathy (aneurysm and occlusive lesions), pulmonary arterial hypertension, and other complications in smooth muscle-dependent organs. We sought to define the clinical history of SMDS to develop recommendations for evaluation and management.MethodsMedical records of 33 patients with SMDS (median age 12 years) were abstracted and analyzed.ResultsAll patients had congenital mydriasis and related pupillary abnormalities at birth and presented in infancy with a patent ductus arteriosus or aortopulmonary window...
January 4, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/29287136/cerebrovascular-hemodynamics-in-fetuses-with-congenital-heart-disease
#17
Tingting Man, Yihua He, Ying Zhao, Lin Sun, Xiaowei Liu, Shuping Ge
BACKGROUND AND OBJECTIVE: It is hypothesized that diminished cerebral vascular resistance or the "brain sparing effect" is associated with fetuses with complex congenital heart defects (CHD) and may affect their neurodevelopmental outcome. An alternative explanation is that it is related to the location, cardiac output, pressure, and resistance in left heart obstructive CHDs. We sought to determine the effects of various left and right heart obstructive defects on the cerebral and placental hemodynamics and to evaluate the utility of these variables for the assessment and prognosis of CHDs...
December 2017: Echocardiography
https://www.readbyqxmd.com/read/29276909/-management-of-patients-with-isolated-aortic-stenosis-considering-negative-prognostic-factors
#18
I G Nikitin, G E Gendlin, E I Emelina, A I Kovaleva
BACKGROUND: Aortic stenosis (AS) is the most common valve defect in developed countries; its prevalence increases with age of the population. The most frequent cause for isolated AS in adults is calcification of normal tricuspid or congenital bicuspid aortic valve (AV). The only effective treatment of AS is aortic valve replacement (AVR). Major negative prognostic factors include low LV ejection fraction (EF), age older than 70, female gender, and comorbidities such as renal dysfunction, chronic obstructive pulmonary disease, and neural disorders...
2017: Kardiologiia
https://www.readbyqxmd.com/read/29274695/diminutive-porcelain-ascending-aorta-with-supravalvular-aortic-stenosis
#19
Mustafa Houmsse, Asia McDavid, Ahmet Kilic
This case report describes a case of a 49-year-old male, with a past medical history significant for congenital aortic stenosis, who presented with progressive shortness of breath and decreased stamina. The patient was found to have a concentric, diminutive porcelain ascending aorta with diffuse supravalvular aortic stenosis. Herein we describe treatment with an aortic root augmentation and Bentall procedure utilizing hypothermic circulatory arrest.
December 21, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29202750/assessment-of-tetralogy-of-fallot-associated-congenital-extracardiac-vascular-anomalies-in-pediatric-patients-using-low-dose-dual-source-computed-tomography
#20
Bi-Yue Hu, Ke Shi, Yu-Ping Deng, Kai-Yue Diao, Hua-Yan Xu, Rui Li, Zhi-Gang Yang, Ying-Kun Guo
BACKGROUND: To investigate the diagnostic value of dual-source computed tomography (DSCT) in the evaluation of tetralogy of Fallot (TOF)-associated extracardiac vascular abnormalities in pediatric patients compared with transthoracic echocardiography (TTE). METHODS: One hundred and twenty-three pediatric patients diagnosed with TOF were included in this retrospective study. All patients underwent DSCT and TTE preoperatively. All associated extracardiac vascular abnormalities and their percentages were recorded...
December 4, 2017: BMC Cardiovascular Disorders
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