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congenital heart block

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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
#1
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...
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
#2
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...
February 14, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28211989/timothy-syndrome-1-genotype-without-syndactyly-and-major-extracardiac-manifestations
#3
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
#4
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
#5
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
#6
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
https://www.readbyqxmd.com/read/28179384/a-complicated-multisystem-flare-of-systemic-lupus-erythematosus-during-pregnancy
#7
Philip Webster, Catherine Nelson-Piercy, Liz Lightstone
We report a case of systemic lupus erythematosus (SLE) in a young woman who became pregnant amid a severe flare. She continued to have active disease in the face of aggressive treatments complicated by several side effects of immunosuppressive drugs including recurrent sepsis and gestational diabetes. Her fetus was at risk for congenital heart block during the second and third trimesters. Despite an extremely guarded prognosis, she delivered a healthy baby girl. This case highlights the complexities of SLE management during pregnancy...
February 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28138914/obstetric-and-perinatal-outcome-in-anti-ro-ssa-positive-pregnant-women-a-prospective-cohort-study
#8
Nuria Martínez-Sánchez, Sergio Pérez-Pinto, Ángel Robles-Marhuenda, Francisco Arnalich-Fernández, María Martín Cameán, Edurne Hueso Zalvide, Jose Luis Bartha
: Anti-Ro/SS-A is one specific type of antinuclear antibodies. They are in the majority of cases associated with primary Sjögren syndrome (SS) but also in Systemic Lupus Erythematosus (SLE), rheumatoid arthritis (RA), and in healthy people. During pregnancy, they are mainly associated to congenital heart block (CHB) and neonatal lupus (NL). The aim of this study was to compare the rate of maternal and fetal complications between a series of anti-Ro/SS-A positive pregnant women prospectively followed...
January 30, 2017: Immunologic Research
https://www.readbyqxmd.com/read/28118945/ro52-autoantibodies-arise-from-self-reactive-progenitors-in-a-mother-of-a-child-with-neonatal-lupus
#9
Joanne H Reed, Miroslaw K Gorny, Liuzhe Li, Timothy Cardozo, Jill P Buyon, Robert M Clancy
The detection of cardiac conduction defects in an 18-24 week old foetus in the absence of structural abnormalities predicts with near certainty the presence of autoantibodies against 60kD and 52kD SSA/Ro in the mother regardless of her health status. Previous studies have emphasized these autoantibodies as key mediators of tissue injury. The aim of this study was to focus on the anti-Ro52 response to determine whether these autoantibodies originate from progenitors that are inherently self-reactive or from B-cells that acquire self-reactivity during an immune response...
January 21, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28106024/-a-rare-cause-of-2-1-atrioventricular-block-and-congestive-heart-failure-in-preterm-infants-hypocalcemia
#10
Emine Azak, Hatice Tatar Aksoy, Handan Ünsal, İbrahim İlker Çetin
Atrioventricular (AV) block in the neonatal period is a rare disorder. It is frequently associated with underlying structural congenital heart disease and maternal lupus. Presently described is premature baby who developed 2:1 AV block and congestive heart failure due to hypocalcemia. Dramatic clinical improvement was observed following treatment of intravenous 10% calcium gluconate. Therefore, it is suggested that serum calcium level of newborns with AV block and congestive heart failure be measured.
January 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28100981/isolated-left-subclavian-artery-complete-atrioventricular-block-and-tricuspid-atresia-in-a-neonate
#11
Kanupriya Chaturvedi, Deepa Prasad, Ravi Ashwath, James P Strainic, Christopher S Snyder
Isolated left subclavian artery is one of the rarer aortic arch anomalies. It has been associated with other congenital heart diseases, typically tetralogy of Fallot, double-outlet right ventricle, and atrial and ventricular septal defects. Its significant clinical implications include a left-to-right shunt from the vertebrobasilar system, which causes pulmonary overcirculation and subclavian steal. We present an unusual case of a premature infant who was diagnosed prenatally with congenital complete atrioventricular block and tricuspid atresia and was found to have an isolated left subclavian artery postnatally...
December 2016: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28097609/a-case-of-a-quadricuspid-aortic-valve-identified-preoperatively-using-transthoracic-echocardiography
#12
Takanori Kono, Takeshi Oda, Keiichi Akaiwa, Katsuhiko Nakamura, Hiroyuki Tanaka
Quadricuspid aortic valve is an extremely rare congenital heart anomaly that often causes valve incompetence, requiring surgical intervention. Care must be taken to avoid surgical complications in patients with quadricuspid aortic valve; thus, preoperative diagnosis is important. A 76-year-old man presented with exertional dyspnea due to aortic regurgitation. Transthoracic and transesophageal echocardiography revealed severe aortic regurgitation caused by quadricuspid aortic valve. To avoid interference with the cardiac conduction system, we performed aortic valve replacement using an ingenious technique, in which pledgeted sutures on the accessory leaflet were placed from outside the sinus of Valsalva to above the aortic annulus...
January 18, 2017: Journal of Medical Ultrasonics
https://www.readbyqxmd.com/read/28092260/-down-syndrome-and-heart-block-secondary-to-neonatal-lupus
#13
Carla Lucila Illana-Bravo, Socorro Méndez-Martínez, María Enriqueta Juan-Martínez
In Down syndrome, so far, has not been reported it's association with congenital heart block, this entity is rare and occurred in only 1% of mothers who have systemic lupus erythematosus, the presence of anti-Ro antibodies cross the placenta presenting neonatal lupus with skin lesions and congenital heart block, bradycardia, which is why we describe the following case. This is a new male end product of asymptomatic young mother, but serological birth with stigmata of Down syndrome, birth presents congenital bradycardia rare manifestation abnormalities, but common in children of mothers with lupus are initiated study protocol, realizing you including laboratory tests and immunological studies cabinet as electrocardiogram and echocardiogram, which gave tone to take the mother immunological studies, being positive antiRo...
January 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28051234/neonatal-lupus-erythematosus-with-congenital-heart-block-in-twins
#14
Lamia Gargouri, Faiza Safi, Bayen Maalej, Souad Mallek, Fatma Turki, Imen Majdoub, Malek Akrout, Dorra Abid, Samir Kamoun, Abdelmajid Mahfoudh
Background - Neonatal lupus erythematosus is an uncommon acquired autoimmune disease caused by transplacental passage of maternal antibodies SSA/Ro, SSB/La or U1 ribonucleoproteins. The most common clinical manifestations are skin rash, cardiac lesions, thrombocytopenia, anemia and hepatosplenomegaly. Complete congenital heart block is usually irreversible needing a pacemaker implantation in two-thirds of cases. Cases report - We report neonatal lupus erythematosus with complete congenital heart block in twins...
July 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/28043470/single-center-experience-with-the-senning-procedure-in-the-current-era
#15
Giuseppe Ferro, Raghav Murthy, Vinod A Sebastian, Kristine J Guleserian, Joseph M Forbess
The Senning procedure is an operative technique for atrial inversion in congenital heart anomalies. We sought to evaluate our contemporary outcomes employing this technique. A retrospective analysis of all patients who underwent the Senning procedure at our institution was performed. Hospital records were reviewed, and follow-up data were obtained to evaluate outcomes. Overall, a total of 19 patients underwent a Senning procedure between August 2005 and July 2014. Median age at repair was 594 days (range: 5 days to 15 years)...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28018021/exome-sequencing-identifies-compound-heterozygous-mutations-in-scn5a-associated-with-congenital-complete-heart-block-in-the-thai-population
#16
Chuphong Thongnak, Pornprot Limprasert, Duangkamol Tangviriyapaiboon, Suchaya Silvilairat, Apichaya Puangpetch, Ekawat Pasomsub, Chonlaphat Sukasem, Wasun Chantratita
Background. Congenital heart block is characterized by blockage of electrical impulses from the atrioventricular node (AV node) to the ventricles. This blockage can be caused by ion channel impairment that is the result of genetic variation. This study aimed to investigate the possible causative variants in a Thai family with complete heart block by using whole exome sequencing. Methods. Genomic DNA was collected from a family consisting of five family members in three generations in which one of three children in generation III had complete heart block...
2016: Disease Markers
https://www.readbyqxmd.com/read/27997827/myocardial-vhl-hif-signaling-controls-an-embryonic-metabolic-switch-essential-for-cardiac-maturation
#17
Ivan Menendez-Montes, Beatriz Escobar, Beatriz Palacios, Manuel Jose Gómez, Jose Luis Izquierdo-Garcia, Lorena Flores, Luis Jesus Jiménez-Borreguero, Julian Aragones, Jesus Ruiz-Cabello, Miguel Torres, Silvia Martin-Puig
While gene regulatory networks involved in cardiogenesis have been characterized, the role of bioenergetics remains less studied. Here we show that until midgestation, myocardial metabolism is compartmentalized, with a glycolytic signature restricted to compact myocardium contrasting with increased mitochondrial oxidative activity in the trabeculae. HIF1α regulation mirrors this pattern, with expression predominating in compact myocardium and scarce in trabeculae. By midgestation, the compact myocardium downregulates HIF1α and switches toward oxidative metabolism...
December 19, 2016: Developmental Cell
https://www.readbyqxmd.com/read/27956337/-postsurgical-morbidity-in-pediatric-patients-undergoing-surgery-for-congenital-heart-disease-in-umae-from-yucatan
#18
Addy Castillo-Espínola, Ana Velázquez-Ibarra, Aurea Zetina-Solórzano, Patricia Bolado-García, Gonzalo Gamboa-López
OBJECTIVE: To describe the clinical course of pediatric patients undergoing surgery for congenital heart disease in UMAE from Yucatan. METHODS: Descriptive survey records of pediatric patients congenital heart undergoing surgery for congenital heart disease from November, 1st 2011 to November, 30st 2013. RESULTS: The most frequent heart diseases were the persistence ductus arteriosus (37.6%) and transposition of great vessels. The intensive care stay was 3days (median)...
December 9, 2016: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/27941748/congenital-complete-heart-block-presenting-in-pregnancy-a-case-report
#19
S Rouf, A W Chowdhury
Congenital complete heart block presenting for the first time in pregnancy is a rare occurrence posing a therapeutic challenge. We present a case of unpaced preexisting congenital complete heart block in pregnancy diagnosed for the first time during routine prenatal care at her early weeks of gestation. Our patient was asymptomatic and haemodynamically stable and was managed conservatively with a multidisciplinary term approach. Intrauterine growth retardation (IUGR) was present and she was delivered by ceasarean section at term for foetal distress with IUGR...
October 2016: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/27922246/neonatal-lupus-erythematosus-report-of-a-case-with-cutaneous-hematological-and-hepatobiliary-findings
#20
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
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