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https://www.readbyqxmd.com/read/29110745/an-unusual-course-of-anti-ro-antibody-mediated-fetal-complete-heart-block
#1
Claudia Renaud, Edgar Jaeggi, Varsha Thakur
Fetal hydrops is a serious complication of immune-mediated congenital complete atrioventricular block. We present the case of a fetus with severe hydrops and profound bradycardia and an unusual favourable outcome. This case enhances the importance of considering the contribution of ventricular ectopic beats to the cardiac output when counselling and predicting outcome of complete heart block.
November 7, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/29045069/association-of-natural-killer-cell-ligand-polymorphism-hla-c-asn80lys-with-the-development-of-anti-ssa-ro-associated-congenital-heart-block
#2
Hannah C Ainsworth, Miranda C Marion, Tiziana Bertero, Antonio Brucato, Rolando Cimaz, Nathalie Costedoat-Chalumeau, Micaela Fredi, Patrick Gaffney, Jennifer Kelly, Kateri Levesque, Alice Maltret, Nathalie Morel, Veronique Ramoni, Amelia Ruffatti, Carl D Langefeld, Jill P Buyon, Robert M Clancy
OBJECTIVE: Fetal exposure to maternal anti-SSA/Ro antibodies is necessary but not sufficient for the development of autoimmune congenital heart block (CHB), suggesting that other factors, such as fetal genetic predisposition, are important. Given the previously described association between major histocompatibility complex alleles and CHB risk, we undertook the present study to test the hypothesis that a variant form of HLA-C Asn80Lys, which binds with high affinity to an inhibitory killer cell immunoglobulin-like receptor (KIR) and thus renders natural killer (NK) cells incapable of restricting inflammation, contributes to the development of CHB...
November 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29023775/prenatal-sonographic-monitoring-of-progressive-cardiac-damages-caused-by-anti-ro-antibodies-a-case-report
#3
Fuanglada Tongprasert, Rekwan Sittiwangkul, Thitikarn Lerthiranwong, Theera Tongsong
Maternal autoantibodies to Ro/SSA are often linked to congenital heart block and rarely associated with structural defects. We describe the case of a fetus with anti-Ro-mediated second-degree block at 19 weeks, which progressed to a complete block, fibroelastosis, atrioventricular valve insufficiency, and semilunar valve stenosis/insufficiency at 20, 22, 24, and 26 weeks, respectively, although the fetus received transplacental anti-arrhythmic drugs. The 2150-g fetus was vaginally delivered at 35 weeks. An external pacemaker was inserted immediately after birth and replaced with permanent pacemaker at the age of 3 months...
October 12, 2017: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/28989287/congenitally-corrected-transposition-of-great-vessels-with-infundibular-pulmonary-stenosis-with-ventricular-septal-defect-presenting-with-atypical-atrioventricular-nodal-reentrant-tachycardia-a-rare-association
#4
Ashwin Kodliwadmath
Congenitally corrected transposition of great vessels (CCTGV) is a rare congenital heart disease (CHD) accounting for <1% of CHDs. CCTGV with infundibular pulmonary stenosis (PS) with ventricular septal defect (VSD) is part of Fallot's physiology. It is known to be associated with bradyarrhythmias like atrioventricular (AV) blocks, and acquired complete AV block occurs at a rate of 2% per year. Patients can have left-sided accessory pathways, which may cause atrioventricular reentrant tachycardia (AVRT)...
2017: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/28983169/left-atrium-in-cardiac-resynchronization-therapy-active-participant-or-innocent-bystander
#5
Haitham A Badran, M A Abdelhamid, Mazen T Ibrahim, Ayman M Abdelmoteleb, John K Zarif
BACKGROUND: Cardiac resynchronization therapy (CRT) is an established treatment for patients with advanced heart failure that results in improvement of left ventricle (LV) systolic function and LV reverse remodeling. This may have a positive effect on the size and the function of the left atrium (LA). We assessed the LA function, dimensions, and volumes before and after CRT implantation. METHODS: A total of 37 patients with mean age of 55.3 ± 9.64 years including 11 (29...
October 2017: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/28976740/3d-printed-pericardium-hydrogels-to-promote-wound-healing-in-vascular-applications
#6
Laura G Bracaglia, Michael Messina, Shira Winston, Che-Ying Kuo, Max Lerman, John P Fisher
Vascular grafts that can support total replacement and maintenance by the body of the injured vessel would improve outcomes of major surgical reconstructions. Building scaffolds using components of the native vessel can encourage biological recognition by native cells as well as mimic mechanical characteristics of the native vessel. Evidence is emerging that incorporating predetermined building-blocks into a tissue engineering scaffold may oversimplify the environment and ignore critical structures and binding sites essential to development at the implant...
October 16, 2017: Biomacromolecules
https://www.readbyqxmd.com/read/28957562/factors-influencing-fetal-cardiac-conduction-in-anti-ro-ssa-positive-pregnancies
#7
Sven-Erik Sonesson, Malin Hedlund, Aurélie Ambrosi, Marie Wahren-Herlenius
Objectives: Congenital heart block (CHB) develops in 1-2% of anti-Ro/SSA-positive pregnancies and has a recurrence rate of 12-20%, which indicates that factors other than maternal autoantibodies are crucial for CHB to occur. Here, we aimed to evaluate the influence of factors previously associated with CHB on the occurrence of milder forms of fetal cardiac conduction disturbances, shown to occur in up to 30% of anti-Ro/SSA-positive pregnancies, and on neonatal outcome in a large cohort of prospectively followed pregnancies...
October 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28955500/environmental-and-lifestyle-factors-influencing-risk-of-congenital-heart-block-during-pregnancy-in-anti-ro-ssa-positive-women
#8
Sabrina Meisgen, Joanna Tingström, Amanda Skog Andreasson, Sven-Erik Sonesson, Ingrid Kockum, Marie Wahren-Herlenius
OBJECTIVES: Congenital heart block (CHB) occurs in 1%-2% of anti-Ro/SSA antibody-positive pregnancies. A population-based recurrence rate of 12% indicates that factors other than maternal autoantibodies influence CHB development. Here we report the first investigation to identify environmental and lifestyle factors influencing the risk of CHB. METHODS: A questionnaire focused on environmental and lifestyle factors was distributed to anti-Ro/SSA antibody-positive women who had given birth to at least one child with CHB, and additional data were retrieved from national health registers...
2017: RMD Open
https://www.readbyqxmd.com/read/28943497/leadless-micra-pacemaker-percutaneous-extraction-from-pulmonary-artery-in-complex-congenital-heart-disease-and-complete-heart-block-patient
#9
Maciej Sterliński, Marcin Demkow, Karolina Plaskota, Artur Oręziak
No abstract text is available yet for this article.
September 26, 2017: EuroIntervention
https://www.readbyqxmd.com/read/28940430/wide-complex-tachycardia-in-a-patient-with-congenital-heart-block-what-is-the-mechanism
#10
Nicholas Serafini, Parikshit S Sharma, Christopher Madias, Richard G Trohman
No abstract text is available yet for this article.
September 20, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28928990/detection-of-a-left-superior-vena-cava-during-a-pacemaker-implantation-in-cotonou
#11
A Sonou, M Hounkponou, L Codjo, P M Adjagba, C Houehanou, H Dohou, S Assani, Y Tchabi, M Houenassi
Persistent left superior vena cava (LSVC) is a rare congenital anomaly. Its prevalence in the general population is 0.1 to 0.5%. LSVC is 5 times rarer when accompanied by an absence of the right superior vena cava (RSVC). We present the case of a 54-year-old man who carries a persistent LSVC without RSVC. Clinically, this patient presented a regular bradycardia at 40 per minute associated with a heart failure syndrome. The electrocardiogram diagnosed a complete atrioventricular block and transthoracic echocardiography showed dilated left heart cavities and a left ventricular ejection fraction of 50%...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/28921728/undifferentiated-connective-tissue-diseases-and-adverse-pregnancy-outcomes-an-undervalued-association
#12
REVIEW
Arsenio Spinillo, Fausta Beneventi, Roberto Caporali, Veronique Ramoni, Carlomaurizio Montecucco
Undifferentiated connective tissue diseases (UCTDs) are a heterogeneous group of disorders characterized by symptoms and signs suggestive of systemic autoimmune rheumatic disease (ARD), but which do not fulfill all the established criteria for definite diagnosis of a condition. Although a third of UCTDs can progress to a definite ARD within months or years, most UCTDs can remain stable for years with minimal disease activity. The annual incidence of UCTD in the general population ranges from 14 to 140 per 100 000 people...
September 16, 2017: American Journal of Reproductive Immunology: AJRI
https://www.readbyqxmd.com/read/28899797/should-we-treat-congenital-heart-block-with-fluorinated-corticosteroids
#13
REVIEW
Antonio Brucato, Angela Tincani, Micaela Fredi, Silvia Breda, Veronique Ramoni, Nathalie Morel, Nathalie Costedoat-Chalumeau
No abstract text is available yet for this article.
November 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28882528/impaired-glycogen-breakdown-and-synthesis-in-phosphoglucomutase-1-deficiency
#14
Nicolai Preisler, Jonathan Cohen, Christoffer Rasmus Vissing, Karen Lindhardt Madsen, Katja Heinicke, Lydia Jane Sharp, Lauren Phillips, Nadine Romain, Sun Young Park, Marta Newby, Phil Wyrick, Pedro Mancias, Henrik Galbo, John Vissing, Ronald Gerald Haller
OBJECTIVE: We investigated metabolism and physiological responses to exercise in an 18-year-old woman with multiple congenital abnormalities and exertional muscle fatigue, tightness, and rhabdomyolysis. METHODS: We studied biochemistry in muscle and fibroblasts, performed mutation analysis, assessed physiological responses to forearm and cycle-ergometer exercise combined with stable-isotope techniques and indirect calorimetry, and evaluated the effect of IV glucose infusion and oral sucrose ingestion on the exercise response...
August 25, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28880700/anti-ro52-antibody-level-is-an-important-marker-of-fetal-congenital-heart-block-risk-in-anti-ro-ssa-antibody-positive-pregnancy
#15
Mai Miyasato-Isoda, Masako Waguri, Yuko Yamada, Akira Miyano, Yoshinao Wada
OBJECTIVE: The aims of this study are to determine the incidence of CHB in the Japanese population and to identify maternal factors predicting fetal congenital heart block (CHB) in anti-Ro/SSA antibody positive pregnancy. METHODS: A retrospective study was performed using 52,147 clinical records of pregnancies followed in a single center. For 183 anti-Ro/SSA antibody-positive women, anti-Ro52 and Ro60 antibodies were measured, and the odds of CHB in relation to maternal clinical features were calculated by multivariate analysis...
September 7, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28877886/pulmonary-right-ventricular-resynchronization-in-congenital-heart-disease-acute-improvement-in-right-ventricular-mechanics-and-contraction-efficiency
#16
Jan Janoušek, Jan Kovanda, Miroslav Ložek, Viktor Tomek, Pavel Vojtovič, Roman Gebauer, Peter Kubuš, Miroslav Krejčíř, Joost Lumens, Tammo Delhaas, Frits Prinzen
BACKGROUND: Electromechanical discoordination may contribute to long-term pulmonary right ventricular (RV) dysfunction in patients after surgery for congenital heart disease. We sought to evaluate changes in RV function after temporary RV cardiac resynchronization therapy. METHODS AND RESULTS: Twenty-five patients aged median 12.0 years after repair of tetralogy of Fallot and similar lesions were studied echocardiographically (n=23) and by cardiac catheterization (n=5) after primary repair (n=4) or after surgical RV revalvulation for significant pulmonary regurgitation (n=21)...
September 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28876373/minimally-invasive-epicardial-pacemaker-implantation-in-neonates-with-congenital-heart-block
#17
Roberto Costa, Katia Regina da Silva, Martino Martinelli Filho, Roger Carrillo
BACKGROUND: Few studies have characterized the surgical outcomes following epicardial pacemaker implantation in neonates with congenital complete atrioventricular block (CCAVB). OBJECTIVE: This study sought to assess the long-term outcomes of a minimally invasive epicardial approach using a subxiphoid access for pacemaker implantation in neonates. METHODS: Between July 2002 and February 2015, 16 consecutive neonates underwent epicardial pacemaker implantation due to CCAVB...
October 2017: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28875729/role-of-cardiac-pacing-in-congenital-complete-heart-block
#18
Stephanie F Chandler, Francis Fynn-Thompson, Douglas Y Mah
Congenital complete heart block affects 1/15,000 live-born infants, predominantly due to atrioventricular nodal injury from maternal antibodies of mothers with systemic lupus erythermatosus or Sjogren's syndrome. The majority of these children will need a pacemaker implanted prior to becoming young adults. This article will review the various patient and technical factors that influence the type of pacemaker implanted, and the current literature on optimal pacing practices. Areas covered: A literature search was performed using PubMed, Embase and Web of Science...
November 2017: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/28862385/tailoring-stents-to-fit-the-anatomy-of-unique-vascular-stenoses-in-congenital-heart-disease
#19
Patrick M Sullivan, Aimee Liou, Cheryl Takao, Henri Justino, Christopher J Petit, Jorge D Salazar, Frank F Ing
BACKGROUND: Unique and small anatomical features often preclude the use of available vascular stents in pediatric patients with congenital heart disease (CHD). OBJECTIVES: To report our experience and outcomes tailoring stents to fit unique anatomy, particularly in small children and infants with CHD. METHODS: Stent tailoring techniques included trimming, folding, and flaring. Patients receiving a tailored stent November 2002 to February 2015 were included in a retrospective analysis...
September 1, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28843719/incidence-risk-factors-and-mortality-of-neonatal-and-late-onset-dilated-cardiomyopathy-associated-with-cardiac-neonatal-lupus
#20
Nathalie Morel, Kateri Lévesque, Alice Maltret, Gabriel Baron, Mohamed Hamidou, Pauline Orquevaux, Jean-Charles Piette, François Barriere, Jérôme Le Bidois, Laurent Fermont, Olivier Fain, Arnaud Theulin, François Sassolas, Quentin Hauet, Gaëlle Guettrot-Imbert, Sophie Georgin-Lavialle, Christophe Deligny, Eric Hachulla, Luc Mouthon, Claire Le Jeunne, Philippe Ravaud, Delphine Le Mercier, Bénédicte Romefort, Elisabeth Villain, Damien Bonnet, Nathalie Costedoat-Chalumeau
BACKGROUND: Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear. METHODS: We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies. RESULTS: Among 187 neonates with CHB, 35 (18.8%, one missing data) had DCM and 22 (11.8%) died during a median follow-up of 7years [range: birth-36years]...
December 1, 2017: International Journal of Cardiology
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