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congenital heart block

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https://www.readbyqxmd.com/read/28097609/a-case-of-a-quadricuspid-aortic-valve-identified-preoperatively-using-transthoracic-echocardiography
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
https://www.readbyqxmd.com/read/27913834/key-role-of-pacing-site-as-determinant-factor-of-exercise-testing-performance-in-pediatric-patients-with-chronic-ventricular-pacing
#10
Michel Cabrera Ortega, Hiram Tápanes Duamy, Dunia B Benítez Ramos
Chronic right ventricular (RV) apical pacing has been associated with deterioration of functional capacity and chronotropic incompetence during exercise testing in children. The effects of alternative pacing site on exercise performance in pediatric population remain unknown. We evaluated the influence of ventricular pacing site on exercise capacity in pediatric patients with complete congenital atrioventricular block requiring permanent pacemaker therapy. Sixty-four children paced from RV apex (n = 26), RV midseptum (n = 15) and left ventricular (LV) apex (n = 23) were studied cross-sectionally...
December 2, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27913750/congenital-heart-block-and-immune-mediated-sensorineural-hearing-loss-possible-cross-reactivity-of-immune-response
#11
C Bason, I Pagnini, A Brucato, S Maestroni, A Puccetti, C Lunardi, R Cimaz
Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27904846/complete-congenital-heart-block-in-a-neonate-with-a-complex-congenital-heart-defect-in-africa
#12
Clovis Nkoke, Edvine Yonta Wawo, Liliane Kuate Mfeukeu, Larissa Makamte, Sandrine Dikosso Edie, Flore Esiene Balana
Congenital heart block (CHB) is rare disorder that has a higher mortality when associated with structural congenital heart defects. Very few cases have been reported in Sub-Saharan Africa (SSA). We present a case of complete CHB associated with a complex congenital heart defect in a neonate in Cameroon. A 1-month-old neonate in Cameroon was referred for the evaluation of bradycardia. The obstetrical ultrasound done during pregnancy revealed fetal bradycardia without further evaluation. Clinical examination showed well a developed neonate with bradycardia at 62 beats/minute, and mild cyanosis with oxygen saturation at 93% at room air...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27899284/defective-lymphatic-valve-development-and-chylothorax-in-mice-with-a-lymphatic-specific-deletion-of-connexin43
#13
Stephanie J Munger, Michael J Davis, Alexander M Simon
Lymphatic valves (LVs) are cusped luminal structures that permit the movement of lymph in only one direction and are therefore critical for proper lymphatic vessel function. Congenital valve aplasia or agenesis can, in some cases, be a direct cause of lymphatic disease. Knowledge about the molecular mechanisms operating during the development and maintenance of LVs may thus aid in the establishment of novel therapeutic approaches to treat lymphatic disorders. In this study, we examined the role of Connexin43 (Cx43), a gap junction protein expressed in lymphatic endothelial cells (LECs), during valve development...
January 15, 2017: Developmental Biology
https://www.readbyqxmd.com/read/27891405/complete-heart-block-in-pregnancy-a-report-of-emergency-caesarean-section-in-a-parturient-without-pacemaker
#14
Vandana Mohapatra, Aparajita Panda, Satyanarayan Behera, Jagadish Chandra Behera
Management of women with Complete Heart Block (CHB) presenting without pacing, during pregnancy and labour is debatable. Temporary pacemakers have been routinely inserted for labour and birth probably to withstand any haemodynamic variations. However, due to lack of large scale prospective studies, the necessity of this procedure has not been objectively assessed. Also, the most appropriate anaesthetic technique for caesarean section in women with CHB is yet to be clarified. We report herein the case of a pregnant woman with CHB who had uneventful emergency caesarean delivery under spinal anaesthesia without temporary pacing...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27883221/alcohol-exposure-causes-overexpression-of-heart-development-related-genes-by-affecting-the-histone-h3-acetylation-via-bmp-signaling-pathway-in-cardiomyoblast-cells
#15
Jin Shi, Weian Zhao, Bo Pan, Min Zheng, Lina Si, Jing Zhu, Lingjuan Liu, Jie Tian
BACKGROUND: Abusive alcohol utilization of pregnant woman may cause congenital heart disease (CHD) of fetus, where alcohol ignites histone H3 hyperacetylation leading to abnormal development of heart morphogenesis and associated genes. Knowledge about the regularized upstream genes is little, but bone morphogenetic protein (BMP) signaling may actively and prominently take part in alteration in acetylation of histone H3. The supreme objective of this study was to unearth the involvement of BMP signaling pathway in alcohol-driven hyperacetylation of histone H3 in cardiomyoblast cells...
January 2017: Alcoholism, Clinical and Experimental Research
https://www.readbyqxmd.com/read/27882249/surviving-all-odds-a-unique-case-of-multiple-congenital-unruptured-sinus-of-valsalva-aneurysms-involving-both-left-and-right-coronary-sinuses-with-biventricular-dysfunction-and-heart-block
#16
Aniketh Vijay B, Vikrant Vijan, Navin Mathew
Aneurysms of the sinus of Valsalva are very uncommon, with an incidence ranging from 0.1 to 3.5% of all congenital heart defects. Very few cases have been reported in the literature that presented with involvement of two or more sinuses. We report a case of 27-year-old male with a history of exertional breathlessness of one-month duration. After complete evaluation using transesophageal echocardiography (TEE) and multiple detector computed tomography (MDCT) scanning, the patient was diagnosed to have large congenital unruptured sinus of Valsalva aneurysms involving both left and right coronary sinuses with extension into the interventricular septum...
2016: Case Reports in Cardiology
https://www.readbyqxmd.com/read/27826129/genotypic-and-phenotypic-predictors-of-complete-heart-block-and-recovery-of-conduction-after-surgical-repair-of-congenital-heart-disease
#17
Laura E Murray, Andrew H Smith, English C Flack, Kim Crum, Jill Owen, Prince J Kannankeril
BACKGROUND: Complete heart block (CHB) is a major complication that occurs after congenital heart surgery. We hypothesized that genetic and clinical factors are associated with the development of postoperative CHB and recovery of atrioventricular (AV) conduction. OBJECTIVE: The purpose of this study was to identify predictors of CHB and recovery after congenital heart surgery. METHODS: Patients undergoing congenital heart surgery at our institution from September 2007 through June 2015 were prospectively enrolled in a parent study of postoperative arrhythmias...
November 5, 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27803518/successful-preventive-treatment-of-congenital-heart-block-during-pregnancy-in-a-woman-with-systemic-lupus-erythematosus-with-anti-la-ro-antibody
#18
Vishwakarma Pratibha, Shankar Kundavi, Varma R Thangam, S Ramakrishnan
No abstract text is available yet for this article.
October 2016: Journal of Obstetrics and Gynaecology of India
https://www.readbyqxmd.com/read/27803192/interchromosomal-core-duplicons-drive-both-evolutionary-instability-and-disease-susceptibility-of-the-chromosome-8p23-1-region
#19
Kiana Mohajeri, Stuart Cantsilieris, John Huddleston, Bradley J Nelson, Bradley P Coe, Catarina D Campbell, Carl Baker, Lana Harshman, Katherine M Munson, Zev N Kronenberg, Milinn Kremitzki, Archana Raja, Claudia Rita Catacchio, Tina A Graves, Richard K Wilson, Mario Ventura, Evan E Eichler
Recurrent rearrangements of Chromosome 8p23.1 are associated with congenital heart defects and developmental delay. The complexity of this region has led to inconsistencies in the current reference assembly, confounding studies of genetic variation. Using comparative sequence-based approaches, we generated a high-quality 6.3-Mbp alternate reference assembly of an inverted Chromosome 8p23.1 haplotype. Comparison with nonhuman primates reveals a 746-kbp duplicative transposition and two separate inversion events that arose in the last million years of human evolution...
November 2016: Genome Research
https://www.readbyqxmd.com/read/27799231/electrophysiology-study-for-complex-supraventricular-tachycardia-in-congenital-heart-disease-patients-with-single-ventricle-physiology
#20
Shuenn-Nan Chiu, Jou-Kou Wang, Chun-Wei Lu, Kun-Lang Wu, Wei-Chieh Tseng, Mei-Hwan Wu
BACKGROUND: Supraventricular tachycardia (SVT) is common in complex congenital heart disease (CCHD) patients with single-ventricle physiology and may cause hemodynamic deterioration. We reported the outcomes of catheter ablation for such complex SVT in these single-ventricle CCHD patients. METHODS AND RESULTS: Patients with single-ventricle physiology (defined as CCHD patients) who received electrophysiology studies and catheter ablation between 1995 and 2015 were studied...
October 31, 2016: Journal of the American Heart Association
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