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https://www.readbyqxmd.com/read/28814167/cardio-cephalic-neural-crest-syndrome-a-novel-hypothesis-of-vascular-neurocristopathy
#1
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)...
January 1, 2017: Interventional Neuroradiology
https://www.readbyqxmd.com/read/28714851/pdgfr%C3%AE-functions-in-endothelial-derived-cells-to-regulate-neural-crest-cells-and-development-of-the-great-arteries
#2
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 cardiac neural crest has emerged as particularly important during this process. Abnormalities in 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...
July 14, 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
#3
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...
June 29, 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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
https://www.readbyqxmd.com/read/28386986/thrombus-formation-in-the-interrupted-segment-of-the-aorta
#10
Yusuf Karavelioğlu, Macit Kalçık, Mucahit Yetim, Tolga Doğan, Zehra Gölbaşı
Interrupted aorta is a very rare heart defect in which there is a gap between the ascending and the descending thoracic aorta. It is usually associated with other cardiac anomalies, including ventricular septal defect, ductus arteriosus, and truncus arteriosus. Severe cases present with serious complications such as hypertension, heart failure, or intracranial hemorrhage. Neurological complications are very rare form of presentation and commonly associated with intracranial aneurysms. We have reported a case of interrupted aorta who presented with transient ischemic attack due to thrombus formation in the interrupted segment of the aorta...
April 7, 2017: Echocardiography
https://www.readbyqxmd.com/read/28346476/reduced-dosage-of-%C3%AE-catenin-provides-significant-rescue-of-cardiac-outflow-tract-anomalies-in-a-tbx1-conditional-null-mouse-model-of-22q11-2-deletion-syndrome
#11
Silvia E Racedo, Erica Hasten, Mingyan Lin, Gnanapackiam Sheela Devakanmalai, Tingwei Guo, Ertugrul M Ozbudak, Chen-Leng Cai, Deyou Zheng, Bernice E Morrow
The 22q11.2 deletion syndrome (22q11.2DS; velo-cardio-facial syndrome; DiGeorge syndrome) is a congenital anomaly disorder in which haploinsufficiency of TBX1, encoding a T-box transcription factor, is the major candidate for cardiac outflow tract (OFT) malformations. Inactivation of Tbx1 in the anterior heart field (AHF) mesoderm in the mouse results in premature expression of pro-differentiation genes and a persistent truncus arteriosus (PTA) in which septation does not form between the aorta and pulmonary trunk...
March 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28290176/valve-dehiscence-after-bentall-procedure-the-detrimental-traits-of-propionibacterium
#12
Robert A F de Lind van Wijngaarden, Richard van Valen, Jos A Bekkers, Ad J J C Bogers
The present case exemplified the detrimental traits of prosthetic valve endocarditis caused by Propionibacterium acnes. As a baby, the patient had a congenital cardiac defect with truncus arteriosus type I with interrupted aortic arch and open ductus Botalli, and had undergone several operations. However, at 18 months after a Bentall procedure performed 29 years later he presented with major prosthetic dehiscence due to endocarditis. The patient underwent a high-risk reoperation for a re-do Bentall procedure and was treated postoperatively with intravenous antibiotics consisting of vancomycin for five weeks and penicillin and rifampicin each for six weeks...
November 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28267978/identification-of-essential-components-of-thoracoscopic-lobectomy-and-targets-for%C3%A2-simulation
#13
Mark K Ferguson, Cathy Bennett
BACKGROUND: Fewer than 50% of lobectomies for lung cancer are performed using thoracoscopic (video-assisted thoracic surgery [VATS]) techniques. This situation could be mitigated through the use of simulation training. Using a Delphi process, this study identified essential components of VATS right upper lobectomy most amenable to focused simulation. METHODS: Experienced VATS surgeons were randomly selected for participation. A custom Internet interface permitted anonymous voting, commenting, and the ability to modify and propose new components...
April 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28267085/hemolytic-anemia-due-to-right-ventricular-to-pulmonary-artery-conduit-stenosis
#14
Sudha Rao, Julie A Creaden, Shunyou Gong, Cynthia Rigsby, John M Costello
Hemolytic anemia is a well-recognized complication in patients with left-sided mechanical heart valves. It is rare to see hemolysis with a bioprosthetic valve in the right ventricular outflow tract. We report a 4-year-old-girl with history of truncus arteriosus status-post repair who developed hemolytic anemia as a result of a calcified and obstructed bioprosthetic right ventricular to pulmonary artery-valved conduit. The hemolysis was alleviated by replacing the obstructed conduit with a larger valved conduit...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28246513/variations-in-the-origin-of-inferior-phrenic-arteries-and-their-relationship-to-celiac-axis-variations-on-ct-angiography
#15
Ramazan Aslaner, Yeliz Pekcevik, Hilal Sahin, Onur Toka
OBJECTIVE: Knowing the origin of the inferior phrenic artery (IPA) is important prior to surgical interventions and interventional radiological procedures related to IPA. We aimed to identify variations in the origin of IPA and to investigate the relationship between the origin of IPA and celiac axis variations using computed tomography angiography (CTA). MATERIALS AND METHODS: The CTA images of 1000 patients (737 male and 263 female, the mean age 60, range 18-94 years) were reviewed in an analysis of IPA and celiac axis variations...
March 2017: Korean Journal of Radiology: Official Journal of the Korean Radiological Society
https://www.readbyqxmd.com/read/28219544/the-society-of-thoracic-surgeons-congenital-heart-surgery-database-2017-update-on-outcomes-and-quality
#16
Jeffrey P Jacobs, John E Mayer, Constantine Mavroudis, Sean M O'Brien, Erle H Austin, Sara K Pasquali, Kevin D Hill, David M Overman, James D St Louis, Tara Karamlou, Christian Pizarro, Jennifer C Hirsch-Romano, Donna McDonald, Jane M Han, Susan Becker, Christo I Tchervenkov, Francois Lacour-Gayet, Carl L Backer, Charles D Fraser, James S Tweddell, Martin J Elliott, Hal Walters, Richard A Jonas, Richard L Prager, David M Shahian, Marshall L Jacobs
The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28208970/isolated-hypoplasia-of-left-pulmonary-artery-with-agenesis-of-left-lobe-of-thyroid-a-case-report
#17
Mohammed Abdul Khadir, Ganesh Narayana, Ganavi Ramagopal, Pradeep G Nayar
Isolated Unilateral hypoplasia or agenesis of a branch of pulmonary artery is very rare. It is usually seen associated with congenital heart diseases such as tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right aortic arch, truncus arteriosus, patent ductus arteriosus and pulmonary atresia. It occurs as a result of lack of embryological development of either the left or right sixth aortic arch and has been found to present itself with various clinical manifestations as during childhood it presents as contralateral pulmonary hypertension and in adults as haemoptysis...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28121517/intramural-coronary-artery-in-truncus-arteriosus
#18
Arshid Mir, Harold M Burkhart, Kumar Ponniah, Kent Ward
Coronary artery anomalies are known to be associated with truncus arteriosus (common arterial trunk). Delineation of coronary anatomy preoperatively is important to avoid complications intraoperatively and postoperatively. We report the case of a 12-year-old boy with repaired truncus arteriosus who presented with moderate to severe truncal valve regurgitation and severe conduit stenosis. He was noted to have intramural left main coronary artery on a transesophageal echocardiogram performed preoperatively.
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28043469/the-initial-glimpse-at-long-term-outcomes-following-the-repair-of-truncus-arteriosus
#19
EDITORIAL
George M Alfieris, Michael F Swartz
The article by Naimo and colleagues (outcomes of truncus arteriosus repair in children: 35 years of experience from a single center) provides the initial glimpse at long-term outcomes following truncus arteriosus repair. Although survival has improved over the past decade, the morbidity resulting from subsequent re-operations now comprises a significant area for improvement in the ensuing decade.
2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28043468/outcomes-of-truncus-arteriosus-repair-in-children-35-years-of-experience-from-a-single-institution
#20
Phillip S Naimo, Tyson A Fricke, Matthew S Yong, Yves d'Udekem, Andrew Kelly, Dorothy J Radford, Andrew Bullock, Robert G Weintraub, Christian P Brizard, Igor E Konstantinov
We evaluated the long-term outcomes following repair of truncus arteriosus (TA) from a single institution. We conducted a retrospective review of children (n = 171) who underwent TA repair between 1979 and 2014. Early mortality rate was 11.7% (20/171). There were 19 late deaths. Most deaths (74%, 29/39) occurred within the first year following surgery. The 1-year mortality rate in 1979-2004 was 18% (25/136) and decreased to 11% (4/35) in 2005-2014. The overall survival rate was 73.6% at 30 years. Multivariate analysis identified postoperative extracorporeal membrane oxygenation (P = 0...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
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