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congenital cardiac defects

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https://www.readbyqxmd.com/read/29344379/changes-in-contractile-protein-expression-are-linked-to-ventricular-stiffness-in-infants-with-pulmonary-hypertension-or-right-ventricular-hypertrophy-due-to-congenital-heart-disease
#1
Andrew R Bond, Dominga Iacobazzi, Safa Abdul-Ghani, Mohammed Ghorbel, Kate Heesom, Mariangela Wilson, Christopher Gillett, Sarah J George, Massimo Caputo, Saadeh Suleiman, Robert M R Tulloh
Background: The right ventricle (RV) is not designed to sustain high pressure leading to failure. There are no current medications to help RV contraction, so further information is required on adaption of the RV to such hypertension. Methods: The Right Ventricle in Children (RVENCH) study assessed infants with congenital heart disease undergoing cardiac surgery with hypertensive RV. Clinical and echocardiographic data were recorded, and samples of RV were taken from matched infants, analysed for proteomics and compared between pathologies and with clinical and echocardiographic outcome data...
2018: Open Heart
https://www.readbyqxmd.com/read/29341296/the-five-year-survival-of-children-with-down-syndrome-in-norway-1994-2009-differed-by-associated-congenital-heart-defects-and-extracardiac-malformations
#2
Kristoffer Brodwall, Gottfried Greve, Elisabeth Leirgul, Kari Klungsøyr, Henrik Holmstrøm, Stein Emil Vollset, Nina Øyen
AIM: We investigated the prevalence of Down syndrome in a nationwide birth cohort, focusing on congenital heart defects, their associations with extracardiac malformations, and survival. METHODS: National registers were used to identify Norwegian births (1994-2009) and deaths (1994-2014) and updated with hospital diagnoses. We estimated birth defect frequencies in Down syndrome and the general population, the association between heart defects and extracardiac malformations and hazard ratios for death from different combinations of heart defects and extracardiac malformations...
January 17, 2018: Acta Paediatrica
https://www.readbyqxmd.com/read/29337121/the-society-of-thoracic-surgeons-congenital-heart-surgery-database-2018-update-on-outcomes-and-quality
#3
Jeffrey P Jacobs, John E Mayer, Sara K Pasquali, Kevin D Hill, David M Overman, James D St Louis, S Ram Kumar, Carl L Backer, Charles D Fraser, James S Tweddell, Marshall L Jacobs
The Society of Thoracic Surgeons Congenital Heart Surgery Database is a comprehensive clinical outcomes registry which captures almost all pediatric cardiac surgical operations in the United States. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency...
January 11, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29336632/m%C3%A3-bius-syndrome-with-cardiac-rhabdomyomas
#4
Aimee Verner, Swati Agarwal-Sinha, Frank Yanfeng Han
BACKGROUND: Möbius syndrome is a rare congenital condition which presents not merely with 6th and 7th nerve palsies, but involves gaze paresis associated with craniofacial, limb, and other abnormalities. Heterogeneity is well known in patients with Möbius syndrome and rather than being of familial inheritance based on rare cases, it is much more recognized as a sporadic syndrome. We report an infant with features of congenital Möbius syndrome associated with cardiac rhabdomyomas in the absence of tuberous sclerosis...
January 16, 2018: Ophthalmic Genetics
https://www.readbyqxmd.com/read/29332374/transcatheter-aortic-valve-replacement-in-bicuspid-aortic-valve-stenosis-where-do-we-stand
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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