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cardiac malformation

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https://www.readbyqxmd.com/read/28329229/isolated-severe-leftward-displacement-of-the-septum-primum-anatomic-and-3d-echocardiographic-findings-and-surgical-repair
#1
Fabio Cuttone, Khaled Hadeed, François Lacour-Gayet, Hugues Lucron, Sebastien Hascoet, Philippe Acar, Bertrand Leobon, Richard Van Praagh
OBJECTIVES: Leftward displacement of the septum primum is usually described as associated with hypoplastic left heart syndrome or visceral heterotaxy. This rare malformation results in partially or totally anomalous pulmonary venous drainage with a normal connection of the pulmonary veins to the left atrium, depending on the degree of septal shift. We report the 3D echocardiographic and anatomic findings as well as the surgical repair in a series of isolated severe leftward displacement of the septum primum, responsible for totally anomalous pulmonary venous drainage...
February 16, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28314534/total-cavopulmonary-connection-with-a-new-bioabsorbable-vascular-graft-first-clinical-experience
#2
Leo A Bockeria, Oleg Svanidze, Alex Kim, Konstantin Shatalov, Vladimir Makarenko, Martijn Cox, Thierry Carrel
OBJECTIVES: To assess safety and clinical performance of a novel bioabsorbable vascular graft in pediatric patients with univentricular cardiac malformation who received surgical correction via an extracardiac cavopulmonary conduit. METHODS: The implanted graft material is designed to attract patient's own cells and proteins, which trigger a cascade of physiological events leading to endogenous tissue restoration. As the graft resorbs progressively after implantation, components of native tissue including collagen, endothelial lining, and capillary blood vessels develop and organize into a natural tissue...
February 7, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28302741/cardiac-regeneration-lessons-from-development
#3
Francisco X Galdos, Yuxuan Guo, Sharon L Paige, Nathan J VanDusen, Sean M Wu, William T Pu
Palliative surgery for congenital heart disease has allowed patients with previously lethal heart malformations to survive and, in most cases, to thrive. However, these procedures often place pressure and volume loads on the heart, and over time, these chronic loads can cause heart failure. Current therapeutic options for initial surgery and chronic heart failure that results from failed palliation are limited, in part, by the mammalian heart's low inherent capacity to form new cardiomyocytes. Surmounting the heart regeneration barrier would transform the treatment of congenital, as well as acquired, heart disease and likewise would enable development of personalized, in vitro cardiac disease models...
March 17, 2017: Circulation Research
https://www.readbyqxmd.com/read/28302550/the-22q11-2-deletion-syndrome-in-congenital-heart-defects-prevalence-of-microdeletion-syndrome-in-cameroon
#4
Ambroise Wonkam, Ricardo Toko, David Chelo, Cedrik Tekendo-Ngongang, Samuel Kingue, Sophie Dahoun
BACKGROUND: The 22q11.2 deletion syndrome is amongst the most common microdeletion syndrome in humans. Its prevalence remains unknown in sub-Saharan Africa, and its clinical features are under-reported for people of African descent. OBJECTIVE: We have investigated the prevalence of the 22q11.2 deletion syndrome in patients with congenital heart defects in Cameroon. METHODS: A total of 70 of 100 cases of congenital cardiac malformation with echocardiographic evidence were examined prospectively and tested for the 22q11...
March 13, 2017: Global Heart
https://www.readbyqxmd.com/read/28300009/prenatal-diagnosis-of-aortopulmonary-window-associated-with-aberrant-subclavian-artery
#5
Adetola F Louis-Jacques, Sarah Obican, Thieu Nguyen, Anthony Odibo
Aortopulmonary window is a rare cardiac developmental anomaly characterised by a communication between the ascending aorta and the pulmonary artery. Aortopulmonary window may be isolated or associated with cardiac defects such as ventricular septal defect, atrial septal defect, interrupted aortic arch, and tetralogy of Fallot. We report a case of aortopulmonary window associated with aberrant subclavian artery based on fetal two-dimensional echocardiogram. The mother was referred for fetal echocardiography because of multiple fetal anomalies...
March 16, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28295440/role-of-hdlive-in-imaging-the-fetal-heart
#6
Selvaraj Ravi Lakshmy, Bharat Jain, Nity Rose
HDLive is a rendering methodology that generates realistic images of the human fetus from sonographic data. The objective of this study is to demonstrate the utility of high-definition live in evaluating fetal heart especially in the first trimester (11-14-week) scan. The normal atrioventricular valve and its abnormalities along with septal defects can be vividly demonstrated with this technique, and eight cases with cardiac defects are illustrated. Its use in the first-trimester evaluation of heart would give a better perspective of the cardiac malformation, which provides the opportunity for counseling options early in pregnancy...
March 11, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28292826/arrhythmia-risk-and-%C3%AE-blocker-therapy-in-pregnant-women-with-long-qt-syndrome
#7
Kohei Ishibashi, Takeshi Aiba, Chizuko Kamiya, Aya Miyazaki, Heima Sakaguchi, Mitsuru Wada, Ikutaro Nakajima, Koji Miyamoto, Hideo Okamura, Takashi Noda, Toshifumi Yamauchi, Hideki Itoh, Seiko Ohno, Hideki Motomura, Yoshiharu Ogawa, Hiroko Goto, Takaomi Minami, Nobue Yagihara, Hiroshi Watanabe, Kanae Hasegawa, Akihiro Terasawa, Hitoshi Mikami, Kayo Ogino, Yukiko Nakano, Sato Imashiro, Yosuke Fukushima, Yoshimitsu Tsuzuki, Koko Asakura, Jun Yoshimatsu, Isao Shiraishi, Shiro Kamakura, Yoshihiro Miyamoto, Satoshi Yasuda, Takashi Akasaka, Minoru Horie, Wataru Shimizu, Kengo Kusano
BACKGROUND: Pregnancy is one of the biggest concerns for women with long QT syndrome (LQTS). OBJECTIVES: This study investigated pregnancy-related arrhythmic risk and the efficacy and safety of β-blocker therapy for lethal ventricular arrhythmias in pregnant women with LQTS (LQT-P) and their babies. METHODS: 136 pregnancies in 76 LQT-P (29±5 years old; 22 LQT1, 36 LQT2, one LQT3, and 17 genotype-unknown) were enrolled. We retrospectively analysed their clinical and electrophysiological characteristics and pregnancy outcomes in the presence (BB group: n=42) or absence of β-blocker therapy (non-BB group: n=94)...
March 14, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28291300/-possible-relation-between-antenatal-venlafaxine-use-and-vacterl-association-in-a-newborn-a-case-report
#8
Muammer Özgür Çevik, Mustafa Çelik, İbrahim Hakan Bucak, Behice Han Almış, Mehmet Turğut
Major depressive disorder is common during antenatal period and many women are prescribed antidepressant drugs despite no antidepressant can be regarded as definitely safe in pregnancy. Previous studies have suggested links between gestational use of selective serotonin reuptake inhibitors (SSRI) or serotonin and norepinephrine reuptake inhibitors (SNRI) and certain birth defects. VACTERL association is a rare group of congenital malformations which were observed to occur together more often than would be expected by chance...
2017: Türk Psikiyatri Dergisi, Turkish Journal of Psychiatry
https://www.readbyqxmd.com/read/28290856/-chronic-heart-failure-with-preserved-systolic-function-and-reversible-dilatation-of-cardiac-chambers
#9
O V Blagova, S V Volkov, A V Nedostup, A O Korobkov, I V Mostovoy, N G Sergushina, N V Gagarina, E A Mershina
Diagnosis of dilation (D) cardiomyopathy (CMP) requires exclusion not only of inflammatory and genetically determined forms but also of some rare diseases. This 51 year old patient with history of moderate arterial hypertension approached a cardiologist because of new onset atrial fibrillation and dyspnea. Echocardiography detected dilation of all cardiac chambers with relatively preserved ejection fraction, causing suspicion of DCMP. Among conditions excluded were coronary atherosclerosis, congenital heart defect with left to right shunt, primary pulmonary hypertension, pulmonary embolism, hypertensive heart, tachycardia induced CMP, arrhythmogenic right ventricular dysplasia, noncompaction myocardium...
June 2016: Kardiologiia
https://www.readbyqxmd.com/read/28284154/contribution-of-g-protein-coupled-estrogen-receptor-1-gper-to-17%C3%AE-estradiol-induced-developmental-toxicity-in-zebrafish
#10
Graciel Diamante, Norma Menjivar-Cervantes, Man Sin Leung, David C Volz, Daniel Schlenk
Exposure to 17β-estradiol (E2) influences the regulation of multiple signaling pathways, and E2-mediated disruption of signaling events during early development can lead to malformations such as cardiac defects. In this study, we investigated the potential role of the G-protein estrogen receptor 1 (GPER) in E2-induced developmental toxicity. Zebrafish embryos were exposed to E2 from 2h post fertilization (hpf) to 76 hpf with subsequent transcriptional measurements of heart and neural crest derivatives expressed 2 (hand2), leucine rich repeat containing 10 (lrrc10), and gper at 12, 28 and 76 hpf...
March 2, 2017: Aquatic Toxicology
https://www.readbyqxmd.com/read/28282706/cardiovascular-malformations-in-infants-of-diabetic-mothers-a-retrospective-case-control-study
#11
Parvin Akbariasbagh, Mamak Shariat, Naseredin Akbariasbagh, Bita Ebrahim
Maternal diabetes has teratogenic effects on the evolution of the fetal cardiovascular system; as a consequence, cardiovascular malformations are the most common anomalies in infants of diabetic mothers. The present study focuses on the association of all types of diabetes in mothers with the incidence of congenital cardiovascular malformations in their infants. In this retrospective case-control study performed between the years 2008 and 2010, 35 infants of diabetic mothers were selected as a case group, and another 35 infants of mothers with normal blood glucose levels were selected as a control group...
February 2017: Acta Medica Iranica
https://www.readbyqxmd.com/read/28273057/the-association-of-accessory-auricular-tissue-with-solid-organ-abnormalities-and-its-effect-on-auditory-and-vestibular-function
#12
Hillary E Jenny, Benjamin B Massenburg, E Hope Weissler, Peter J Taub
BACKGROUND: Accessory auricular tissue is a common congenital anomaly ranging from an accessory skin appendage to a separate pinna. The association between auditory or vestibular dysfunction and accessory auricular tissue is debated, and little is known about related solid organ abnormalities. We examine the prevalence of accessory auricular tissue, its association between solid organ abnormalities and auditory/vestibular dysfunction, and its management. METHODS: A retrospective cohort study was performed using the 2000 to 2012 HCUP kids' inpatient database...
April 2017: Annals of Plastic Surgery
https://www.readbyqxmd.com/read/28268004/aortic-shear-stress-in-patients-with-bicuspid-aortic-valve-with-stenosis-and-insufficiency
#13
Yan Shan, Jun Li, Yongshi Wang, Boting Wu, Alex J Barker, Michael Markl, Chunsheng Wang, Xiaolin Wang, Xianhong Shu
OBJECTIVES: Bicuspid aortic valve, characterized by valve malformation and risk for aortopathy, displays profound alteration in systolic aortic outflow and wall shear stress distribution. The present study performed 4-dimensional flow magnetic resonance imaging in patients with bicuspid aortic valve with right-left cusp fusion, focusing on the impact of valve function on hemodynamic status within the ascending aorta. METHODS: Four-dimensional flow magnetic resonance imaging was performed in 50 subjects with right-left bicuspid aortic valve and 15 age- and aortic size-matched controls with tricuspid aortic valve...
February 10, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28265215/nonfluoroscopic-imaging-as-guidance-for-radiofrequency-ablation-of-atrioventricular-nodal-reentrant-tachycardia-after-mustard-repair
#14
Jan Hluchy, Dinh Q Nguyen, Henrik Sobczak, Bodo Brandts
Most tachycardias in the pulmonary venous atrium are inaccessible by direct means and require either a retrograde approach or a transseptal approach for ablation. We present a case in which successful radiofrequency ablation of common atrioventricular nodal reentrant tachycardia was accomplished via a retrograde transaortic approach guided by nonfluoroscopic mapping with use of the NavX(™) mapping system. The patient was a 49-year-old woman who at the age of 4 years had undergone Mustard repair for complete dextrotransposition of the great arteries...
February 2017: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28258482/a-rare-malformation-of-bilateral-superior-vena-cava-with-bilateral-partial-anomalous-pulmonary-venous-connection-in-the-presence-of-ostium-secundum-atrial-septal-defect-management-strategies-and-pitfalls
#15
Malkesh Tarsaria, Kartik Patel, Amber Malhotra, Pranav Sharma, Vivek Wadhawa, Jaydeep Ramani
Bilateral superior vena cava (SVC) with bilateral partial anomalous pulmonary venous connection is a very rare congenital cardiac malformation. Here, we are reporting a case of 18-year-old male who had bilateral SVC with bilateral anomalous pulmonary venous connection associated with ostium secundum atrial septal defect. The patient underwent successful surgical correction for the same.
March 3, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28246457/medico-legal-aspects-of-congenital-heart-diseases-in-buying-and-selling-of-pets
#16
Annamaria Passantino, Michela Pugliese, Valeria Quartarone, Natalia Russo, Roberto Bussadori, Bartolomeo Guercio
AIM: The veterinarian should be able to assess congenital and inherited malformations such as heart defects because they may be object of legal disputes. In this study, the authors report some cases of congenital heart defects in pets (dogs and cats) to clarify whether or not they may be considered a redhibitory defect. MATERIALS AND METHODS: A total of 28 medical records of pets referred with suspected congenital heart disease were examined. All patients aged between 3 and 24 months underwent clinical examination, chest X-ray examination, electrocardiogram, and echocardiography and angiocardiography when necessary...
January 2017: Veterinary World
https://www.readbyqxmd.com/read/28244688/determinants-of-exercise-capacity-in-school-aged-esophageal-atresia-patients
#17
Leontien C C Toussaint-Duyster, Monique H M van der Cammen-van Zijp, Marjolein Spoel, Mhanfei Lam, Rene M H Wijnen, Johan C de Jongste, Dick Tibboel, Joost van Rosmalen, Hanneke IJsselstijn
BACKGROUND AND AIMS: Data on long-term outcome of exercise capacity in school-aged children with esophageal atresia (EA) are scarce. We evaluated maximal exercise capacity and its relation to lung function. Moreover, we studied other possible determinants of exercise capacity and lung function. METHODS: Exercise capacity of 63 children with EA born 1999-2007 was evaluated at the age of 8 years with the Bruce-protocol. Dynamic and static lung volumes, bronchodilator response and diffusion capacity were measured...
February 28, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28237811/the-capz-interacting-protein-rcsd1-is-required-for-cardiogenesis-downstream-of-wnt11a-in-xenopus-laevis
#18
Annemarie Hempel, Susanne J Kühl, Melanie Rothe, Purushothama Rao Tata, Ioan Ovidiu Sirbu, Seppo J Vainio, Michael Kühl
Wnt proteins are critical for embryonic cardiogenesis and cardiomyogenesis by regulating different intracellular signalling pathways. Whereas canonical Wnt/β-catenin signalling is required for mesoderm induction and proliferation of cardiac progenitor cells, β-catenin independent, non-canonical Wnt signalling regulates cardiac specification and terminal differentiation. Although the diverse cardiac malformations associated with the loss of non-canonical Wnt11 in mice such as outflow tract (OFT) defects, reduced ventricular trabeculation, myofibrillar disorganization and reduced cardiac marker gene expression are well described, the underlying molecular mechanisms are still not completely understood...
April 1, 2017: Developmental Biology
https://www.readbyqxmd.com/read/28237611/abnormal-pregnancy-outcome-associated-with-high-dose-maternal-tranylcypromine-therapy-case-report-and-literature-review
#19
Debra Kennedy, William S Webster, Majella Hill, Helen E Ritchie
BACKGROUND: Tranylcypromine is a non-selective inhibitor of monamine oxidase which also inhibits the reuptake of norepinephrine. Spontaneous hypertensive reactions to the drug have been reported. In sheep tranylcypromine has been shown to cause a dose-dependent reduction in uterine blood flow. A similar effect in a pregnant woman might induce constriction of the uterine arteries and temporary fetal hypoxia. CASES: MotherSafe is a state-based Teratogen Information service and currently provides counselling to around 22,000 consumers and healthcare professionals annually regarding exposures during pregnancy and breastfeeding We report on the outcome of 2 pregnancies in a patient treated with high dose tranylcypromine as well as pimozide, diazepam and alprazolam...
February 22, 2017: Reproductive Toxicology
https://www.readbyqxmd.com/read/28237394/developmental-changes-in-the-balance-of-glycolytic-atp-production-and-oxidative-phosphorylation-in-ventricular-cells-a-simulation-study
#20
Hitomi I Sano, Tamami Toki, Yasuhiro Naito, Masaru Tomita
The developmental program of the heart requires accurate regulation to ensure continuous circulation and simultaneous cardiac morphogenesis, because any functional abnormalities may progress to congenital heart malformation. Notably, energy metabolism in fetal ventricular cells is regulated in a manner that differs from adult ventricular cells: fetal cardiomyocytes generally have immature mitochondria and fetal ventricular cells show greater dependence on glycolytic ATP production. However, although various characteristics of energy metabolism in fetal ventricular cells have been reported, to our knowledge, a quantitative description of the contributions of these factors to fetal ventricular cell functions has not yet been established...
February 24, 2017: Journal of Theoretical Biology
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