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Truncus arteriosus

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https://www.readbyqxmd.com/read/29111286/role-of-computed-tomography-angiography-in-the-differentiation-of-feline-truncus-arteriosus-communis-from-pulmonary-atresia-with-ventricular-septal-defect
#1
Lauren E Markovic, Brian A Scansen, Brianna M Potter
Two domestic shorthair cats, a 6-month-old castrated male and a 7-month-old intact female, were diagnosed with complex congenital heart disease. Transthoracic echocardiography in both cats revealed a dilated arterial trunk overriding the interventricular septum with a large ventricular septal defect. The pulmonary trunk and branch pulmonary arteries were not visible using standard echocardiographic views in either cat. The differential diagnosis for both cats included truncus arteriosus communis vs. pulmonary atresia with ventricular septal defect...
October 27, 2017: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
https://www.readbyqxmd.com/read/29101065/targeted-sequencing-identifies-novel-gata6-variants-in-a-large-cohort-of-patients-with-conotruncal-heart-defects
#2
Erge Zhang, Nanchao Hong, Sun Chen, Qihua Fu, Fen Li, Yu Yu, Kun Sun
Studies have highlighted the critical role of GATA6 in conotruncal heart defects (CTDs). Nevertheless, relationship between GATA6 variants and different CTDs remains largely unknown. Here GATA6 gene was screened in 542 patients with CTDs using targeted sequencing. Variant frequency was 2.0% (11/542). Three novel variants: c.86C>A (p.A29E), c.296T>A (p.V99D) and c.1254delC (p.S418fs) were identified in patients with transposition of the great arteries, double outlet right ventricle and persistent truncus arteriosus, respectively, but in none of the 400 controls...
October 31, 2017: Gene
https://www.readbyqxmd.com/read/29082593/a-rare-mechanism-of-aortic-regurgitation-in-a-young-patient
#3
Ahmet Guner, Sabahattin Gunduz, Cagatay Onal, Taylan Akgun, Emrah Bayam, Ozkan Candan, Mehmet Ozkan
A 19-year-old male patient was admitted to our institute with dyspnea. His medical history had no rheumatic fever or infective endocarditis. Physical examination revealed a diastolic murmur over the aortic area, rales of bilateral lungs. Bedside transthoracic echocardiography (TTE) revealed a severe aortic regurgitation (AR) without aortic valve stenosis and a moderately dilated left ventricle accompanied by an ejection fraction of 55%. The aortic valve could not be clearly demonstrated as either bicuspid or tricuspid...
October 29, 2017: Echocardiography
https://www.readbyqxmd.com/read/29070092/late-management-of-truncus-arteriosus-20-years-of-humanitarian-experience
#4
Marielle Gouton, Vincent Lucet, Olivier Bical, Francine Leca
OBJECTIVES: Early surgical management of common arterial trunk is well established and has good prognosis. Late diagnosis is less common. We reviewed late-diagnosed common arterial trunk management and prognosis for children in developing countries. We also discuss the need for prior catheterisation. Material and methods We reviewed all common arterial trunk patients managed by our humanitarian organization since 1996. RESULTS: A total of 41 children with common arterial trunk were managed at a mean age of 3 years old...
October 26, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/29069409/initial-2-year-results-of-cardiocel%C3%A2-patch-implantation-in-children
#5
Carine Pavy, Guido Michielon, Jan Lukas Robertus, François Lacour-Gayet, Olivier Ghez
OBJECTIVES: We present the initial 2-year results of CardioCel® patch (Admedus Regen Pty Ltd, Perth, WA, Australia) implantation in paediatric patients with congenital heart diseases. METHODS: This was a single-centre retrospective study with prospectively collected data of all patients aged 18 years and under operated for congenital heart disease. The patch was introduced in 2014, with clinical practice committee approval and a special consent in case of an Ozaki procedure...
October 24, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29068803/anaphylaxis-to-surgiflo-during-posterior-spinal-fusion-in-an-adolescent-status-post-truncus-arteriosus-repair-a-case-report
#6
Ana Maria Manrique Espinel, Jeffrey M Feldman, Susan Nelson, Tatiana Smaliak, John M Flynn, Susan C Nicolson
Anaphylactic reaction to gelatin-containing hemostatic agents has been reported in the orthopedic literature, most commonly during scoliosis repair in adolescents. However, the risk, differential diagnosis, and management of anaphylaxis in patients with complex congenital heart disease undergoing noncardiac procedures have not been previously reported. We describe the case of an adolescent with a history of repaired truncus arteriosus undergoing posterior spinal fusion who developed sudden and profound hypotension that was ultimately confirmed to be an anaphylactic reaction to Surgiflo...
October 24, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/29049800/ex-utero-intrapartum-treatment-to-extracorporeal-membrane-oxygenation-followed-by-cardiac-operation-for-truncus-arteriosus-communis
#7
Hidetsugu Asai, Tsuyoshi Tachibana, Yasushige Shingu, Yoshiro Matsui
Extracorporeal membrane oxygenation has been recently indicated as an ex utero intrapartum treatment procedure for placental support in patients with a large thoracic mass. In our case, a foetus with truncus arteriosus communis and hydrops with ventricular failure due to severe truncal valve stenosis was delivered under ex utero intrapartum treatment-to-extracorporeal membrane oxygenation at 34 weeks of gestation. The neonate underwent truncal valve plasty. He could be weaned off extracorporeal membrane oxygenation, but he died because of the infection on postnatal Day 24...
September 13, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29046108/prenatal-diagnosis-of-truncus-arteriosus-in-the-first-trimester-with-a-high-frequency-curved-transducer
#8
Yan Yi, Tong Tong, Tao Liu, Qi Lin, Yi Xiong, Jinfeng Xu
No abstract text is available yet for this article.
October 19, 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/29034225/the-perception-of-a-three-dimensional-printed-heart-model-from-the-perspective-of-different-stakeholders-a-complex-case-of-truncus-arteriosus
#9
Giovanni Biglino, Sarah Moharem-Elgamal, Matthew Lee, Robert Tulloh, Massimo Caputo
The case of an 11-year-old male patient with truncus arteriosus is presented. The patient has a right aortic arch, a repaired truncus arteriosus, pulmonary artery stenosis, as well as conduit stenosis, with a complex surgical plan being discussed. In order to gather additional insight into the patient's anatomy prior to the surgery and to facilitate communication with the patient's parents, a three-dimensional (3D) model of his heart and main vessels was created from computed tomography data. Feedback was collected from different stakeholders...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28961240/myocardial-wall-stiffening-in-a-mouse-model-of-persistent-truncus-arteriosus
#10
Christine Miller Buffinton, Alyssa K Benjamin, Ashley N Firment, Anne M Moon
BACKGROUND: Genetic and epigenetic programs regulate dramatic structural changes during cardiac morphogenesis. Concurrent biomechanical forces within the heart created by blood flow and pressure in turn drive downstream cellular, molecular and genetic responses. Thus, a genetic-morphogenetic-biomechanical feedback loop is continually operating to regulate heart development. During the evolution of a congenital heart defect, concomitant abnormalities in blood flow, hemodynamics, and patterns of mechanical loading would be predicted to change the output of this feedback loop, impacting not only the ultimate morphology of the defect, but potentially altering tissue-level biomechanical properties of structures that appear structurally normal...
2017: PloS One
https://www.readbyqxmd.com/read/28925321/an-unusual-combination-of-truncus-arteriosus-interrupted-aortic-arch-and-hypoplastic-left-ventricle
#11
Supreet P Marathe, Sanjeev H Naganur, Sabarinath Menon, Yishay Orr, Stephen G Cooper, David S Winlaw
Truncus arteriosus (common arterial trunk) is known to be associated with interrupted aortic arch in 10% to 15% of cases. However, the association of either of these lesions with a hypoplastic left ventricle is rare. The combination of all three of these lesions along with an intact interventricular septum is virtually unknown with only two cases reported in the literature. We report such a case with its anatomical and surgical management aspects.
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28814167/cardio-cephalic-neural-crest-syndrome-a-novel-hypothesis-of-vascular-neurocristopathy
#12
M Komiyama
A novel hypothesis proposes that "cardio-cephalic neural crest (NC) syndrome," i.e. cephalic NC including cardiac NC, contributes to the concurrent occurrence of vascular diseases in the cardio- and cerebrovascular regions. NC is a transient structure present in early embryogenesis. Cephalic NC provides mesenchymal cells to the vascular media in these regions. Concurrent cardio- and cerebrovascular lesions have been reported in PHACE syndrome, ACTA2 mutation syndrome, and less frequently in the spontaneous occlusion of the circle of Willis (so-called moyamoya disease)...
December 2017: Interventional Neuroradiology
https://www.readbyqxmd.com/read/28714851/pdgfr%C3%AE-functions-in-endothelial-derived-cells-to-regulate-neural-crest-cells-and-the-development-of-the-great-arteries
#13
Haig Aghajanian, Young Kuk Cho, Nicholas W Rizer, Qiaohong Wang, Li Li, Karl Degenhardt, Rajan Jain
Originating as a single vessel emerging from the embryonic heart, the truncus arteriosus must septate and remodel into the aorta and pulmonary artery to support postnatal life. Defective remodeling or septation leads to abnormalities collectively known as conotruncal defects, which are associated with significant mortality and morbidity. Multiple populations of cells must interact to coordinate outflow tract remodeling, and the cardiac neural crest has emerged as particularly important during this process. Abnormalities in the cardiac neural crest have been implicated in the pathogenesis of multiple conotruncal defects, including persistent truncus arteriosus, double outlet right ventricle and tetralogy of Fallot...
September 1, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28669502/the-impact-of-22q11-2-deletion-syndrome-on-surgical-repair-outcomes-of-conotruncal-cardiac-anomalies
#14
COMPARATIVE STUDY
Bahaaldin Alsoufi, Courtney McCracken, Subhadra Shashidharan, Shriprasad Deshpande, Kirk Kanter, Brian Kogon
BACKGROUND: We aim to describe the impact of 22q11.2 deletion syndrome (22q11DS) on clinical characteristics, postoperative course, and early and late outcomes of neonates undergoing surgery for conotruncal anomalies. METHODS: A retrospective review was performed (2002 to 2012) of 224 neonates who underwent surgery for interrupted aortic arch (n = 67), truncus arteriosus (n = 85), or ductal-dependent pulmonary atresia and ventricular septal defect (n = 72). Patients were divided into three groups: group 1, n = 119, no genetic syndrome; group 2, n = 64, 22q11DS; and group 3, n = 41, other genetic syndrome...
November 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28645293/prenatal-screening-of-fetal-ventriculoarterial-connections-benefits-of-4d-technique-in-fetal-heart-imaging
#15
Yu Wang, Miao Fan, Faiza Amber Siddiqui, Meilian Wang, Wei Sun, Xue Sun, Wenjia Lei, Ying Zhang
BACKGROUND: Identification of prenatal ventriculoarterial connections in fetuses with conotruncal anomalies (CTA) remains one of the greatest challenges for sonographers performing screening examinations. Herein, we propose a novel protocol of 4D volume analysis that identifies ventriculoarterial connections and evaluate its clinical utility in routine screenings. METHODS: Twenty-nine cases of transposition of the great arteries (TGA), 22 cases of double-outlet right ventricle (DORV), 36 cases of tetralogy of Fallot (TOF), 14 cases of truncus arteriosus (TCA), and randomly selected 70 normal fetuses were reviewed in this study...
June 23, 2017: Cardiovascular Ultrasound
https://www.readbyqxmd.com/read/28633267/perventricular-implantation-of-melody-valve-in-child-with-pulmonary-hypertension-after-a-potts-shunt
#16
Tomasz Mroczek, Marcin Demkow, Tomasz Moszura, Aleksandra Morka, Janusz Skalski
The Potts operation is a surgical option for the treatment of children with suprasystemic pulmonary arterial hypertension (PAH). Its positive hemodynamic effect may be obliterated by pulmonary valvar insufficiency. We describe a case in which the Potts shunt was followed by perventricular Melody valve implantation in the pulmonary position in a 12-kg child with a previously repaired truncus arteriosus communis (TAC) in whom PAH developed. The combined approach using both procedures significantly improved functional capacity and led to spectacular reverse remodeling of the right ventricle seen at 2-year follow-up...
July 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28610465/successful-truncal-valve-replacement-with-a-mechanical-valve-after-bilateral-pulmonary-artery-banding
#17
Shuichi Yoshitake, Yukihiro Kaneko, Kazuki Yakuwa, Ikuya Achiwa
Surgical management of truncus arteriosus, or common arterial trunk, has expanded over the past three decades to include earlier surgical intervention, initially during infancy and now chiefly in the neonatal period. Many studies have shown that preoperative truncal valve insufficiency is an independent risk factor for mortality. We herein present the case of a five-month-old boy with severe truncal valve insufficiency who underwent repair of common arterial trunk and prosthetic replacement of the truncal valve with associated annular enlargement after initial stabilization in the newborn period via palliative bilateral pulmonary artery banding...
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28553164/tbx20-loss-of-function-mutation-responsible-for-familial-tetralogy-of-fallot-or-sporadic-persistent-truncus-arteriosus
#18
Ri-Tai Huang, Juan Wang, Song Xue, Xing-Biao Qiu, Hong-Yu Shi, Ruo-Gu Li, Xin-Kai Qu, Xiao-Xiao Yang, Hua Liu, Ning Li, Yan-Jie Li, Ying-Jia Xu, Yi-Qing Yang
Congenital heart disease (CHD), the most common form of developmental abnormality in humans, remains a leading cause of morbidity and mortality in neonates. Genetic defects have been recognized as the predominant causes of CHD. Nevertheless, CHD is of substantial genetic heterogeneity and the genetic defects underlying CHD in most cases remain unclear. In the current study, the coding regions and splicing junction sites of the TBX20 gene, which encodes a T-box transcription factor key to cardiovascular morphogenesis, were sequenced in 175 unrelated patients with CHD, and a novel heterozygous TBX20 mutation, p...
2017: International Journal of Medical Sciences
https://www.readbyqxmd.com/read/28466610/neonatal-cardiac-surgery-in-the-new-era-lessons-learned-from-1000-consecutive-cases
#19
Gabriel Amir, Georgy Frenkel, Elchanan Bruckheimer, Alexander Lowenthal, Amichay Rotstein, Jacob Katz, Yelena Zeitlin, Ofer Schiller, Einat Birk
BACKGROUND: neonatal cardiac surgery has evolved over the last 50 years with a large percentage of the patients achieving complete physiological repair in the neonatal period. The remaining patients achieve staged palliation with an increasing amount of success. OBJECTIVES: To report our experience with 1000 neonatal cardiac surgical procedures performed in the last 10 years. METHODS: We conducted a retrospective analysis of surgical outcome in all neonatal patients who underwent cardiac surgery between January 2007 and July 2016 at Schneider Children's Medical Center of Israel...
November 2016: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28401036/anomalous-origin-of-the-left-pulmonary-artery-hemi-truncus-arteriosus
#20
Ali Shabbir Hussain, Mariam Shakir, Shabina Ariff, Rehan Ali, Babar Hassan
Hemi-truncus arteriosus is a rare congenital cardiovascular malformation. It usually presents in infancy and leads to development of progressive pulmonary vascular disease, heart failure, and death. We report a case of hemi truncus arteriosus in a 12-day-old neonate who was successfully managed at our institute.
March 2017: APSP Journal of Case Reports
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