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Cardiac congenital defects

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https://www.readbyqxmd.com/read/28103457/-congenital-heart-disease-in-children-with-down-syndrome-what-has-changed-in-the-last-three-decades
#1
Filipa Mestre Dias, Susana Cordeiro, Isabel Menezes, Graça Nogueira, Ana Teixeira, Marta Marques, Miguel Abecasis, Rui Anjos
INTRODUCTION: The prevalence of Down syndrome has increased in the last 30 years; 55% of these children have congenital heart disease. MATERIAL AND METHODS: A retrospective longitudinal cohort study; clinical data from 1982 to 2013 databases with the diagnosis of Down syndrome or trisomy 21 in a reference hospital in pediatric cardiology and cardiac surgery. OBJECTIVE: to assess the progress in the last three decades of cardiological care given to children with Down syndrome and congenital heart disease...
October 2016: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/28100980/accessory-mitral-valve-leaflet-causing-severe-left-ventricular-outflow-tract-obstruction-in-a-preterm-neonate-with-a-partial-atrioventricular-septal-defect
#2
J Kevin Wilkes, Charles D Fraser, Thomas J Seery
Atrioventricular septal defects represent a class of congenital cardiac malformations that vary in presentation and management strategy depending upon the severity of the particular lesions present. We present the case of a premature neonate who had a partial atrioventricular septal defect and an accessory mitral (or left atrioventricular) valve leaflet. The latter caused severe left ventricular outflow tract obstruction and severely depressed left ventricular function. We found only one other report of this atrioventricular valve abnormality in association with atrioventricular septal defect...
December 2016: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28100569/multiple-pregnancy-in-a-primigravida-with-uncorrected-pentalogy-of-fallot
#3
Pamela Partana, Jarrod Kah Hwee Tan, Ju Le Tan, Lay Kok Tan
Pentalogy of Fallot is a cyanotic congenital heart disease that has guarded prognosis without surgical intervention in infancy. Women with uncorrected defects rarely survive into childbearing age and pregnancy in this group is associated with a high rate of perinatal loss. Physiological cardiovascular changes in pregnancy can lead to maternal haemodynamic instability with subsequent adverse cardiac sequelae with or without fetal decompensation. Optimum management and pregnancy outcomes in mother with uncorrected Pentalogy of Fallot and twin pregnancy have not been described in the literature...
January 18, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28099512/age-dependent-oxidative-dna-damage-does-not-correlate-with-reduced-proliferation-of-cardiomyocytes-in-humans
#4
Yanhui Huang, Haifa Hong, Minghui Li, Jinfen Liu, Chuan Jiang, Haibo Zhang, Lincai Ye, Jinghao Zheng
BACKGROUND: Postnatal human cardiomyocyte proliferation declines rapidly with age, which has been suggested to be correlated with increases in oxidative DNA damage in mice and plays an important role in regulating cardiomyocyte proliferation. However, the relationship between oxidative DNA damage and age in humans is unclear. METHODS: Sixty right ventricular outflow myocardial tissue specimens were obtained from ventricular septal defect infant patients during routine congenital cardiac surgery...
2017: PloS One
https://www.readbyqxmd.com/read/28096041/advance-care-planning-in-adults-with-congenital-heart-disease-a-patient-priority
#5
Lisa X Deng, Lacey P Gleason, Abigail M Khan, David Drajpuch, Stephanie Fuller, Leah A Goldberg, Christopher E Mascio, Sara L Partington, Lynda Tobin, Yuli Y Kim, Adrienne H Kovacs
BACKGROUND: Adult congenital heart disease (ACHD) patients with moderate or great defect complexity are at risk for premature death. Although early engagement in advance care planning (ACP) is recommended, previous research suggests that it seldom occurs. METHODS: This study investigated ACHD patient preferences for ACP and factors that impact preferences. ACHD patients completed an ACP preferences questionnaire, the Hospital Anxiety and Depression Scale and a measure of attachment styles...
January 4, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28074828/three-dimensional-transesophageal-echocardiography-guided-transcathetar-closure-of-ruptured-noncoronary-sinus-of-valsalva-aneurysm
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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