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https://www.readbyqxmd.com/read/28439482/association-of-non-alcoholic-fatty-liver-disease-with-conduction-defects-on-electrocardiogram
#1
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
#2
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...
April 24, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28431249/macrophages-facilitate-electrical-conduction-in-the-heart
#3
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
#4
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
#5
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
#6
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
#7
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
https://www.readbyqxmd.com/read/28341515/computerised-interpretation-of-fetal-heart-rate-during-labour-infant-a-randomised-controlled-trial
#8
(no author information available yet)
BACKGROUND: Continuous electronic fetal heart-rate monitoring is widely used during labour, and computerised interpretation could increase its usefulness. We aimed to establish whether the addition of decision-support software to assist in the interpretation of cardiotocographs affected the number of poor neonatal outcomes. METHODS: In this unmasked randomised controlled trial, we recruited women in labour aged 16 years or older having continuous electronic fetal monitoring, with a singleton or twin pregnancy, and at 35 weeks' gestation or more at 24 maternity units in the UK and Ireland...
March 21, 2017: Lancet
https://www.readbyqxmd.com/read/28331377/management-of-systemic-lupus-erythematosus-during-pregnancy-challenges-and-solutions
#9
REVIEW
Caroline L Knight, Catherine Nelson-Piercy
Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease predominantly affecting women, particularly those of childbearing age. SLE provides challenges in the prepregnancy, antenatal, intrapartum, and postpartum periods for these women, and for the medical, obstetric, and midwifery teams who provide their care. As with many medical conditions in pregnancy, the best maternal and fetal-neonatal outcomes are obtained with a planned pregnancy and a cohesive multidisciplinary approach. Effective prepregnancy risk assessment and counseling includes exploration of factors for poor pregnancy outcome, discussion of risks, and appropriate planning for pregnancy, with consideration of discussion of relative contraindications to pregnancy...
2017: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/28302382/congenital-heart-defect-causing-mutation-in-nkx2-5-displays-in-vivo-functional-deficit
#10
Abeer F Zakariyah, Rashida F Rajgara, John P Veinot, Ilona S Skerjanc, Patrick G Burgon
The Nkx2.5 gene encodes a transcription factor that plays a critical role in heart development. In humans, heterozygous mutations in NKX2.5 result in congenital heart defects (CHDs). However, the molecular mechanisms by which these mutations cause the disease remain unknown. NKX2.5-R142C is a mutation that was reported to be associated with atrial septal defect (ASD) and atrioventricular (AV) block in 13-patients from one family. The R142C mutation is located within both the DNA-binding domain and the nuclear localization sequence of NKX2...
April 2017: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/28280512/a-case-of-congenitally-corrected-transposition-of-great-arteries-an-infrequent-happenstance
#11
Prakash Ajmera, Vikas Medep
Congenitally corrected transposition of the great arteries (CCTGA) is rare form of congenital heart diseases. It may be present with or without associated anomalies. Patients with CCTGA are usually diagnosed at early stages of life due to associated anomalies, but they may even remain asymptomatic till later decades of their life. We report a case of a 42-year-old man who presented at neurosurgery department with dizziness, seizures, and loss of consciousness, in whom isolated CCTGA was discovered incidentally...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28265782/predictors-of-acute-inefficacy-and-the-radiofrequency-energy-time-required-for-cavotricuspid-isthmus-dependent-atrial-flutter-ablation
#12
Jordi Pérez-Rodon, Julian Rodriguez-García, Axel Sarrias-Merce, Nuria Rivas-Gandara, Ivo Roca-Luque, Jaume Francisco-Pascual, Alba Santos-Ortega, Gabriel Martín-Sánchez, Ignacio Ferreira-González, Jose Rodríguez-Palomares, Artur Evangelista-Masip, David García-Dorado, Àngel Moya-Mitjans
PURPOSE: Although cavotricuspid isthmus-dependent atrial flutter (CTI-AFL) ablation is a highly efficacious treatment, a bidirectional CTI block cannot be achieved in some patients. In this study, we sought to determine the predictors of acute inefficacy of CTI-AFL ablation and the predictors of the radiofrequency (RF) energy time required to achieve a complete bidirectional CTI block. METHODS: All consecutive patients who underwent stand-alone CTI-AFL ablation in our institution, except patients with congenital heart disease, were included in this retrospective study...
March 6, 2017: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/28259982/prevalence-and-spectrum-of-nkx2-5-mutations-in-patients-with-congenital-atrial-septal-defect-and-atrioventricular-block
#13
Ying-Jia Xu, Xing-Biao Qiu, Fang Yuan, Hong-Yu Shi, Lei Xu, Xu-Min Hou, Xin-Kai Qu, Xu Liu, Ri-Tai Huang, Song Xue, Yi-Qing Yang, Ruo-Gu Li
Congenital atrial septal defect (ASD) and progressive atriventricular block (AVB) are the two most common phenotypes linked to NK2 homeobox 5 (NKX2.5) mutations in animals and humans. However, the prevalence and spectrum of NKX2.5 mutation in patients with ASD and AVB remain to be elucidated. In the present study, the coding exons and flanking introns of the NKX2.5 gene, which encodes a homeobox‑containing transcription factor essential for development of the heart, were sequenced in a cohort of 62 unrelated patients with ASD and AVB, and subsequently in a mutation carrier's available family members...
April 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28251027/spontaneous-remission-of-congenital-complete-atrioventricular-block-in-anti-ro-la-antibody-negative-monozygotic-twins-case-report
#14
Taner Kasar, Murat Saygı, İsa Özyılmaz, Yakup Ergül
BACKGROUND: Congenital complete atrioventricular block without any structural heart disease and anti-Ro/La negativity is very rare. Discordant complete atrioventricular block, which is more frequently defined in the literature as an autoimmune mechanism, is much more rare in monozygotic twins. CASE REPORT: The 26-year-old healthy mother had given birth in her first spontaneous, uneventful pregnancy to monozygotic twins at week 35. While the first twin's physical examination proved her to be normal with a pulse rate consistent with her age, the second twin had a pulse rate of approximately 40 beats/minute...
January 2017: Balkan Medical Journal
https://www.readbyqxmd.com/read/28229981/postprocedural-outcomes-and-risk-factors-for-arrhythmias-following-transcatheter-closure-of-congenital-perimembranous-ventricular-septal-defect-a-single-center-retrospective-study
#15
Li-Jian Zhao, Bo Han, Jian-Jun Zhang, Ying-Chun Yi, Dian-Dong Jiang, Jian-Li Lyu
BACKGROUND: Currently, transcatheter closure of perimembranous ventricular septal defect (pmVSD) is a widely accepted therapeutic modality. However, arrhythmias, especially postprocedural heart blocks, are a concern and outcomes are not very clear. This study explored the outcomes and risk factors of arrhythmias associated with transcatheter device closure of pmVSD. METHODS: A total of 395 patients diagnosed with pmVSD who successfully underwent transcatheter intervention between January 2010 and December 2015 in our center were retrospectively reviewed...
March 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28212920/neonatal-lupus-follow-up-in-infants-with-anti-ssa-ro-antibodies-and-review-of-the-literature
#16
REVIEW
Antonio Alberto Zuppa, Riccardo Riccardi, Simonetta Frezza, Francesca Gallini, Rita Maria Paola Luciano, Giovanni Alighieri, Costantino Romagnoli, Sara De Carolis
Neonatal Lupus Syndrome (NLS) is a distinct clinical entity caused by transplacental passage of maternal anti-SSA/Ro antibodies (Ab). Mothers may have systemic lupus erythematosus, Sjögren syndrome, or other connective tissue disease, or may be completely healthy at the time of giving birth. NLS includes several clinical manifestations: complete congenital heart block (CCHB) and cutaneous lupus are the most common, while hepatobiliary disease, hematological manifestations and central nervous system involvement may occur...
April 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28211989/timothy-syndrome-1-genotype-without-syndactyly-and-major-extracardiac-manifestations
#17
Róbert Sepp, Lidia Hategan, Attila Bácsi, Judit Cseklye, László Környei, János Borbás, Márta Széll, Tamás Forster, István Nagy, Zoltán Hegedűs
Timothy syndrome 1 (TS1) is a rare genetic disorder characterized by multisystem abnormalities including QT prolongation, congenital heart defects, facial dysmorphism, episodic hypoglycemia, and neurological symptoms. A morphological hallmark of TS1 is syndactyly, present in all cases. TS1 is caused by the canonical p.Gly406Arg mutation in the alternatively spliced exon 8A in the CACNA1C gene, encoding for the main cardiac L-type calcium channel. A variant case of TS1 is reported. The proband had intermittent fetal bradycardia with heart rate of 72 bpm...
March 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28204891/low-titer-isolated-anti-ro-ssa-60-kd-antibodies-is-correlated-with-positive-pregnancy-outcomes-in-women-at-risk-of-congenital-heart-block
#18
Marta Tonello, Ariela Hoxha, Elena Mattia, Alessandra Zambon, Silvia Visentin, Alessia Cerutti, Anna Ghirardello, Ornella Milanesi, Amelia Ruffatti
Congenital heart block (CHB) is an autoantibody mediated disorder presumably caused by placental transmission of maternal autoantibodies to Ro/SSA 52 kd, p200, Ro/SSA 60 kd, La/SSB ribonucleoproteins. This study investigated the clinical significance of isolated anti-Ro/SSA 52 kd, anti-p200, anti-Ro/SSA 60 kd, and anti-La/SSB antibodies in positive pregnant patients. One hundred sixty-three pregnant women positive to anti-Ro/SSA 52 kd and/or anti-Ro/SSA 60 kd and/or anti-La/SSB antibodies were prospectively enrolled in the study...
February 15, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28197280/predictors-of-permanent-pacemaker-implantation-after-coronary-artery-bypass-grafting-and-valve-surgery-in-adult-patients-in-current-surgical-era
#19
Bandar Al-Ghamdi, Yaseen Mallawi, Azam Shafquat, Alexandra Ledesma, Nadiah AlRuwaili, Mohamed Shoukri, Shahid Khan, Aly Al Sanei
BACKGROUND: Permanent pacemaker (PPM) implantation after cardiac surgery is required in 0.4-6% of patients depending on cardiac surgery type. PPM implantation in the early postoperative period may reduce morbidity and postoperative hospital stay. We performed a retrospective review of electronic medical records of adult patients with coronary artery bypass grafting (CABG), valve surgery, or both, over a 3-year period. Our aim was to identify predictors of PPM requirements and PPM dependency on follow-up in the current surgical era...
August 2016: Cardiology Research
https://www.readbyqxmd.com/read/28192468/matrix-metalloproteinases-are-required-for-membrane-motility-and-lumenogenesis-during-drosophila-heart-development
#20
Qanber S Raza, Jessica L Vanderploeg, J Roger Jacobs
Matrix Metalloproteinases (Mmps) degrade glycoproteins and proteoglycans of the extracellular matrix (ECM) or cell surface and are crucial for morphogenesis. Mmps and their inhibitors are expressed during early stages of cardiac development in vertebrates and expression is altered in multiple congenital cardiomyopathies such as cardia bifida. Drosophila genome encodes two copies of Mmps, Mmp1 and Mmp2 whereas in humans up to 25 Mmps have been identified with overlapping functions. We investigated the role of Mmps during embryonic heart development in Drosophila, a process which is morphogenetically similar to early heart tube formation in vertebrates...
2017: PloS One
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