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congenital heart block

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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
#1
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
#2
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
https://www.readbyqxmd.com/read/28585599/discordant-disease-expression-of-neonatal-lupus-erythematosus-in-twins
#3
Faiza Mustafa, Yawar Najam, Mohammad Tauqeer Akbar, Tahir Aziz Ahmed
Neonatal lupus erythematosus is an autoimmune disease resulting from the trans-placental passage of maternal anti-SSA/Ro, anti-SSB/La, and less frequently anti-RNP antibodies to the foetus. At the time of diagnosis 50% of mothers are asymptomatic. Neonatal manifestations of this multisystem disease may include congenital heart block, cutaneous lesions and haematological abnormalities. We present the case of congenital neonatal lupus erythematosus in non-identical twins, showing variability in clinical manifestation of this disease,despite receiving the same level of antibodies from the mother...
June 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28555332/protective-role-of-downregulated-mlk3-in-myocardial-adaptation-to-chronic-hypoxia
#4
Siyi He, Shunbi Liu, Xiaochen Wu, Mei Xin, Sheng Ding, Dong Xin, Hui Ouyang, Jinbao Zhang
A series of protective responses could be evoked to achieve compensatory adaptation once cardiomyocytes are subjected to chronic hypoxia. MLK3/JNK/c-jun signaling pathway was previously demonstrated to be involved in this process. In the present study, we aim to further examine the performance of MLK3 in hypoxic H9C2 cells and potential mechanism. Myocardial samples of patients with congenital heart disease (CHD) were collected. H9C2 cells were cultured in hypoxic conditions for various durations. MLK3 was silenced by transfection of shRNA to evaluate its role in cell viability...
May 30, 2017: Journal of Physiology and Biochemistry
https://www.readbyqxmd.com/read/28532196/fetal-and-neonatal-involvement-in-maternal-rheumatologic-disease
#5
Shigeo Iijima
A pregnancy complicated with rheumatologic diseases can have various influences on the fetus and/or neonate. Maternal systemic lupus erythematosus (SLE) may cause preterm and/or small for gestational age (SGA) delivery and neonatal lupus (NL). Some neonates with NL have congenital heart block (CHB) with increased morbidity and mortality, even requiring pacemakers. Antiphospholipid syndrome may occur with SLE and affect fetal and/or neonatal outcomes. Pregnancy involving primary Sjögren's syndrome (pSS) tends to result in preterm delivery and low birthweight infants...
June 8, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28528723/ventricular-pacing-in-single-ventricles-a-bad-combination
#6
Anica Bulic, Frank J Zimmerman, Scott R Ceresnak, Ira Shetty, Kara S Motonaga, Anne Freter, Anthony V Trela, Deb Hanisch, Lisa Russo, Kishor Avasarala, Anne M Dubin
BACKGROUND: Chronic ventricular pacing (VP) is associated with systolic dysfunction in a subset of pediatric patients with heart block and structurally normal hearts. The effect of chronic VP in congenital heart disease is less well understood, specifically in the single-ventricle (SV) population. OBJECTIVE: To determine the longitudinal effect of VP in SV patients. METHODS: SV patients with heart block and dual-chamber pacemakers requiring >50% VP were compared with nonpaced (controls) SV patients matched for age, sex, and SV morphology...
June 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28512719/hypoplastic-left-heart-syndrome-with-congenital-complete-heart-block
#7
Maytham Al-Kubaisi, Safwat A Aly, Bassel Mohammad Nijres, Sawsan Awad
Congenital complete atrioventricular block (CCAVB) is a rare condition with an incidence of 1 of 20,000 live births. Hypoplastic left heart syndrome (HLHS) occurs more frequently than CCAVB and occurs in 1 of 5000 live births. HLHS in association with CCAVB is exceedingly rare. In this report, we describe a rare case of HLHS and CCAVB diagnosed in utero. Postnatal diagnosis, management and outcome are presented as well as review of the medical literature.
May 17, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28501799/high-maternal-expression-of-siglec1-on-monocytes-as-a-surrogate-marker-of-a-type-i-interferon-signature-is-a-risk-factor-for-the-development-of-autoimmune-congenital-heart-block
#8
Anna R Lisney, Franziska Szelinski, Karin Reiter, Gerd R Burmester, Thomas Rose, Thomas Dörner
OBJECTIVES: Autoimmune congenital heart block (CHB) is associated with placental transcytosis of maternal autoantibodies directed against Ro/SS-A and La/SS-B. However, only about 2% of children born to mothers with the respective antibodies are affected, indicating that further risk factors exist, which are not yet fully understood. In this study, we investigated whether a maternal type I interferon (IFN) signature represents a risk factor for the development of CHB. METHODS: Blood samples, clinical data and serological parameters from 9 women with CHB pregnancies, 14 pregnant women with antibodies against Ro/SS-A but without a CHB complication and another 30 healthy pregnant women as controls were studied...
May 13, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28500812/the-prevalence-of-chagas-disease-among-latin-american-immigrants-with-pacemakers-in-los-angeles-california
#9
Sandy Park, Daniel R Sanchez, Mahmoud I Traina, Jason S Bradfield, Salvador Hernandez, Alvaro Joaquin Altamirano Ufion, Jalal Dufani, Patrick Bergin, Robin Y Wachsner, Sheba K Meymandi
AbstractChagas disease (CD), with associated conduction abnormalities, is a common indication for pacemaker implantation in Latin America. The prevalence of CD in Latin American immigrants with pacemakers residing in the United States has never been studied. This single-center cross-sectional study included pacemaker patients who were aged 18 years or more with a previous residence in Latin America for at least 6 months. Patients with an implantable cardioverter-defibrillator, cardiac resynchronization therapy, or iatrogenic and/or congenital heart block were excluded...
May 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/28495809/comparison-of-enteral-versus-intravenous-potassium-supplementation-in-hypokalaemia-in-paediatric-patients-in-intensive-care-post-cardiac-surgery-open-label-randomised-equivalence-trial-eips
#10
Naveed Ur Rehman Siddiqu, Quratulain Merchant, Babar S Hasan, Arjumand Rizvi, Muneer Amanullah, Amina Rehmat, Anwarul Ul Haq
OBJECTIVES: The primary objective was to compare the efficacy of enteral potassium replacement (EPR) and intravenous potassium replacement (IVPR) as first-line therapy. Secondary objectives included comparison of adverse effects and number of doses required to resolve the episode of hypokalaemia. TRIAL DESIGN: The EIPS trial is designed as a randomised, equivalence trial between two treatment arms. STUDY SETTING: The study was conducted at the paediatric cardiac intensive care unit (PCICU) at Aga Khan University Hospital, Karachi...
May 10, 2017: BMJ Open
https://www.readbyqxmd.com/read/28476422/long-term-results-of-anatomic-correction-for-congenitally-corrected-transposition-of-the-great-arteries-a-19-year-experience
#11
Christian P Brizard, Alice Lee, Diana Zannino, Andrew M Davis, Tyson A Fricke, Yves d'Udekem, Igor E Konstantinov, Johann Brink, Michael M H Cheung
OBJECTIVE: The surgical indication, timing, strategy, and surgical technique for anatomic correction of congenitally corrected transposition of the great arteries are challenging. We evaluated the long-term results at The Royal Children's Hospital Melbourne. METHODS: Review of 32 successive anatomic corrections between 1996 and 2015. RESULTS: Twenty-one double-switch (66%), 6 Senning/Bex-Nikaidoh (19%), and 5 Senning/Rastelli (16%) procedures were performed (median age, 1...
July 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28469691/cardiac-resynchronization-therapy-device-implantation-in-a-patient-with-congenitally-corrected-transposition-of-great-vessels
#12
Reza Mollazadeh, Masoud Eslami
A 29-year-old woman was referred to our hospital due to exacerbation in dyspnea on exertion and easy fatigability. A known case of congenitally corrected transposition of the great vessels and congenital complete heart block, she had already received a permanent single-chamber pacemaker. Decision was made to implant a biventricular pacemaker for the treatment of the failing heart. Excellent coronary sinus lead implantation was done, conferring amelioration of symptoms, QRS narrowing in the electrocardiogram, and improvement of systemic ventricular systolic function in echocardiography...
January 2017: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/28460773/the-development-of-pacing-induced-ventricular-dysfunction-is-influenced-by-the-underlying-structural-heart-defect-in-children-with-congenital-heart-disease
#13
Seshadri Balaji, Narayanswami Sreeram
BACKGROUND: Right ventricular pacing can cause pacing-induced ventricular dysfunction (PIVD) correctable with biventricular pacing (BiVP). Factors associated with PIVD are poorly understood. METHODS: We reviewed children receiving epicardial dual-chamber pacemakers for complete heart block (CHB) after congenital heart disease (CHD) surgery. PIVD was defined as% fractional shortening <15% improving after BiVP. RESULTS: Between 2005 and 2014, 47 children <2years developed CHB after CHD surgery...
March 2017: Indian Heart Journal
https://www.readbyqxmd.com/read/28439482/association-of-non-alcoholic-fatty-liver-disease-with-conduction-defects-on-electrocardiogram
#14
Muhammad A Mangi, Abdul M Minhas, Hiba Rehman, Furquan Pathan, Hong Liang, Sary Beidas
BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) is a leading cause of liver disease in developed countries. The association of NAFLD with conduction defects is unknown. The aim of our study was to find whether an association exists between conduction defects and NAFLD. METHODS: This is a case-control retrospective study of 700 patients admitted to Orange Park Medical Center, Orange Park, Florida from 2009 to 2015. Patients with a history of alcohol use, congenital heart disease, infiltrative malignancy, and myocarditis were excluded from the study...
March 21, 2017: Curēus
https://www.readbyqxmd.com/read/28436940/disruption-of-spatiotemporal-hypoxic-signaling-causes-congenital-heart-disease-in-mice
#15
Xuejun Yuan, Hui Qi, Xiang Li, Fan Wu, Jian Fang, Eva Bober, Gergana Dobreva, Yonggang Zhou, Thomas Braun
Congenital heart disease (CHD) represents the most prevalent inborn anomaly. Only a minority of CHD cases are attributed to genetic causes, suggesting a major role of environmental factors. Nonphysiological hypoxia during early pregnancy induces CHD, but the underlying reasons are unknown. Here, we have demonstrated that cells in the mouse heart tube are hypoxic, while cardiac progenitor cells (CPCs) expressing islet 1 (ISL1) in the secondary heart field (SHF) are normoxic. In ISL1+ CPCs, induction of hypoxic responses caused CHD by repressing Isl1 and activating NK2 homeobox 5 (Nkx2...
June 1, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28431249/macrophages-facilitate-electrical-conduction-in-the-heart
#16
Maarten Hulsmans, Sebastian Clauss, Ling Xiao, Aaron D Aguirre, Kevin R King, Alan Hanley, William J Hucker, Eike M Wülfers, Gunnar Seemann, Gabriel Courties, Yoshiko Iwamoto, Yuan Sun, Andrej J Savol, Hendrik B Sager, Kory J Lavine, Gregory A Fishbein, Diane E Capen, Nicolas Da Silva, Lucile Miquerol, Hiroko Wakimoto, Christine E Seidman, Jonathan G Seidman, Ruslan I Sadreyev, Kamila Naxerova, Richard N Mitchell, Dennis Brown, Peter Libby, Ralph Weissleder, Filip K Swirski, Peter Kohl, Claudio Vinegoni, David J Milan, Patrick T Ellinor, Matthias Nahrendorf
Organ-specific functions of tissue-resident macrophages in the steady-state heart are unknown. Here, we show that cardiac macrophages facilitate electrical conduction through the distal atrioventricular node, where conducting cells densely intersperse with elongated macrophages expressing connexin 43. When coupled to spontaneously beating cardiomyocytes via connexin-43-containing gap junctions, cardiac macrophages have a negative resting membrane potential and depolarize in synchrony with cardiomyocytes. Conversely, macrophages render the resting membrane potential of cardiomyocytes more positive and, according to computational modeling, accelerate their repolarization...
April 20, 2017: Cell
https://www.readbyqxmd.com/read/28431056/long-term-outcome-of-transvenous-pacemaker-implantation-in-infants-a-retrospective-cohort-study
#17
Laura M Vos, Janneke A E Kammeraad, Matthias W Freund, Andreas C Blank, Johannes M P J Breur
Aim: Evaluation of long-term outcome of transvenous pacemaker (PM) implantation in infants. Methods and Results: A retrospective analysis of all transvenous PM implantations in infants <10 kg between September 1997 and October 2001 was made. Indications for PM implantation, age at implantation, and determinants of long-term outcome including cardiac function, PM function, and PM (system) complications were noted. Seven patients underwent transvenous VVI(R) PM implantation...
April 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28429903/causes-of-interruptions-in-postoperative-enteral-nutrition-in-children-with-congenital-heart-disease
#18
Jirong Qi, Zhuo Li, Yueshuang Cun, Xiaonan Li
BACKGROUND AND OBJECTIVES: Perioperative nutritional support has become a hot topic in the clinical management of congenital heart disease (CHD). Postoperative enteral nutrition (EN) offers many benefits, such as protection of the intestinal mucosa, reduced risk of infection, and low clinical costs. Interruptions in EN frequently influence nutritional support and clinical outcomes. We, therefore, aimed to determine the causes of interruptions in postoperative EN in CHD patients and discuss clinical counter measures...
May 2017: Asia Pacific Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28373784/left-ventricular-noncompaction-a-rare-form-of-cardiomyopathy-revelation-modes-and-predictors-of-mortality-in-adults-through-23-cases
#19
Iliyasse Asfalou, Sanae Boulaamayl, Maha Raissouni, Najat Mouine, Mohamed Sabry, Jamal Kheyi, Nawal Doghmi, Aatif Benyass
OBJECTIVES: To describe modes of clinical presentation and echocardiographic, angiographic, and rhythmic features, and prognostic characteristics of left ventricular noncompaction cardiomyopathy (LVNC) in North African adults, through one of the first series in Morocco. BACKGROUND: LVNC is a rare congenital disorder, described for the first time by Engberding in 1984. The suspected diagnosis in thromboembolic, hemodynamic, or rhythm events requires both echocardiography and cardiovascular magnetic resonance (CMR)...
April 2017: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/28371864/a-multicentre-study-of-patients-with-timothy-syndrome
#20
Mark A Walsh, Christian Turner, Katherine W Timothy, Neil Seller, Dominic L Hares, Andrew F James, Jules C Hancox, Orhan Uzun, Dean Boyce, Alan G Stuart, Paul Brennan, Caroline Sarton, Karen McGuire, Ruth A Newbury-Ecob, Karen Mcleod
Aims: Timothy syndrome (TS) is an extremely rare multisystem disorder characterized by marked QT prolongation, syndactyly, seizures, behavioural abnormalities, immunodeficiency, and hypoglycaemia. The aim of this study was to categorize the phenotypes and examine the outcomes of patients with TS. Methods and results: All patients diagnosed with TS in the United Kingdom over a 24-year period were reviewed. Fifteen centres in the British Congenital Arrhythmia Group network were contacted to partake in the study...
March 24, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
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