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Congenital cardiopathy

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https://www.readbyqxmd.com/read/27366529/anaesthesia-management-in-a-patient-with-waardenburg-syndrome-and-review-of-the-literature
#1
Kevser Peker, Julide Ergil, İbrahim Öztürk
Waardenburg syndrome is a rare autosomal dominant disease that may cause hearing loss, pigmentary abnormalities, neurocristopathy and partial albinism. Incidence is estimated as 2%-3% among the cases of congenital deafness and 1/42,000 of the general population. Children with Waardenburg syndrome usually require anaesthesia for the cochlear implant operation in early age. The features of the syndrome that may bear importance for anaesthetic management are laryngomalacia, multiple muscle contractures, limited neck movements, cyanotic cardiopathy and electrolyte imbalance...
October 2015: Turkish Journal of Anaesthesiology and Reanimation
https://www.readbyqxmd.com/read/27296499/ischaemic-stroke-in-children-with-cardiopathy-an-epidemiological-study
#2
M Vázquez-López, P Castro-de Castro, E Barredo-Valderrama, M C Miranda-Herrero, N Gil-Villanueva, A J Alcaraz-Romero, A Jiménez-de Domingo, S I Pascual-Pascual
INTRODUCTION: Ischaemic stroke is rare during childhood. Congenital and acquired heart diseases are one of the most important risk factors for arterial ischaemic stroke (AIS) in children. PATIENTS AND METHODS: We conducted a retrospective study of all children with AIS and heart disease diagnosed between 2000 and 2014. RESULTS: We included 74 children with heart disease who were eligible for inclusion. 60% were boys with a mean stroke age of 11 months...
June 10, 2016: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/27239352/multiple-coronary-artery-microfistulas-in-a-girl-with-kleefstra-syndrome
#3
Euthymia Vargiami, Athina Ververi, Hamda Al-Mutawa, Georgia Gioula, Spyridon Gerou, Fotios Rouvalis, Marios Kambouris, Dimitrios I Zafeiriou
Kleefstra syndrome is characterized by hypotonia, developmental delay, dysmorphic features, congenital heart defects, and so forth. It is caused by 9q34.3 microdeletions or EHMT1 mutations. Herein a 20-month-old girl with Kleefstra syndrome, due to a de novo subterminal deletion, is described. She exhibits a rare and complex cardiopathy, encompassing multiple coronary artery microfistulas, VSD/ASD, and PFO.
2016: Case Reports in Genetics
https://www.readbyqxmd.com/read/26885487/spectrum-of-heart-diseases-in-children-an-echocardiographic-study-of-1-666-subjects-in-a-pediatric-hospital-yaounde-cameroon
#4
David Chelo, Félicitée Nguefack, Alain P Menanga, Suzanne Ngo Um, Jean C Gody, Sandra A Tatah, Paul O Koki Ndombo
BACKGROUND: Children's health programs in Sub-Saharan Africa have always been oriented primarily to infectious diseases and malnutrition. We are witnessing in the early 21(st) century an epidemiological transition marked by the decline of old diseases and the identification of new diseases including heart disease. Therefore, it is necessary to describe the spectrum of these diseases in order to better prepare health workers to these new challenges. METHODS: We conducted a cross-sectional study focused on heart disease diagnosed by echocardiography in children seen from January 2006 to December 2014 in a pediatric hospital of Yaounde...
February 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/26559982/palliative-senning-in-the-treatment-of-congenital-heart-disease-with-severe-pulmonary-hypertension
#5
Juliano Gomes da Penha, Leina Zorzanelli, Antonio Augusto Barbosa-Lopes, Edimar Atik, Leonardo Augusto Miana, Carla Tanamati, Luiz Fernando Caneo, Nana Miura, Vera Demarchi Aiello, Marcelo Biscegli Jatene
BACKGROUND: Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level. OBJECTIVE: Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double outlet right ventricle with severe pulmonary vascular disease, and to evaluate the early and late clinical progression of the palliative Senning procedure...
October 2015: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/26476482/-assessment-of-exercise-capacity-in-congenital-heart-disease
#6
Raquel Bouzo-López, Alicia González-Represas
For many years, the treatment of congenital heart diseases has been a field in which, based on the seriousness of these conditions, treatment options were viewed with the greatest deference. This has conditioned, in many cases, the interventions to be undertaken in each. In this sense, exercise was thought to have a negative impact and thus the practise of almost any physical activity was limited. Although there has recently been a change in the paradigm with respect to exercise, this idea continues to hold sway...
January 2016: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/26291842/palliative-senning-in-the-treatment-of-congenital-heart-disease-with-severe-pulmonary-hypertension
#7
Juliano Gomes da Penha, Leina Zorzanelli, Antonio Augusto Barbosa-Lopes, Edimar Atik, Leonardo Augusto Miana, Carla Tanamati, Luiz Fernando Caneo, Nana Miura, Vera Demarchi Aiello, Marcelo Biscegli Jatene
BACKGROUND: Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level. OBJECTIVE: Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double outlet right ventricle with severe pulmonary vascular disease, and to evaluate the early and late clinical progression of the palliative Senning procedure...
August 18, 2015: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/26263419/8p23-1-interstitial-deletion-in-a-patient-with-congenital-cardiopathy-neurobehavioral-disorders-and-minor-signs-suggesting-22q11-2-deletion-syndrome
#8
Miriam C Molck, Fabíola P Monteiro, Milena Simioni, Vera L Gil-da-Silva-Lopes
Copy number variation studies of known disorders have the potential to improve the characterization of clinical phenotypes and may help identifying candidate genes and their pathways. The authors described a child with congenital heart disease, microcephaly, facial dysmorphisms, developmental delay, learning difficulties, and behavioral problems. There was initially a clinical suspicion of 22q11.2 deletion syndrome (22q11.2 DS), but molecular cytogenetic analysis (array genomic hybridization [aGH]) showed the presence of a de novo 3...
September 2015: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/25650280/-pulmonary-hypertension-associated-with-congenital-heart-disease-and-eisenmenger-syndrome
#9
REVIEW
Juan Calderón-Colmenero, Julio Sandoval Zárate, Miguel Beltrán Gámez
Pulmonary arterial hypertension is a common complication of congenital heart disease (CHD). Congenital cardiopathies are the most frequent congenital malformations. The prevalence in our country remains unknown, based on birthrate, it is calculated that 12,000 to 16,000 infants in our country have some cardiac malformation. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodeling and endothelial dysfunction secondary to an imbalance in vasoactive mediators which promotes vasoconstriction, inflammation, thrombosis, cell proliferation, impaired apotosis and fibrosis...
January 2015: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/25548340/mixed-type-total-anomalous-pulmonary-venous-connection-early-results-and-surgical-techniques
#10
Gláucio Furlanetto, Beatriz H S Furlanetto, Sandra R C Henriques, Lilian M Lopes, Eduardo T Miranda, Carolina M G Porto, Maria Emilia N T Pereira, Claudia Davini, Elssi C Espinosa
INTRODUCTION: The mixed total anomalous pulmonary connection is a rare type of congenital cardiopathy. The occurrence of multiple connections of the pulmonary veins has important implication in the diagnosis and surgical planning. These types of total anomalous pulmonary venous connection (TAPVC) and the infracardiac have higher surgical mortality. METHODS: Between December 1994 and July 2013, a total of 58 children underwent surgical treatment of TAPVC. Twenty-five (43...
January 2015: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/25524197/a-rare-case-of-myocardial-infarction-by-myocardial-bridging-of-circumflex-artery
#11
F Massoni, D Di Sabatino, L Ricci, E Onofri, S Ricci
Myocardial Bridging (MB) is a benign condition that may be considered as a possible cause of ischaemic cardiopathy up to myocardial infarction. The authors describe the autopsy case of an MB of circumflex artery in a person with a myocardial infarction of the posterior wall of the left ventricle. In this case, the chronic ischemic heart disease can be explained by the congenital anomaly and in the literature there is scientific evidence to support the thesis. The MB is a benign condition that in combination with other risk factors can cause ischemic heart disease...
2014: La Clinica Terapeutica
https://www.readbyqxmd.com/read/25388582/-cilia-and-heart-morphogenesis
#12
REVIEW
Nicolas Diguet, Sigolène M Meilhac
After the seminal discovery in 2000 that primary cilia are functional organelles which are essential for embryonic development, several mouse models of ciliopathies have been generated. The heart is frequently affected, with a large spectrum of malformations. The cilia of the node are required early in development in the determination of the left/right laterality of the embryo, which has secondary consequences on the formation of the heart. Thus, abnormal looping of the heart is a recurrent phenotype in models of ciliopathies...
November 2014: Médecine Sciences: M/S
https://www.readbyqxmd.com/read/25192527/-educational-program-for-congenital-cardiopathy-children-s-parents
#13
Paola Pino Armijo, Carmen Gloria Valdés Valenzuela, Paula Fajuri Moyano, Olga Garrido Villablanca, Andrés Castillo Moya
The children with congenital heart disease are faced with a series of procedures in chronological sequence. Throughout this process the infant and their parents require timely information, education and preparation for discharge. However, the information about each of the aspects affected by the disease is usually addressed in isolation by different professionals and not as part of a comprehensive educational program. The educational program should consider the nature of the disease, of the users and educators during their planning and must be implemented by a multidisciplinary team, continuously during hospitalization and follow-up, using various teaching methods available, and incorporating the minimum content described in the literature...
October 2014: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/25132776/chronic-permanent-hypoxemia-predisposes-to-mild-elevation-of-liver-stiffness
#14
Mohamed Tahiri, Abdenasser Drighil, Yasmine Jalal, Dounia Ghellab, Wafaa Hliwa, Haddad Fouad, Wafaa Badre, Ahmad Bellabah, Rachida Habbal, Rhimou Alaoui
AIM: To evaluate the impact of long term permanent hypoxemia noticed in patients with non operated congenital cyanogenic cyanotic cardiopathy on liver stiffness. METHODS: We included ten adult patients with non operated inoperate cyanotic cardiopathy and ten matched patients for age and gender admitted to the gastroenterology department for proctologic diseases; Clinical and laboratory data were collected [age, gender, body mass index, oxygen saturation, glutamate oxaloacetate transaminase (GOT), glutamate pyruvate transaminase (GPT), glycemia and cholesterol]...
August 14, 2014: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/24998101/simultaneous-transcatheter-treatment-of-perimembranous-ventricular-septal-defect-and-other-congenital-cardiopathies
#15
Xu-Dong Xu, Yuan Bai, Xiao-Li Chen, Su-Xuan Liu, Xian-Xian Zhao, Yong-Wen Qin
OBJECTIVE: To assess the efficacy and safety of simultaneous transcatheter corrections of perimembranous ventricular septal defect (VSD) and other congenital cardiopathies. PATIENTS/METHODS: From 2004 to 2012, 56 patients (25 male, 31 female), aged 14.2±10.1, with compound congenital cardiovascular abnormalities underwent simultaneous transcatheter interventional procedure. Of the 56 patients, 32 had VSD and atrial septal defects (ASD); 17 had VSD and patent ductus arteriosus (PDA); and seven had VSD and pulmonary valve stenosis (PS)...
December 2014: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/24936633/-fause-attie-on-his-transcendent-performance-in-studying-and-managing-congenital-cardiopathies-in-mexico
#16
Alfonso Buendia
No abstract text is available yet for this article.
January 2010: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/24612475/intra-hospital-mortality-among-neonates-transported-by-ambulance-in-colombia
#17
Jorge Alvarado-Socarras, Anderson Bermon, Nancy Bernal, Néstor F Naranjo-Estupiñán, Alvaro J Idrovo
BACKGROUND: The aims of this study were to identify the main variables associated with intra-hospital mortality among patients transferred to a specialized neonatal care center, and to evaluate agreement and accuracy of referring and admission diagnoses. METHODS: A 6 month observational study was conducted to obtain clinical variables for intra-hospital mortality among patients requiring interfacility transport. Association among variables was estimated using Poisson regression with robust variance...
August 2014: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/24517132/red-blood-cell-transfusion-in-critically-ill-children-cme
#18
COMPARATIVE STUDY
Pierre Demaret, Marisa Tucci, Thierry Ducruet, Helen Trottier, Jacques Lacroix
BACKGROUND: Red blood cell (RBC) transfusions are common in the pediatric intensive care unit (PICU). However, there are no recent data on transfusion practices in the PICU. Our objective was to determine transfusion practice in the PICU, to compare this practice with that observed 10 years earlier, and to estimate the compliance to the recommendation of a large randomized clinical trial, the Transfusion Requirements in Pediatric Intensive Care Unit (TRIPICU) study. STUDY DESIGN AND METHODS: This was a single-center prospective observational study over a 1-year period...
February 2014: Transfusion
https://www.readbyqxmd.com/read/24344641/simultaneous-atelectasis-in-human-bocavirus-infected-monozygotic-twins-was-it-plastic-bronchitis
#19
Christoph M Rüegger, Walter Bär, Peter Iseli
BACKGROUND: Plastic bronchitis is an extremely rare disease characterized by the formation of tracheobronchial airway casts, which are composed of a fibrinous exudate with rubber-like consistency and cause respiratory distress as a result of severe airflow obstruction. Bronchial casts may be associated with congenital and acquired cardiopathies, bronchopulmonary diseases leading to mucus hypersecretion, and pulmonary lymphatic abnormalities. In recent years, however, there is growing evidence that plastic bronchitis can also be triggered by common respiratory tract infections and thereby cause atelectasis even in otherwise healthy children...
2013: BMC Pediatrics
https://www.readbyqxmd.com/read/24309873/clinical-and-neuropsychiatric-status-in-children-with-williams-beuren-syndrome-in-upper-egypt
#20
Khaled Saad, Ahmed A Abdelrahman, Alameldin M Abdallah, Hisham A K Othman, Reda Badry
UNLABELLED: The aim of this study was to evaluate and explore the clinical, neuropsychiatric status and EEG pattern in a series of children with Williams-Beuren syndrome (WBS) in Assiut, Upper Egypt. We aimed to provide a comprehensive data comparable to what has been published, to enable us to make comparisons across different cultural areas. This will contribute to a better definition of the neuropsychiatric features that may be specific to WBS that allows early and better detection and management of those children...
December 2013: Asian Journal of Psychiatry
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