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Congenital cardiac defect

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https://www.readbyqxmd.com/read/28074828/three-dimensional-transesophageal-echocardiography-guided-transcathetar-closure-of-ruptured-noncoronary-sinus-of-valsalva-aneurysm
#1
G Anil Kumar, P S Parimala, M Jayaranganath, A M Jagadeesh
Sinus of Valsalva aneurysm accounts for only 1% of congenital cardiac anomalies. Sinus of Valsalva aneurysm can cause aortic insufficiency, coronary artery flow compromise, cardiac arrhythmia, or aneurysm rupture. Three-dimensional transesophageal echocardiography (3DTEE) represents an adjunctive tool to demonstrate the ruptured sinus of Valsalva with better delineation. We present an adult patient with rupture of noncoronary sinus of Valsalva aneurysm into the right atrium (RA). 3DTEE accurately delineated the site of rupture into the RA and showed the exact size and shape of the defect, which helped in the successful transcatheter closure of the defect with a duct occluder device...
January 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28070867/analytical-modeling-for-computing-lead-stress-in-a-novel-epicardial-micropacemaker
#2
Li Zhou, Yaniv Bar-Cohen, Raymond A Peck, Giorgio V Chirikian, Brett Harwin, Ramen H Chmait, Jay D Pruetz, Michael J Silka, Gerald E Loeb
Implantation and maintenance of a permanent cardiac pacing system in children remains challenging due to small patient size, congenital heart defects and somatic growth. We are developing a novel epicardial micropacemaker for children that can be implanted on the epicardium within the pericardial space via a minimally-invasive technique. The key design configurations include a novel open-coiled lead in which living tissue replaces the usual polymeric support for the coiled conductor. To better understand and be able to predict the behavior of the implanted lead, we performed a radiographic image-based modeling study on a chronic animal test...
January 9, 2017: Cardiovascular Engineering and Technology
https://www.readbyqxmd.com/read/28062680/mitral-valve-replacement-using-a-handmade-construct-in-an-infant
#3
Steven Bibevski, Alexandra Levy, Frank G Scholl
We recently reported the feasibility of a handmade left atrioventricular valve using decellularized extracellular matrix for 3 months in a failed repair of AV canal defect. We present the use of an extracellular matrix mitral valve for 9 months in an infant with an un-repairable congenitally malformed valve. The valve functioned perfectly without anticoagulation until the patient expired from non-cardiac complications of their fibrillin gene defect. This report adds further experience using valves made from extracellular matrix in the systemic ventricle both in terms of application and in terms of short-term durability of the construct...
January 6, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28062416/blood-flow-patterns-underlie-developmental-heart-defects
#4
Madeline Midgett, Kent L Thornburg, Sandra Rugonyi
Although cardiac malformations at birth are typically associated with genetic anomalies, blood flow dynamics also play a crucial role in heart formation. However, the relationship between blood flow patterns in the early embryo and later cardiovascular malformation has not been determined. We used the chicken embryo model to quantify the extent to which anomalous blood flow patterns predict cardiac defects that resemble those in humans, and found that restricting either the inflow to the heart or the outflow led to reproducible abnormalities with a dose-response type relationship between blood flow stimuli and the expression of cardiac phenotypes...
January 6, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28060152/an-elevated-low-cardiac-output-syndrome-score-is-associated-with-morbidity-in-infants-after-congenital-heart-surgery
#5
Kalia P Ulate, Ofer Yanay, Howard Jeffries, Harris Baden, Jane L Di Gennaro, Jerry Zimmerman
OBJECTIVES: To evaluate an empirically derived Low Cardiac Output Syndrome Score as a clinical assessment tool for the presence and severity of Low Cardiac Output Syndrome and to examine its association with clinical outcomes in infants who underwent surgical repair or palliation of congenital heart defects. DESIGN: Prospective observational cohort study. SETTING: Cardiac ICU at Seattle Children's Hospital. PATIENTS: Infants undergoing surgical repair or palliation of congenital heart defects...
January 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28054902/novel-goal-directed-hemodynamic-optimization-therapy-based-on-major-vasopressor-during-corrective-cardiac-surgery-in-patients-with-severe-pulmonary-arterial-hypertension-a-pilot-study
#6
Shuwen Li, Qing Ma, Yanwei Yang, Jiakai Lu, Zhiquan Zhang, Mu Jin, Weiping Cheng
INTRODUCTION: Pulmonary arterial hypertension (PAH) is a common and fatal complication of congenital heart disease (CHD). PAH-CHD increases the risk for postoperative complications. Recent evidence suggests that perioperative goal-directed hemodynamic optimization therapy (GDHOT) significantly improves outcomes in surgery patients. Standard GDHOT is based on major solution volume, vasodilators and inotropic therapy, while novel GDHOT is based on major vasopressor and inotropic therapy...
22, 2016: Heart Surgery Forum
https://www.readbyqxmd.com/read/28052532/persistent-left-superior-vena-cava-accompanying-congenital-heart-disease-in-children-experience-of-a-tertiary-care-center
#7
Mehmet Emre Ari, Vehbi Doğan, Senem Özgür, Özben Ceylan, İlker Ertuğrul, Şeyma Kayalı, Tamer Yoldaş, Utku Arman Örün, Özkan Kaya, Selmin Karademir
BACKGROUND: This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD). METHODS: Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3.3%) of whom were diagnosed with persistent left SVC, were evaluated retrospectively. The demographic characteristics of patients, clinical and radiographic findings, echocardiography, cardiac catheterization, and angiography results obtained from the patients' records were reviewed...
January 3, 2017: Echocardiography
https://www.readbyqxmd.com/read/28049534/case-report-maternal-mosaicism-resulting-in-inheritance-of-a-novel-gata6-mutation-causing-pancreatic-agenesis-and-neonatal-diabetes-mellitus
#8
Daphne Yau, Elisa De Franco, Sarah E Flanagan, Sian Ellard, Miriam Blumenkrantz, John J Mitchell
BACKGROUND: Haploinsufficiency of the GATA6 transcription factor gene was recently found to be the most common cause of pancreatic agenesis, a rare cause of neonatal diabetes mellitus. Although most cases are de novo, we describe three siblings with inherited GATA6 haploinsufficiency and the rare finding of parental mosaicism. CASE PRESENTATION: The proband was born at term with severe intrauterine growth restriction, the first child of non-consanguineous parents...
January 3, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28049487/endothelial-microparticles-are-increased-in-congenital-heart-diseases-and-contribute-to-endothelial-dysfunction
#9
Ze-Bang Lin, Hong-Bo Ci, Yan Li, Tian-Pu Cheng, Dong-Hong Liu, Yan-Sheng Wang, Jun Xu, Hao-Xiang Yuan, Hua-Ming Li, Jing Chen, Li Zhou, Zhi-Ping Wang, Xi Zhang, Zhi-Jun Ou, Jing-Song Ou
BACKGROUND: We previously demonstrated that endothelial microparticles (EMPs) are increased in mitral valve diseases and impair valvular endothelial cell function. Perioperative systemic inflammation is an important risk factor and complication of cardiac surgery. In this study, we investigate whether EMPs increase in congenital heart diseases to promote inflammation and endothelial dysfunction. METHODS: The level of plasma EMPs in 20 patients with atrial septal defect (ASD), 23 patients with ventricular septal defect (VSD), and 30 healthy subjects were analyzed by flow cytometry...
January 4, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28043462/right-ventricular-outflow-tract-reconstruction-with-a-polytetrafluoroethylene-monocusp-valve-a-20-year-experience
#10
Mohineesh Kumar, Mark W Turrentine, Mark D Rodefeld, Teresa Bell, John W Brown
In patients with tetralogy of Fallot (TOF), pulmonary atresia (PA), and other congenital right ventricular outflow tract (RVOT) malformations, polytetrafluoroethylene (PTFE) monocusp outflow tract patches (MOTP) relieve obstruction and provide pulmonary valve competence. The purpose of this study was to determine whether our PTFE-MOTP was an acceptable short- and mid-term remedy for patients with TOF or PA as assessed by freedom from severe pulmonary regurgitation and freedom from reoperation. From 1994-2014, 171 patients (mean age 1...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28040762/glial-fibrillary-acidic-protein-plasma-levels-are-correlated-with-degree-of-hypothermia-during-cardiopulmonary-bypass-in-congenital-heart-disease-surgery
#11
Luca Vedovelli, Massimo Padalino, Sara D'Aronco, Giovanni Stellin, Carlo Ori, Virgilio P Carnielli, Manuela Simonato, Paola Cogo
OBJECTIVES: Improved congenital heart defect (CHD) operations have reduced operative mortality to 3%. The major concern is now long-term neurological outcomes. We measured plasma glial fibrillary acidic protein (GFAP), an early marker of brain injury, during different phases of cardiopulmonary bypass (CPB), to correlate the increase of GFAP to clinical parameters or specific operative phases. METHODS: We performed a prospective, single-centre, observational study in children undergoing cardiac operations...
December 31, 2016: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28040751/minimally-invasive-endoscopic-surgery-versus-catheter-based-device-occlusion-for-atrial-septal-defects-in-adults-reconsideration-of-the-standard-of-care
#12
Yvonne Schneeberger, Andreas Schaefer, Lenard Conradi, Jens Brickwedel, Hermann Reichenspurner, Rainer Kozlik-Feldmann, Christian Detter
OBJECTIVES: Percutaneous ostium secundum atrial septal defect (ASD II) closure has become the standard of care for treatment of congenital ASD II in adults. Nevertheless, patients are frequently ineligible for this technique due to challenging morphology. In such cases, closure via minimally invasive cardiac surgery (MICS) is an appropriate treatment option. The aim of this study is to compare outcomes of MICS and use of a percutaneous Amplatzer septal occluder (ASO) device for treatment of ASD II in adults...
December 31, 2016: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28038838/-interventional-cardiac-catheterization-in-congenital-heart-disease
#13
François Godart, Ali Houeijeh
Interventional cardiac catheterization has a major place in the management of congenital heart disease. Since the Rashkind atrioseptostomy in mid-1960s, many techniques have been developed. For some, it is necessary to close a cardiac or extracardiac shunt using occluder (double disc system, plug, coil…): closure of atrial septal defect, ventricular septal defect or patent arterial duct. For others, it is necessary to treat a valvular or vascular stenosis using a balloon catheter: dilatation of the pulmonary or the aortic valve, dilatation of aortic coarctation...
December 27, 2016: La Presse Médicale
https://www.readbyqxmd.com/read/28038803/neu3-sialidase-role-in-activating-hif-1%C3%AE-in-response-to-chronic-hypoxia-in-cyanotic-congenital-heart-patients
#14
Marco Piccoli, Erika Conforti, Alessandro Varrica, Andrea Ghiroldi, Federica Cirillo, Giulia Resmini, Francesca Pluchinotta, Guido Tettamanti, Alessandro Giamberti, Alessandro Frigiola, Luigi Anastasia
BACKGROUND: Hypoxia is a common feature of many congenital heart defects (CHDs) and significantly contributes to their pathophysiology. Thus, understanding the mechanism underlying cell response to hypoxia is vital for the development of novel therapeutic strategies. Certainly, the hypoxia inducible factor (HIF) has been extensively investigated and it is now recognized as the master regulator of cell defense machinery counteracting hypoxic stress. Along this line, we recently discovered and reported a novel mechanism of HIF activation, which is mediated by sialidase NEU3...
December 21, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/28035425/myocardial-stress-perfusion-magnetic-resonance-initial-experience-in-a-pediatric-and-young-adult-population-using-regadenoson
#15
Cory V Noel, Ramkumar Krishnamurthy, Brady Moffett, Rajesh Krishnamurthy
BACKGROUND: Dipyridamole and adenosine are traditional pharmacological stressors for myocardial perfusion. Regadenoson, a selective adenosine A2A agonist, has a lower side effect profile with lower incidence of bronchospasm and bradycardia. There is a growing need for myocardial perfusion assessment within pediatrics. There is no report on the utility of regadenoson as a stress agent in children. OBJECTIVE: To observe the safety and feasibility of regadenoson as a pharmacologic stressor for perfusion cardiac MR in a pilot cohort of pediatric patients weighing more than 40 kg who have congenital heart disease and pediatric acquired heart disease...
December 29, 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/28033081/clinical-databases-and-registries-in-congenital-and-pediatric-cardiac-surgery-cardiology-critical-care-and-anesthesiology-worldwide
#16
David F Vener, Michael Gaies, Jeffrey P Jacobs, Sara K Pasquali
The growth in large-scale data management capabilities and the successful care of patients with congenital heart defects have coincidentally paralleled each other for the last three decades, and participation in multicenter congenital heart disease databases and registries is now a fundamental component of cardiac care. This manuscript attempts for the first time to consolidate in one location all of the relevant databases worldwide, including target populations, specialties, Web sites, and participation information...
January 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28026852/altered-topography-of-systemic-veins-following-lung-tissue-resection-the-effect-on-de-novo-cardiac-implantable-electronic-device-cied-implantation
#17
Roman Steckiewicz, Elżbieta Barbara Świętoń, Katarzyna Rowińska-Berman, Przemysław Stolarz
Venous anomalies discovered on cardiac implantable electronic device (CIED) implantation may hinder both the insertion of cardiac leads and the selection of their optimal intraventricular placement. Such venous anomalies may be a result of congenital vascular defects, e.g. anomalies of the fetal venous system, or be a consequence of earlier cardio- or thoracosurgical procedures. In the case of the latter, the extent of morphometric changes to mediastinal structures may depend on the extent of prior lung tissue resection...
December 27, 2016: Folia Morphologica (Warsz)
https://www.readbyqxmd.com/read/28009949/a-patient-with-quadricuspid-aortic-valve-and-ischemic-stroke
#18
Maximillian Krisper, Clemens Köhncke, Felicitas Escher, Daniel A Morris, Carsten Tschöpe, Burkert Pieske
Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect. Aortic regurgitation is the predominant hemodynamically relevant abnormality in patients with QAV, and the main reason for patients requiring valve surgery. Calcific valve disease of the left heart valves is classified as 'low embolic risk' according to current guidelines. However, it remains an important risk factor of cardiovascular events, including ischemic stroke. A 71-year-old woman presented with new-onset aphasia and hemiparesis of the right side of her body...
July 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28009100/rare-copy-number-variants-in-a-population-based-investigation-of-hypoplastic-right-heart-syndrome
#19
Aggeliki Dimopoulos, Robert J Sicko, Denise M Kay, Shannon L Rigler, Charlotte M Druschel, Michele Caggana, Marilyn L Browne, Ruzong Fan, Paul A Romitti, Lawrence C Brody, James L Mills
BACKGROUND: Hypoplastic right heart syndrome (HRHS) is a rare congenital defect characterized by underdevelopment of the right heart structures commonly accompanied by an atrial septal defect. Familial HRHS reports suggest genetic factor involvement. We examined the role of copy number variants (CNVs) in HRHS. METHODS: We genotyped 32 HRHS cases identified from all New York State live births (1998-2005) using Illumina HumanOmni2.5 microarrays. CNVs were called with PennCNV and prioritized if they were ≥20 Kb, contained ≥10 SNPs and had minimal overlap with CNVs from in-house controls, the Database of Genomic Variants, HapMap3, and Childrens Hospital of Philadelphia database...
December 23, 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
https://www.readbyqxmd.com/read/28008647/palmoplantar-keratoderma-in-costello-syndrome-responsive-to-acitretin
#20
Nareh V Marukian, Jonathan L Levinsohn, Brittany G Craiglow, Leonard M Milstone, Keith A Choate
Costello syndrome (CS) is a multisystem congenital disorder characterized by coarse facial features, cardiac defects, intellectual disability, and predisposition to malignancies. Dermatologic findings can include cutaneous papillomas, skin redundancy, acanthosis nigricans, and keratosis pilaris. Palmoplantar keratoderma (PPK) is present in approximately 76% of patients with CS, with disabling functional consequences in severe cases. We report a case of CS with severe PPK that improved dramatically with systemic administration of acitretin 0...
December 23, 2016: Pediatric Dermatology
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