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ebstein syndrome

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https://www.readbyqxmd.com/read/27741366/the-diverse-outcome-of-early-prenatal-diagnosis-of-pulmonary-stenosis
#1
Moshe Bronshtein, Zeev Blumenfeld, Asaad Choury, Ayala Gover
OBJECTIVES: To assess the natural history and outcome of fetal pulmonary stenosis [PS] detected at 14 to 16 weeks gestation. METHODS: This is a retrospective study, in the years 2004-2015, with serial follow up during pregnancy. Patients referred for complete early fetal ultrasound including all fetal systems and a fetal echocardiogram. Ninety seven percent of the women were low risk, and 3% had risk factors such as maternal type 1 diabetes mellitus, exposure to teratogenic drugs or anomalies in previous pregnancies or in other family members...
October 14, 2016: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27704512/what-prognosis-for-ebstein-s-anomaly-without-surgery-hassan-ii-teaching-hospital-experience
#2
Moumouni Garba, Samir Atmani, Rabiou Sani, Kaled Adamou Nouhou
Background From the first description in 1886, significant progress was made on the treatment of Ebstein disease by mono and bi-ventricular surgery. Aim To highlight the prognosis of Ebstein's anomaly in the pediatric department of Hassan II hospital in Fez. Methods This is a descriptive and retrospective study of 4 years. Results We collected nine patients (seven boys and two girls) with a mean age of 5.3 years. Cyanosis was the main mode of revelation of the disease. In three patients Ebstein's anomaly was part of a malformative syndrome...
April 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/27646593/ep05-04-a-rare-case-of-ebstein-s-anomaly-and-down-s-syndrome-diagnosed-intra-utero
#3
C A Capuruço, C N Martins, R M Lopes, F A Fantini
No abstract text is available yet for this article.
September 2016: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27572669/the-changing-epidemiology-of-ebstein-s-anomaly-and-its-relationship-with-maternal-mental-health-conditions-a-european-registry-based-study
#4
Breidge Boyle, Ester Garne, Maria Loane, Marie-Claude Addor, Larraitz Arriola, Clara Cavero-Carbonell, Miriam Gatt, Nathalie Lelong, Catherine Lynch, Vera Nelen, Amanda J Neville, Mary O'Mahony, Anna Pierini, Anke Rissmann, David Tucker, Natalia Zymak-Zakutnia, Helen Dolk
OBJECTIVES: The aim of this study was to describe the epidemiology of Ebstein's anomaly in Europe and its association with maternal health and medication exposure during pregnancy. DESIGN: We carried out a descriptive epidemiological analysis of population-based data. SETTING: We included data from 15 European Surveillance of Congenital Anomalies Congenital Anomaly Registries in 12 European countries, with a population of 5.6 million births during 1982-2011...
August 30, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27558473/surgical-outcome-in-pediatric-patients-with-ebstein-s-anomaly-a-multicenter-long-term-study
#5
Lianne M Geerdink, Gideon J du Marchie Sarvaas, Irene M Kuipers, Willem A Helbing, Tammo Delhaas, Henriette Ter Heide, Lieke Rozendaal, Chris L de Korte, Sandeep K Singh, Tjark Ebels, Mark G Hazekamp, Felix Haas, Ad J J C Bogers, Livia Kapusta
OBJECTIVE: Surgical outcomes of pediatric patients with Ebstein's anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study. DESIGN: Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed...
August 25, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27439929/successful-ablation-of-coexistent-mahaim-tachycardia-and-right-posterior-accessory-pathway-in-a-patient-with-ebstein-s-anomaly
#6
Enes Elvin Gül, Celal Akdeniz, Volkan Tuzcu
The atriofascicular accessory pathway (AP), known as the Mahaim pathway, is a rare form of pre-excitation, comprising less than 3% of all APs. Mahaim AP is characterized by decremental, anterograde-only conduction, and antidromic tachycardia with left bundle branch morphology. Prevalence of Mahaim AP in Ebstein's anomaly is significantly high. In addition, combination of Wolff-Parkinson-White (WPW) syndrome and Mahaim AP in patients with Ebstein's anomaly has been reported. Presently described is the coexistence of Mahaim AP and manifest WPW syndrome in a patient with Ebstein's anomaly, who was successfully ablated without fluoroscopy...
July 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/27411422/systemic-onset-juvenile-idiopathic-arthritis-with-macrophage-activation-syndrome-and-coronary-artery-dilatation-misdiagnosed-as-kawasaki-disease
#7
Gonca Keskindemirci, Nuray Aktay Ayaz, Neslihan Melikoğlu, Helen Bornaun, Çiğdem Aydoğmuş, Esin Aldemir, Gönül Aydoğan
Systemic onset juvenile idiopathic arthritis (SoJIA) is characterized by arthritis, fever and visceral organ involvement including hepatosplenomegaly, lympadenopathy and serositis. This is a case of SoJIA misdiagnosed as Kawasaki disease (KD) and developed machrophage activation syndrome (MAS) secondary to Ebstein-Barr virus (EBV) infection. It is presented to point out the conditions that may come along. First of all, SoJIA should be kept in mind while making the differential diagnosis of coronary arterial ectasias and dilatations usually seen in vasculitic diseases like KD...
September 2015: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27363529/right-ventricular-outflow-tract-obstruction-in-complicated-monochorionic-twin-pregnancies
#8
S J Eschbach, L S T M Boons, E van Zwet, J M Middeldorp, F J C M Klumper, E Lopriore, A K K Teunissen, M E Rijlaarsdam, D Oepkes, A D J Ten Harkel, M C Haak
OBJECTIVE: Severe right Ventricular Outflow Tract Obstruction (RVOTO) is a complication in recipient twins in Twin-to-Twin-Transfusion Syndrome (TTTS), which requires post-partum follow-up or treatment. The objective of our study was to evaluate pregnancy characteristics of neonates with RVOTO in complicated monochorionic twin pregnancies. In the TTTS cases we aimed to determine the incidence and to construct a prediction model for the development of RVOTO. METHODS: An observational cohort study of all complicated monochorionic twin pregnancies with a post-partum neonatal diagnosis of RVOTO was performed...
July 1, 2016: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27339563/a-retrospective-evaluation-of-lymphadenopathy-in-children-in-a-single-center-s-experience
#9
Sevgi Buyukbese Sarsu, Kamil Sahin
OBJECTIVE: To determine the causes of lymphadenopathies in children living in our region, and detect the frequency of malignant disease. METHODS: Our study evaluated demographic characteristics, lymph node involvement sites, tests, and viral serologiesperformed to search for the presence of infection, and ultrasonographic, and histologic findings of 1700 children who were referred to the outpatient clinics of the Paediatric Diseases and Paediatric Surgery between January 2012, and January 2015...
June 2016: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/27238951/-ebstein-anomaly-and-congenital-long-qt-syndrome-report-of-a-case
#10
Daniel Hurtado-Sierra, Leonardo Rivera-Rodríguez
No abstract text is available yet for this article.
May 26, 2016: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/27225164/surgical-aspects-of-atrial-arrhythmia-right-atrial-ablation-and-anti-arrhythmic-surgery-in-congenital-heart-disease
#11
Hideki Uemura
BACKGROUND: Atrial arrhythmias are frequently described in congenital heart disease. OBJECTIVES: To provide a surgical perspective of anti-arrhythmic procedures and strategic approaches. METHODS: Discussion of the history of anti-arrhythmic treatments in congenital heart disease. RESULTS: Before the advent of the Maze procedure (first published in 1991), surgery mainly focused on patients with Wolff-Parkinson-White syndrome and also on arrhythmias in Ebstein's malformation...
June 2016: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/26841643/-altered-z-disks-of-myofibrils-in-the-cardiomyocytes-from-patients-with-ebstein-s-anomaly
#12
I F Egorova, E V Penyaeva, L A Bockeria
UNLABELLED: The aim of the study was to examine the peculiarities of the changes in Z-bands of myofibrils in the cardiomyocytes from patients with Ebstein's anomaly. MATERIAL AND METHODS: Electron microscopy assay of intraoperative biopsies of the right heart chambers in 41 patients aged from 9 months to 57 years was performed. RESULTS: Some patients exhibited Z-disk alterations of two types in individual cardiomyocytes, namely: local symmetrical bead-like expansions of Z-disks or longitudinal deposits of Z-material of different lengths along the myofibrils...
November 2015: Arkhiv Patologii
https://www.readbyqxmd.com/read/25854848/biventricular-badness-rare-images-of-ebstein-anomaly-of-the-tricuspid-valve-in-a-patient-with-hypoplastic-left-heart-syndrome
#13
David K Werho, Thor Thorsson, Sonal T Owens, Carlen Fifer
We report a rare case of hypoplastic left heart syndrome coexisting in a patient with Ebstein anomaly of the tricuspid valve, which has previously been described only in pathological studies. A fetal echocardiogram at 27-weeks gestation showed severe aortic stenosis with evolving hypoplastic left heart syndrome, significant endocardial fibroelastosis, a dysplastic tricuspid valve with moderate regurgitation, right atrial and ventricular dilation, and signs of fetal congestive heart failure. Due to inadequate left heart size, the patient was not a candidate for fetal intervention for critical aortic stenosis, and repeat studies showed progression of the lesion through the pregnancy...
August 2015: Pediatric Cardiology
https://www.readbyqxmd.com/read/25811010/vitamin-b12-and-vitamin-d-deficiencies-an-unusual-cause-of-fever-severe-hemolytic-anemia-and-thrombocytopenia
#14
Vikas A Mishra, Rishit Harbada, Akhilesh Sharma
The array of diagnostic workup for pyrexia of unknown origin (PUO) generally revolves in searching for infections, inflammatory/autoimmune, and endocrine etiologies. A differential diagnosis of fever, hemolytic anemia, and thrombocytopenia can have etiologies varying from infections like malaria, dengue, cytomegalovirus, Ebstein barr virus, Parvovirus, infective endocarditis, to autoimmune disorder (systemic lupus erythromatosis), vasculitis, hemolytic uremic syndrome, thrombotic thrombocytopenic purpura (TTP), autoimmune hemolytic anemia/Evan's syndrome, paroxysmal nocturnal hemoglobinuri (PNH), or drugs...
January 2015: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/25775725/prevalence-and-echocardiographic-characteristics-of-common-congenital-heart-disease-in-adult-patients-at-siriraj-hospital-10-year-study
#15
Nithima Ratanasit, Khemajira Karaketklang, Decho Jakrapanichakul, Maytinee Kittipovanonth, Kesaree Punlee, Wandee Rochanasiri, Suteera Phrudprisan
BACKGROUND: The number of adult patients with congenital heart disease (CHD) has increased rapidly and represents a major challenge in cardiovascular medicine. Data regarding the prevalence and characteristics of adult CHD in Thai population over the past decade are lacking. MATERIAL AND METHOD: Patients who underwent comprehensive transthoracic echocardiographic study at Siriraj Hospital during January 2003 to May 2013 with the diagnosis of unrepaired CHD and complete data were enrolled in the study Patients with uncertain diagnosis, prior cardiac intervention, bicuspid aortic valve, patent foramen ovale, mitral valve prolapse, isolated persistent left superior vena cava and congenital cardiomyopathies were excluded...
January 2015: Journal of the Medical Association of Thailand, Chotmaihet Thangphaet
https://www.readbyqxmd.com/read/25474186/echocardiographic-differential-diagnosis-of-the-cyanotic-newborn
#16
REVIEW
K H Deeg
Cyanosis in newborns can be caused by cyanotic heart defects, such as transposition of the great arteries, tetralogy of Fallot, pulmonary and tricuspid atresia, hypoplastic left heart syndrome, common arterial trunk, Ebstein's anomaly of the tricuspid valve, and total anomalous pulmonary venous return. The indicated cyanotic heart defects can be diagnosed or ruled out with three simple echocardiographic views: The parasternal long and short axis view and the apical or subcostal 4-chamber view. If these three views are normal, a cyanotic heart defect can be ruled out...
April 2015: Ultraschall in der Medizin
https://www.readbyqxmd.com/read/25440283/intraatrial-conduit-fontan-procedure-indications-operative-techniques-and-clinical-outcomes
#17
Zhong Qun Zhu, Hai Fa Hong, Hui Wen Chen, Hai Bo Zhang, Zhao Hui Lu, Jing Hao Zheng, Guo Cheng Shi, Jing Fen Liu
BACKGROUND: The intraatrial conduit (IAC) Fontan procedure is one of the Fontan modifications and is usually not considered the first choice. In this large series, we report our experience of the IAC Fontan procedure for the treatment of a functional single ventricle and review its indications, techniques, and clinical outcomes. METHODS: Between 2009 and 2013, 101 patients with a functional single ventricle underwent an IAC Fontan procedure. The median surgical age was 44 months and weight was 15 kg...
January 2015: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/25421365/multiple-manifested-accessory-atrioventricular-pathways-in-a-patient-without-obvious-structural-heart-disease
#18
Songwen Chen, Genqing Zhou, Shaowen Liu
No abstract text is available yet for this article.
April 2015: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/25332588/hepatitis-a-virus-infection-associated-hemophagocytic-lymphohistiocytosis-in-two-children
#19
Kirubakaran Navamani, Manivachagan Muthappa Natarajan, Arul Premanand Lionel, Sathish Kumar
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by high fever, maculopapular rash, neurological symptoms, abnormal liver functions and coagulopathy. Primary HLH is due to an underlying genetic abnormality. Secondary HLH are due to an underlying infection, autoimmune disease or malignancy. Secondary HLH due to viral infections are commonly due to the herpes group commonest of which is the Ebstein Barr virus (EBV). We describe two children with virus associated hemophagocytic lymphohistiocytosis (VAHLH) secondary to hepatitis A infection...
September 2014: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/25327996/fetal-and-neonatal-imaging-and-strategy-of-primary-neonatal-heart-transplantation-in-hypoplastic-left-heart-with-ebstein-s-anomaly
#20
James M Hammel, David A Danford, Robert L Spicer, Shelby Kutty
We present the anatomic constellation of mitral stenosis/aortic atresia variant of hypoplastic left heart syndrome, Ebstein's anomaly, and partial anomalous pulmonary venous return, an exceeding rare congenital heart defect. Prenatal echocardiography led to concern about the capacity of the right ventricle to increase cardiac output with lung expansion and pulmonary arterial runoff at birth, prompting the precaution of extracorporeal membrane oxygenator standby at delivery. Stage I palliation was not attempted, and control of pulmonary arterial blood flow was achieved with pulmonary artery banding, allowing sufficient ongoing hemodynamic stability...
March 2015: Echocardiography
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