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congenital heart block

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https://www.readbyqxmd.com/read/28928990/detection-of-a-left-superior-vena-cava-during-a-pacemaker-implantation-in-cotonou
#1
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
#2
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
#3
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.
September 9, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28882528/impaired-glycogen-breakdown-and-synthesis-in-phosphoglucomutase-1-deficiency
#4
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
#5
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
#6
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
#7
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...
September 4, 2017: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28875729/role-of-cardiac-pacing-in-congenital-complete-heart-block
#8
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...
September 6, 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
#9
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
#10
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]...
August 7, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28803248/a-functional-assay-for-sick-sinus-syndrome-genetic-variants
#11
Chuanchau J Jou, Cammon B Arrington, Spencer Barnett, Jiaxiang Shen, Scott Cho, Xiaoming Sheng, Patrick C McCullagh, Neil E Bowles, Chase M Pribble, Elizabeth V Saarel, Thomas A Pilcher, Susan P Etheridge, Martin Tristani-Firouzi
BACKGROUND/AIMS: Congenital Sick Sinus Syndrome (SSS) is a disorder associated with sudden cardiac death due to severe bradycardia and prolonged pauses. Mutations in HCN4, the gene encoding inward Na+/K+ current (If), have been described as a cause of congenital SSS. The objective of this study is to develop an SSS model in embryonic zebrafish, and use zebrafish as a moderate-throughput assay to functionally characterize HCN4 variants. METHODS: To determine the function of hcn4 in zebrafish, embryos were either bathed in the If -specific blocker (ZD-7288), or endogenous hcn4 expression was knocked down using splice-blocking morpholinos...
August 11, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28779859/double-right-coronary-artery-in-primary-pci-finding-the-hidden-treasure%C3%A2
#12
Luciano Candilio, Roberto Nerla, Jonathan Byrne
Double Right Coronary Artery (RCA) is one of the rarest congenital coronary anomalies. Although it can be regarded as a benign condition in the vast majority of cases, it has been associated with acute and life-threatening presentations, including inferior ST-segment elevation myocardial infarction (STEMI), cardiogenic shock and complete heart block, tachyarrhythmias, or even sudden death. However, the role of a double RCA in these conditions is yet to be clarified as more recent data prove no statistically significant association between prevalence of significant atherosclerosis in anomalous coronary arteries in comparison to normal coronary arteries...
July 5, 2017: Cardiovascular Revascularization Medicine: Including Molecular Interventions
https://www.readbyqxmd.com/read/28744930/what-endocardial-right-ventricular-pacing-site-shows-better-contractility-and-synchrony-in-children-and-adolescents
#13
Massimo Stefano Silvetti, Antonio Ammirati, Rosalinda Palmieri, Vincenzo Pazzano, Silvia Placidi, Lucilla Ravà, Romolo Remoli, Fabio Anselmo Saputo, Letizia Verticelli, Fabrizio Drago
AIMS: Right ventricular (RV) apical (RVA) pacing can induce left ventricular (LV) dyssynchrony, remodeling, and dysfunction in children with complete atrioventricular block (CAVB). We compared the functional outcome of RVA with RV alternative pacing sites (RVAPS), including para-Hisian, septal, and outflow tract sites. METHODS: This is a single-center, retrospective study. Data were collected before pacemaker implantation (transvenous leads), postoperatively, at 6 months, and at 1-2-3-4 years...
July 25, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28728260/-clinical-analysis-of-pacing-therapy-and-treatment-of-complications-during-follow-up-in-children
#14
P J Zhao, Y W Chen, F Li, Y Li, J P Yang, J J Wu
Objective: To analyze characteristics of permanent pacemaker implantation in children as well as treatment and prognosis of complications. Method: Clinical data of children who underwent endocardial or epicardial permanent pacemaker implantation between April 2000 and June 2016 in Shanghai Xinhua Hospital and Shanghai Children's Medical Center were analyzed retrospectively. These patients were discharged 5 days after implantation. Electrocardiogram, chest X ray, echocardiography and pacemaker programming were performed during follow-up...
July 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28709760/clinical-and-pathologic-implications-of-extending-the-spectrum-of-maternal-autoantibodies-reactive-with-ribonucleoproteins-associated-with-cutaneous-and-now-cardiac-neonatal-lupus-from-ssa-ro-and-ssb-la-to-u1rnp
#15
REVIEW
Peter M Izmirly, Marc K Halushka, Avi Z Rosenberg, Sean Whelton, Khodayar Rais-Bahrami, Dilip S Nath, Hilary Parton, Robert M Clancy, Sara Rasmussen, Amit Saxena, Jill P Buyon
While the relationship between maternal connective tissue diseases and neonatal rashes was described in the 1960s and congenital heart block in the 1970s, the "culprit" antibody reactivity to the SSA/Ro-SSB/La ribonucleoprotein complex was not identified until the 1980s. However, studies have shown that approximately 10-15% of cases of congenital heart block are not exposed to anti-SSA/Ro-SSB/La. Whether those cases represent a different disease entity or whether another antibody is associated has yet to be determined...
July 12, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28687669/atrioventricular-nodal-reentrant-tachycardia-in-patients-with-congenital-heart-disease-outcome-after-catheter-ablation
#16
MULTICENTER STUDY
John Papagiannis, Daniel Joseph Beissel, Ulrich Krause, Michel Cabrera, Marta Telishevska, Stephen Seslar, Christopher Johnsrude, Charles Anderson, Svjetlana Tisma-Dupanovic, Diana Connelly, Dimosthenis Avramidis, Christopher Carter, Laszlo Kornyei, Ian Law, Nicholas Von Bergen, Jan Janusek, Jennifer Silva, Eric Rosenthal, Mark Willcox, Peter Kubus, Gabriele Hessling, Thomas Paul
BACKGROUND: The relationship of atrioventricular nodal reentrant tachycardia to congenital heart disease (CHD) and the outcome of catheter ablation in this population have not been studied adequately. METHODS AND RESULTS: A multicenter retrospective study was performed on patients with CHD who had atrioventricular nodal reentrant tachycardia and were treated with catheter ablation. There were 109 patients (61 women), aged 22.1±13.4 years. The majority, 86 of 109 (79%), had CHD resulting in right heart pressure or volume overload...
July 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28666343/intraoperative-arrhythmias-in-children-with-congenital-heart-disease-transient-innocent-events
#17
Charlotte A Houck, Tanwier T T K Ramdjan, Ameeta Yaksh, Christophe P Teuwen, Eva A H Lanters, Ad J J C Bogers, Natasja M S de Groot
Aims: The significance and incidences of intraoperative arrhythmias occurring in the operating room (OR) in children with congenital heart disease (CHD) are unknown. Aims of this study were to determine incidences of intraoperative arrhythmias in children with CHD and to examine whether they are associated with persistent arrhythmias during follow-up. Methods and results: Continuous ECG recordings obtained from 134 consecutive paediatric CHD patients were manually examined from the moment the aortic cross-clamp (ACC) was removed [use of ACC and cardiopulmonary bypass (CPB)], when CPB was stopped (use of only CPB) or when the sternum was closed (no use of ACC and CPB) until departure from the OR...
June 28, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28649500/complete-congenital-heart-block-a-case-of-multilevel-block
#18
Ian W Hovis, David N Schidlow, Jeffrey P Moak
No abstract text is available yet for this article.
June 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28626076/cardiac-fibroblast-transcriptome-analyses-support-a-role-for-interferogenic-profibrotic-and-inflammatory-genes-in-anti-ssa-ro-associated-congenital-heart-block
#19
Robert M Clancy, Androo J Markham, Tanisha Jackson, Sara E Rasmussen, Miroslav Blumenberg, Jill P Buyon
The signature lesion of SSA/Ro autoantibody-associated congenital heart block (CHB) is fibrosis and a macrophage infiltrate, supporting an experimental focus on cues influencing the fibroblast component. The transcriptomes of human fetal cardiac fibroblasts were analyzed using two complementary approaches. Cardiac injury conditions were simulated in vitro by incubating human fetal cardiac fibroblasts with supernatants from macrophages transfected with the SSA/Ro-associated hY3. The top ten upregulated transcripts in the stimulated fibroblasts reflected a type I interferon (IFN) response (e...
June 16, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28598777/no-histologic-evidence-of-foetal-cardiotoxicity-following-exposure-to-maternal-hydroxychloroquine
#20
Deborah Friedman, Leif Lovig, Marc Halushka, Robert M Clancy, Peter M Izmirly, Jill P Buyon
It is currently recommended that hydroxychloroquine (HCQ) be maintained during pregnancy in patients with systemic lupus erythematosus. Recent data suggest that this Toll-like receptor inhibitor may also reduce the recurrence rate of anti-SSA/Ro associated congenital heart block (CHB). This case report describes a unique situation in which a CHB-afflicted, HCQ-exposed pregnancy was electively terminated. The heart did not reveal any characteristic features of cardiotoxicity, providing further evidence supporting the safety of foetal exposure to HCQ...
June 6, 2017: Clinical and Experimental Rheumatology
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