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Cardiac congenital defects

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https://www.readbyqxmd.com/read/29332374/transcatheter-aortic-valve-replacement-in-bicuspid-aortic-valve-stenosis-where-do-we-stand
#1
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/29326834/ablation-of-atrial-fibrillation-in-patients-with-congenital-heart-disease
#2
Marwan M Refaat, Jad Ballout, Moussa Mansour
With improved surgical techniques and medical management for patients with congenital heart diseases, more patients are living longer and well into adulthood. This improved survival comes with a price of increased morbidity, mainly secondary to increased risk of tachyarrhythmias. One of the major arrhythmias commonly encountered in this subset of cardiac patients is AF. Similar to the general population, the risk of AF increases with advancing age, and is mainly secondary to the abnormal anatomy, abnormal pressure and volume parameters in the hearts of these patients and to the increased scarring and inflammation seen in the left atrium following multiple surgical procedures...
December 2017: Arrhythmia & Electrophysiology Review
https://www.readbyqxmd.com/read/29326113/acute-heart-failure-with-new-onset-continuous-murmur-in-a-26-year-old-man
#3
Yi-Chen Wang, Chen-Wei Huang, Ting-Wei Lin
CLINICAL INTRODUCTION: A 26-year-old man with an unremarkable medical history sensed a momentary retrosternal 'pop' followed by overwhelming dyspnoea, without reporting any prodromal symptoms. At the emergency department, he had a significant tachycardia (170 bpm) and an extreme low diastolic blood pressure (<30 mm Hg). A loud, harsh and continuous murmur could be maximally auscultated at the right lower sternal border. ECG revealed global ST depression with ST elevation in lead aVR...
January 11, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29326110/contemporary-management-and-outcomes-in-congenitally-corrected-transposition-of-the-great-arteries
#4
REVIEW
Shelby Kutty, David A Danford, Gerhard-Paul Diller, Oktay Tutarel
Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration...
January 11, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29317056/a-potentially-curative-fetal-intervention-for-hypoplastic-left-heart-syndrome
#5
Andrew T Hattam
Hypoplastic left heart syndrome (HLHS) encapsulates a spectrum of complex congenital cardiovascular malformations involving varying degrees of underdevelopment of the left-sided heart structures. However, despite improved survival rates since the introduction of staged surgical reconstruction, treatment options for HLHS remain palliative rather than curative. A major limiting factor in the development of definitive curative therapy for HLHS is an incomplete understanding of its pathogenesis. Currently, the aetiology HLHS is best conceptualised by the 'flow theory' of cardiogenesis, which states that normal cardiac development is reliant on the interrelationship of normal flow patterns of blood through the developing heart, and appropriate growth of the cardiac valves and myocardium...
January 2018: Medical Hypotheses
https://www.readbyqxmd.com/read/29310562/programmatic-changes-to-reduce-mortality-and-morbidity-in-humanitarian-congenital-cardiac-surgery
#6
Tyler J Wallen, George J Arnaoutakis, Randa Blenden, Rodrigo Soto
BACKGROUND: This report documents the outcomes of cardiac surgical mission trips organized by the International Children's Heart Foundation (ICHF), a nongovernmental organization that provides congenital heart surgery services to the developing world, and discusses factors associated with a reduction of mortality and morbidity in this setting. METHODS: A retrospective review of a prospectively maintained database was conducted to identify any patient who underwent surgical intervention during the course of an ICHF mission trip...
January 2018: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29310559/arrhythmias-in-children-in-early-postoperative-period-after-cardiac-surgery
#7
Manoj Kumar Sahu, Anupam Das, Bharat Siddharth, Sachin Talwar, Sarvesh Pal Singh, Atul Abraham, Arin Choudhury
BACKGROUND: Postoperative arrhythmias are a known complication after cardiac surgical repairs for congenital heart disease. METHODS: Data were reviewed pertaining to incidence, diagnosis, potential risk factors, and management of postoperative arrhythmias in 369 consecutive patients under 18 years of age, undergoing elective open heart surgery. All children were admitted to the intensive care unit and continuous electrocardiographic monitoring was performed. Patient factors such as Aristotle Basic Complexity Score, total surgical duration, hypotension, tachycardia, serum lactate level, and inotropic score were analyzed...
January 2018: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29310556/coexistence-of-right-ventricular-myxoma-and-atrioventricular-septal-defect
#8
Shigeaki Aoyagi, Tomokazu Kosuga, Kumiko Wada, Eiji Nakamura, Hiroshi Yasunaga
Although a small number of atrial myxomas are found in association with congenital cardiac defects, ventricular myxomas coexisting with congenital cardiac anomalies are extremely rare. We report a case of right ventricular (RV) myxoma coexistent with atrioventricular septal defect in an adolescent. Echocardiography showed an RV mass, a small ostium primum atrial septal defect, and a cleft of the left atrioventricular valve. Magnetic resonance imaging revealed a mass, suggesting a myxoma. The mass was excised simultaneously with repair of the anomalies and was histologically confirmed as a myxoma...
January 2018: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29306027/trichloroethylene-perturbs-hnf4a-expression-and-activity-in-the-developing-chick-heart
#9
Alondra P Harris, Kareem A Ismail, Martha Nunez, Ira Martopullo, Alejandro Lencinas, Ornella I Selmin, Raymond B Runyan
Exposure to trichloroethylene (TCE) is linked to formation of congenital heart defects in humans and animals. Prior interactome analysis identified the transcription factor, Hepatocyte Nuclear Factor 4 alpha (HNF4a), as a potential target of TCE exposure. As a role for HNF4a is unknown in the heart, we examined developing avian hearts for HNF4a expression and for sensitivity to TCE and the HNF4a agonist, Benfluorex. In vitro analysis using a HNF4a reporter construct showed both TCE and HFN4a to be antagonists of HNF4a-mediated transcription at the concentrations tested...
January 3, 2018: Toxicology Letters
https://www.readbyqxmd.com/read/29305187/cardiac-arrest-and-ventricular-arrhythmia-in-adults-with-ebstein-anomaly-and-left-ventricular-non-compaction
#10
Magdalena Kumor, Magdalena Lipczyńska, Elzbieta Katarzyna Biernacka, Anna Klisiewicz, Anna Wójcik, Marek Konka, Katarzyna Kożuch, Piotr Szymański, Piotr Hoffman
BACKGROUND: Ebstein anomaly is a complex, congenital heart defect that is associated with a variety of cardiac abnormalities. Studies found a similar sarcomere gene mutation in patients with Ebstein anomaly (EA) and patients with isolated left ventricular non-compaction (LVNC). AIM: We aimed to show the prevalence of LVNC and its potential relationship with severe cardiac events (VT - ventricular tachycardia, cardiac arrest) in adult patients with EA. METHODS: We conducted a retrospective search of our institutional database from 2010 to 2014 for patients with EA and reviewed patients' medical records (age, sex, clinical presentation, electrocardiographic, echocardiographic, and CMR - cardiac magnetic resonance features)...
January 2, 2018: Journal of Cardiology
https://www.readbyqxmd.com/read/29302951/percutaneous-therapy-of-a-stenotic-parachute-mitral-valve-previously-treated-by-surgery
#11
Roberta De Rosa, Dietmar Schranz, Mariuca Vasa-Nicotera, Birgit Assmus, Petar Risteski, Anton Moritz, Andreas M Zeiher, Stephan Fichtlscherer
Parachute mitral valve (PMV) is a congenital heart anomaly which consists of a unifocal attachment of the mitral valve chordae into a single or dominant papillary muscle. This morphological anomaly determines the impairment of mitral leaflet motion, resulting in different grades of mitral stenosis. Due to its frequent association with other congenital cardiac defects requiring surgical correction, the therapy of a relevant stenotic PMV is usually represented by surgical commissurotomy. Herein is reported the case of a PMV treated by surgery in infancy, which showed a severe restenosis after 34 years and was successfully treated by percutaneous valvuloplasty with the additional creation of a restrictive atrial communication...
July 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/29302749/simultaneous-repair-of-congenital-heart-defects-and-pectus-excavatum-in-young-children
#12
Junke Wang, Quan Wang, Zhengxia Pan
PURPOSE: The present study was undertaken to summarize our experience with the concurrent repair of pectus excavatum (PE) and congenital heart defects in young children over the past 15 years. METHODS: A retrospective study was conducted that included patients who underwent combined repair of PE and a congenital heart defect in a large clinical center between 2002 and 2017. Intraoperative and postoperative patient characteristics, postoperative complications and surgical outcomes were recorded...
January 5, 2018: Pediatric Surgery International
https://www.readbyqxmd.com/read/29301846/epithelial-properties-of-the-second-heart-field
#13
REVIEW
Claudio Cortes, Alexandre Francou, Christopher De Bono, Robert G Kelly
The vertebrate heart tube forms from epithelial progenitor cells in the early embryo and subsequently elongates by progressive addition of second heart field (SHF) progenitor cells from adjacent splanchnic mesoderm. Failure to maximally elongate the heart results in a spectrum of morphological defects affecting the cardiac poles, including outflow tract alignment and atrioventricular septal defects, among the most common congenital birth anomalies. SHF cells constitute an atypical apicobasally polarized epithelium with dynamic basal filopodia, located in the dorsal wall of the pericardial cavity...
January 5, 2018: Circulation Research
https://www.readbyqxmd.com/read/29298094/a-rare-rs139365823-polymorphism-in-pre-mir-138-is-associated-with-risk-of-congenital-heart-disease-in-a-chinese-population
#14
Xiaobo Gao, Liping Yang, Haiyan Luo, Fengwei Tan, Xu Ma, Cailing Lu
miR-138 modulates cardiac morphogenesis in zebrafish. We explored whether a genetic polymorphism in miR-138 might contribute to the occurrence of sporadic congenital heart disease (CHD) and the potential mechanism. We performed a case-control study consisting of 857 CHD cases and 938 non-CHD controls by genotyping miR-138 in a Chinese population. Two SNPs, including rare rs139365823 located in the pre-miR-138 sequence and rs76987351 located in the pri-miR-138 sequence, were identified by sequencing miR-138...
January 3, 2018: DNA and Cell Biology
https://www.readbyqxmd.com/read/29297190/pulmonary-valve-and-right-ventricular-outflow-tract-surgery-in-adults-23-year-experience
#15
Marcin Szczechowicz, Sabreen Mkalaluh, Mina Farag, Ahmed Mashhour, Tsvetomir Loukanov, Jacek Kolcz, Matthias Karck, Alexander Weymann
BACKGROUND: Surgery of the pulmonary valve, right ventricular outflow tract, and pulmonary artery falls under the domain of paediatric cardiac surgery. However, 97 adult patients underwent such operations in our establishment from 1993 to 2016. AIM: This study aims to analyse preoperative risk factors, intraoperative data, postoperative outcomes, and long-term survival to identify the potential predictors of mortality and high-risk patients.
 METHODS: We divided our patient cohort into three groups in accordance with surgical indications: 17 patients with pulmonary valve endocarditis (group A), 70 patients with congenital defects involving the pulmonary valve (group B), and 10 patients who underwent pulmonary valve surgery for other indications, like tumor or other acquired valvular disease (group C)...
January 3, 2018: Kardiologia Polska
https://www.readbyqxmd.com/read/29296065/uhl-s-anomaly-a-one-and-a-half-ventricular-repair-in-a-patient-presenting-with-cardiac-arrest
#16
Reginald Chounoune, Adam Lowry, Karthik Ramakrishnan, Gail D Pearson, Jeffrey P Moak, Dilip S Nath
Uhl's anomaly, first reported in 1952, is an extremely rare congenital cardiac defect characterized by partial or complete loss of the right ventricular myocardium and unknown etiology. Fewer than 100 cases have been described. The response to medical management is poor and there is no known ideal surgical approach or timing for treatment. We report the case of a previously active adolescent male presenting with cardiac arrest, who underwent successful bidirectional cavopulmonary anastomosis ("Glenn" anastomosis) with right atrial reduction and right ventricular free wall plication...
January 2018: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/29287138/4d-fetal-echocardiography-an-update
#17
Greggory R DeVore, Gary Satou, Mark Sklansky
With the introduction of the electronic 4-dimensional and spatial-temporal image Correlation (e-STIC), it is now possible to obtain large volume datasets of the fetal heart that are virtually free of artifact. This allows the examiner to use a number of imaging modalities when recording the volumes that include two-dimensional real time, power and color Doppler, and B-flow images. Once the volumes are obtained, manipulation of the volume dataset allows the examiner to recreate views of the fetal heart that enable examination of cardiac anatomy...
December 2017: Echocardiography
https://www.readbyqxmd.com/read/29287136/cerebrovascular-hemodynamics-in-fetuses-with-congenital-heart-disease
#18
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/29286939/clinical-profile-and-pattern-of-congenital-heart-disease-in-infants-of-diabetic-mother-and-infants-of-non-diabetic-mother-at-a-tertiary-care-hospital
#19
Suraiya Begum, Sanjoy Kumar Dey
OBJECTIVES: To assess the clinical manifestations and pattern of congenital heart diseases (CHD) in infants of diabetic mothers (IDMs) and infants of non-diabetic mothers. METHODS: A prospective cross sectional study was carried out at tertiary care center over a period of thirty months. All neonates were included in this study if CHD were identified by echocardiography within this study period. Chi-square test and Fisher's exact test were used to compare between groups...
2017: Journal of Neonatal-perinatal Medicine
https://www.readbyqxmd.com/read/29286273/the-2017-seventh-world-congress-of-pediatric-cardiology-cardiac-surgery-week-in-review-imaging
#20
Elif S Selamet Tierney
The Imaging Program at the 7th World Congress highlighted the versatility and diagnostic power of the current and upcoming imaging tools in Pediatric Cardiology and Cardiac Surgery. Several experts presented interesting as well as practical data on the use of 2D and 3D Echocardiography, magnetic resonance imaging and computed tomography in the fetus, child, and adult with congenital heart disease. Bridging sessions coupled use of these imaging modalities and screening practices in patients with acquired heart disease...
December 2017: Cardiology in the Young
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