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https://www.readbyqxmd.com/read/27922246/neonatal-lupus-erythematosus-report-of-a-case-with-cutaneous-hematological-and-hepatobiliary-findings
#1
Andaç Salman, Merve Hatun Sarıçam, Ayşe Deniz Yücelten, Cuyan Demirkesen, Tülin Ergun
Neonatal lupus erythematosus is an autoimmune disorder mainly affecting the heart and skin. It is the most common cause of congenital heart block. In addition, hematological, hepatobiliary and neurological involvement may occur. Herein, we report a 23-day-old infant presented with annular, erythematous, and scaly and atrophic lesions on the face and trunk. Based on the clinical, laboratory and histopathological findings, she was diagnosed as neonatal lupus erythematosus. Neonatal lupus eryhtematosus should be considered in infants presenting with annular skin lesions, and we present this case to highlight the value of high index of clinical suspicion in diagnosis...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27913834/key-role-of-pacing-site-as-determinant-factor-of-exercise-testing-performance-in-pediatric-patients-with-chronic-ventricular-pacing
#2
Michel Cabrera Ortega, Hiram Tápanes Duamy, Dunia B Benítez Ramos
Chronic right ventricular (RV) apical pacing has been associated with deterioration of functional capacity and chronotropic incompetence during exercise testing in children. The effects of alternative pacing site on exercise performance in pediatric population remain unknown. We evaluated the influence of ventricular pacing site on exercise capacity in pediatric patients with complete congenital atrioventricular block requiring permanent pacemaker therapy. Sixty-four children paced from RV apex (n = 26), RV midseptum (n = 15) and left ventricular (LV) apex (n = 23) were studied cross-sectionally...
December 2, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27913750/congenital-heart-block-and-immune-mediated-sensorineural-hearing-loss-possible-cross-reactivity-of-immune-response
#3
C Bason, I Pagnini, A Brucato, S Maestroni, A Puccetti, C Lunardi, R Cimaz
Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27904846/complete-congenital-heart-block-in-a-neonate-with-a-complex-congenital-heart-defect-in-africa
#4
Clovis Nkoke, Edvine Yonta Wawo, Liliane Kuate Mfeukeu, Larissa Makamte, Sandrine Dikosso Edie, Flore Esiene Balana
Congenital heart block (CHB) is rare disorder that has a higher mortality when associated with structural congenital heart defects. Very few cases have been reported in Sub-Saharan Africa (SSA). We present a case of complete CHB associated with a complex congenital heart defect in a neonate in Cameroon. A 1-month-old neonate in Cameroon was referred for the evaluation of bradycardia. The obstetrical ultrasound done during pregnancy revealed fetal bradycardia without further evaluation. Clinical examination showed well a developed neonate with bradycardia at 62 beats/minute, and mild cyanosis with oxygen saturation at 93% at room air...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27899284/defective-lymphatic-valve-development-and-chylothorax-in-mice-with-a-lymphatic-specific-deletion-of-connexin43
#5
Stephanie J Munger, Michael J Davis, Alexander M Simon
Lymphatic valves (LVs) are cusped luminal structures that permit the movement of lymph in only one direction and are therefore critical for proper lymphatic vessel function. Congenital valve aplasia or agenesis can, in some cases, be a direct cause of lymphatic disease. Knowledge about the molecular mechanisms operating during the development and maintenance of LVs may thus aid in the establishment of novel therapeutic approaches to treat lymphatic disorders. In this study, we examined the role of Connexin43 (Cx43), a gap junction protein expressed in lymphatic endothelial cells (LECs), during valve development...
November 27, 2016: Developmental Biology
https://www.readbyqxmd.com/read/27891405/complete-heart-block-in-pregnancy-a-report-of-emergency-caesarean-section-in-a-parturient-without-pacemaker
#6
Vandana Mohapatra, Aparajita Panda, Satyanarayan Behera, Jagadish Chandra Behera
Management of women with Complete Heart Block (CHB) presenting without pacing, during pregnancy and labour is debatable. Temporary pacemakers have been routinely inserted for labour and birth probably to withstand any haemodynamic variations. However, due to lack of large scale prospective studies, the necessity of this procedure has not been objectively assessed. Also, the most appropriate anaesthetic technique for caesarean section in women with CHB is yet to be clarified. We report herein the case of a pregnant woman with CHB who had uneventful emergency caesarean delivery under spinal anaesthesia without temporary pacing...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27883221/alcohol-exposure-causes-overexpression-of-heart-development-related-genes-by-affecting-the-histone-h3-acetylation-via-bmp-signaling-pathway-in-cardiomyoblast-cells
#7
Jin Shi, Weian Zhao, Bo Pan, Min Zheng, Lina Si, Jing Zhu, Lingjuan Liu, Jie Tian
BACKGROUND: Abusive alcohol utilization of pregnant woman may cause congenital heart disease (CHD) of fetus, where alcohol ignites histone H3 hyperacetylation leading to abnormal development of heart morphogenesis and associated genes. Knowledge about the regularized upstream genes is little, but bone morphogenetic protein (BMP) signaling may actively and prominently take part in alteration in acetylation of histone H3. The supreme objective of this study was to unearth the involvement of BMP signaling pathway in alcohol-driven hyperacetylation of histone H3 in cardiomyoblast cells...
November 24, 2016: Alcoholism, Clinical and Experimental Research
https://www.readbyqxmd.com/read/27882249/surviving-all-odds-a-unique-case-of-multiple-congenital-unruptured-sinus-of-valsalva-aneurysms-involving-both-left-and-right-coronary-sinuses-with-biventricular-dysfunction-and-heart-block
#8
Aniketh Vijay B, Vikrant Vijan, Navin Mathew
Aneurysms of the sinus of Valsalva are very uncommon, with an incidence ranging from 0.1 to 3.5% of all congenital heart defects. Very few cases have been reported in the literature that presented with involvement of two or more sinuses. We report a case of 27-year-old male with a history of exertional breathlessness of one-month duration. After complete evaluation using transesophageal echocardiography (TEE) and multiple detector computed tomography (MDCT) scanning, the patient was diagnosed to have large congenital unruptured sinus of Valsalva aneurysms involving both left and right coronary sinuses with extension into the interventricular septum...
2016: Case Reports in Cardiology
https://www.readbyqxmd.com/read/27826129/genotypic-and-phenotypic-predictors-of-complete-heart-block-and-recovery-of-conduction-following-surgical-repair-of-congenital-heart-disease
#9
Laura Murray, Andrew H Smith, English C Flack, Kim Crum, Jill Owen, Prince J Kannankeril
BACKGROUND: Complete heart block (CHB) is a major complication after congenital heart surgery. We hypothesized that genetic and clinical factors are associated with the development of postoperative CHB and recovery of atrioventricular (AV) conduction. OBJECTIVE: To identify predictors of CHB and recovery following congenital heart surgery. METHODS: Patients undergoing congenital heart surgery at our institution from September 2007 through June 2015 were prospectively enrolled in a parent study of postoperative arrhythmias...
November 5, 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27803518/successful-preventive-treatment-of-congenital-heart-block-during-pregnancy-in-a-woman-with-systemic-lupus-erythematosus-with-anti-la-ro-antibody
#10
Vishwakarma Pratibha, Shankar Kundavi, Varma R Thangam, S Ramakrishnan
No abstract text is available yet for this article.
October 2016: Journal of Obstetrics and Gynaecology of India
https://www.readbyqxmd.com/read/27803192/interchromosomal-core-duplicons-drive-both-evolutionary-instability-and-disease-susceptibility-of-the-chromosome-8p23-1-region
#11
Kiana Mohajeri, Stuart Cantsilieris, John Huddleston, Bradley J Nelson, Bradley P Coe, Catarina D Campbell, Carl Baker, Lana Harshman, Katherine M Munson, Zev N Kronenberg, Milinn Kremitzki, Archana Raja, Claudia Rita Catacchio, Tina A Graves, Richard K Wilson, Mario Ventura, Evan E Eichler
Recurrent rearrangements of Chromosome 8p23.1 are associated with congenital heart defects and developmental delay. The complexity of this region has led to inconsistencies in the current reference assembly, confounding studies of genetic variation. Using comparative sequence-based approaches, we generated a high-quality 6.3-Mbp alternate reference assembly of an inverted Chromosome 8p23.1 haplotype. Comparison with nonhuman primates reveals a 746-kbp duplicative transposition and two separate inversion events that arose in the last million years of human evolution...
November 2016: Genome Research
https://www.readbyqxmd.com/read/27799231/electrophysiology-study-for-complex-supraventricular-tachycardia-in-congenital-heart-disease-patients-with-single-ventricle-physiology
#12
Shuenn-Nan Chiu, Jou-Kou Wang, Chun-Wei Lu, Kun-Lang Wu, Wei-Chieh Tseng, Mei-Hwan Wu
BACKGROUND: Supraventricular tachycardia (SVT) is common in complex congenital heart disease (CCHD) patients with single-ventricle physiology and may cause hemodynamic deterioration. We reported the outcomes of catheter ablation for such complex SVT in these single-ventricle CCHD patients. METHODS AND RESULTS: Patients with single-ventricle physiology (defined as CCHD patients) who received electrophysiology studies and catheter ablation between 1995 and 2015 were studied...
October 31, 2016: Journal of the American Heart Association
https://www.readbyqxmd.com/read/27777518/long-term-follow-up-study-of-temporary-tricuspid-valve-detachment-as-approach-to-vsd-repair-without-consequent-tricuspid-dysfunction
#13
Gianluca Lucchese, Lucia Rossetti, Giuseppe Faggian, Giovanni B Luciani
Temporary tricuspid valve detachment improves the operative view of certain congenital ventricular septal defects (VSDs), but its long-term effects on tricuspid valve function are still debated. From 2002 through 2012, we performed a prospective study of 68 children (mean age, 1.28 ± 1.01 yr) who underwent transatrial closure of VSDs following temporary tricuspid valve detachment. Sixty patients had conoventricular and 8 had mid-muscular VSDs. All were in sinus rhythm. Seventeen patients had systemic pulmonary artery pressures...
October 2016: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/27756424/isolated-congenital-complete-heart-block-in-a-five-year-old-seronegative-girl-born-to-a-woman-seropositive-for-human-immunodeficiency-virus-a-case-report
#14
Pedro Pallangyo, Isaac Mawenya, Paulina Nicholaus, Henry Mayala, Amida Kalombola, Godwin Sharau, Naiz Majani, Mohamed Janabi
BACKGROUND: Congenital complete heart block is a life-threatening condition which is highly associated with autoimmune and connective tissue disorders. Presence of maternal autoantibodies for associated conditions increases the risk of delivering a child with congenital complete heart block, however, less than a half of all women with such antibodies are symptomatic even after delivery. Mortality rate is highest during the neonatal period (45 %) and about two-thirds of all cases will require permanent pacing at some point in their lives...
October 19, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27733495/ranolazine-for-congenital-long-qt-syndrome-type-iii-experimental-and-long-term-clinical-data
#15
Ehud Chorin, Dan Hu, Charles Antzelevitch, Aviram Hochstadt, Luiz Belardinelli, David Zeltser, Hector Barajas-Martinez, Uri Rozovski, Raphael Rosso, Arnon Adler, Jesaia Benhorin, Sami Viskin
BACKGROUND: The basic defect in long-QT syndrome type III (LQT3) is an excessive inflow of sodium current during phase 3 of the action potential caused by mutations in the SCN5A gene. Most sodium channel blockers reduce the early (peak) and late components of the sodium current (INa and INaL), but ranolazine preferentially reduces INaL. We, therefore, evaluated the effects of ranolazine in LQT3 caused by the D1790G mutation in SCN5A. METHODS AND RESULTS: We performed an experimental study of ranolazine in TSA201 cells expressing the D1790G mutation...
October 2016: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/27728859/fluorinated-steroids-do-not-improve-outcome-of-isolated-atrioventricular-block
#16
N W E Van den Berg, M G Slieker, I M van Beynum, C M Bilardo, D de Bruijn, S A Clur, J M J Cornette, I M E Frohn-Mulder, M C Haak, K E H van Loo-Maurus, G T R Manten, A B M H Rackowitz, L A J Rammeloo, A Reimer, M E B Rijlaarsdam, M W Freund
INTRODUCTION: Congenital atrioventricular block (CAVB) is a rare disorder with a significant morbidity and mortality. Consensus regarding the prescription and efficacy of prenatal corticosteroids is lacking. This nationwide study was initiated to evaluate the effects of prenatal treatment with corticosteroids on the outcome of CAVB in The Netherlands. METHODS: All fetuses identified with isolated congenital AVB-II° or AVB-III° in any of the eight academic fetal heart centers of The Netherlands between 2003 and 2013 were included and reviewed...
September 30, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27722056/life-threatening-cardiac-episode-in-a-polish-patient-carrying-contiguous-gene-microdeletion-of-the-tbx5-and-the-tbx3-genes
#17
Katarzyna Iwanicka-Pronicka, Magdalena Socha, Maria Jędrzejowska, Małgorzata Krajewska-Walasek, Aleksander Jamsheer
Holt-Oram syndrome (HOS) features radial ray hypoplasia, heart defect and cardiac conduction impairment. Ulnar-mammary syndrome (UMS) characterizes congenital defects of the ulnar side of the upper limbs, underdevelopment of apocrine glands including hypoplasia and the dysfunction of mammary glands, hypogonadism and obesity. Inheritance of both conditions is autosomal dominant, mutations or deletions are found in the TBX5 and TBX3 gene, respectively. The Polish patient presented short stature, obesity, congenital malformation of the radial and ulnar side of the upper limbs, heart block, hypogonadism and dysmorphic features...
2016: SpringerPlus
https://www.readbyqxmd.com/read/27708487/the-use-of-continuous-electrocardiographic-holter-monitoring-in-pediatric-cardiology
#18
Zijo Begic, Edin Begic, Senka Mesihovic-Dinarevic, Izet Masic, Senad Pesto, Mirza Halimic, Almira Kadic, Amra Dobraca
OBJECTIVE: To show the place and role of continuous electrocardiographic twenty-four-hour ECG monitoring in daily clinical practice of pediatric cardiologists. METHODS: According to protocol, 2753 patients underwent dynamic continuous ECG Holter monitoring (data collected from the "Register of ECG Holter monitoring" of Pediatric Clinic, UCC Sarajevo in period April 2003- April 2015). RESULTS: There were 50,5% boys and 49,5% girls, aged from birth to 19 years (1,63% - neonates and infants, 2,6% - toddlers, 9,95% - preschool children, 35,5% - gradeschoolers and 50,3% children in puberty and adolescence)...
July 16, 2016: Acta Informatica Medica: AIM
https://www.readbyqxmd.com/read/27676165/transhepatic-permanent-pacing-in-a-child-with-complex-cyanotic-heart-disease-after-total-cavo-pulmonary-shunt-kawashima-repair
#19
Anilkumar Singhi, Ejaz Ahmed Sheriff, Kothandam Sivakumar
Complex cyanotic congenital heart diseases with left isomerism are sometimes associated with atrioventricular nodal conduction disturbances that may need permanent pacing. Surgical palliation in such anatomy connecting the superior vena cava to the pulmonary artery precludes a transvenous access for an endocardial pacing lead to the ventricles. Epicardial leads in these patients fail if the pacing thresholds are very high. We report transhepatic permanent ventricular lead implantation for a young boy with heterotaxy complicated by complete heart block...
March 2016: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/27665336/-congenitally-corrected-transposition-of-the-great-arteries-in-patient-with-atrial-septal-defect-and-acute-inferior-segment-myocardial-infarction
#20
Veysel Tosun, Necmettin Korucuk, Mustafa Mustafa Karakaş, Ünal Güntekin
Congenitally corrected transposition of the great arteries is a rare abnormality accounting for approximately 1% of clinically apparent congenital heart disease. Age at time of diagnosis and survival rate vary and depend on associated anomalies, including pulmonary stenosis, ventricular septal defect, atrial septal defect, atrioventricular block, and atrioventricular valve regurgitation. Reported cases of corrected transposition of the great arteries with single coronary ostium anomaly and atrial septal defect are very rare...
September 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
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