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https://www.readbyqxmd.com/read/29242651/perioperative-sildenafil-therapy-for-children-with-ventricular-septal-defects-and-associated-pulmonary-hypertension-undergoing-corrective-surgery-a-randomised-clinical-trial
#1
Sidharth Bhasin, Pooja Gogia, Rajeev Nair, Tapan Kumar Sahoo
Background and Aims: Sildenafil is known to reduce pulmonary artery pressure but its role in the perioperative period has not been well studied. We aimed to evaluate the efficacy of sildenafil in controlling post-operative pulmonary hypertension in children with pulmonary hypertension undergoing surgeries for correction of ventricular septal defect. Methods: The patients were divided randomly into two groups of thirty each. Group 1 (placebo) received pre-operative placebo and post-operative sildenafil (0...
October 2017: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/29240627/extracorporeal-membrane-oxygenation-bridges-inoperable-patients-to-definitive-cardiac-operation
#2
Nikola Dobrilovic, Omar Lateef, Lauren Michalak, Maja Delibasic, Jaishankar Raman
Extracorporeal membrane oxygenation (ECMO) offers an opportunity for patient recovery through complete cardiopulmonary support but is associated with complications that limit duration and overall utility. We examine the role of ECMO as a potential bridge to high-risk cardiac surgery in otherwise inoperable cases. This study reports a retrospective, multi-institution experience examining all patients for whom ECMO was used preoperatively as a bridge to definitive cardiac surgery without exception. A consecutive patient database (December 2011 through August 2017) was utilized...
December 11, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29237020/extrinsic-compression-of-left-main-coronary-artery-by-aneurysmal-pulmonary-artery-in-severe-pulmonary-hypertension-its-correlates-clinical-impact-and-management-strategies
#3
Ozgur Yasar Akbal, Cihangir Kaymaz, Ibrahim Halil Tanboga, Aykun Hakgor, Fatih Yilmaz, Sevim Turkday, Cem Dogan, Seda Tanyeri, Durmus Demir, Zubeyde Bayram, Mahmut Bugrahan Cicek, Rezzan Deniz Acar, Nihal Ozdemir
Aims: Although left main coronary artery (LMCA) compression (Co) by pulmonary artery (PA) aneurysm (A) has been reported in some pulmonary hypertension (PH) series, clinical importance and management of this complication remain to be determined. In this single-centre prospective study, we evaluated correlates, clinical impact, and management strategies of LMCA-Co in patients with PH. Methods and results: Our study group comprised 269 (female 166, age 52.9 ± 17...
December 8, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/29235722/alterations-in-ventricular-pumping-in-patients-with-atrial-septal-defect-at-rest-during-dobutamine-stress-and-after-defect-closure
#4
Sigurdur S Stephensen, Ellen Ostenfeld, Katarina Steding-Ehrenborg, Ulf Thilén, Einar Heiberg, Hakan Arheden, Marcus Carlsson
BACKGROUND: Regional ventricular pumping mechanisms in patients with volume-loaded right ventricles (RV) are altered, but the cause is unknown. The aim was to determine whether these changes in ventricular pumping mechanisms are influenced by the RV dilatation itself or the aetiology behind it. METHODS: Seventeen patients with atrial septal defects (ASD) and 10 healthy controls underwent cardiovascular magnetic resonance (CMR) at rest and during dobutamine/atropine stress...
December 13, 2017: Clinical Physiology and Functional Imaging
https://www.readbyqxmd.com/read/29233213/transcatheter-closure-of-congenital-perimembranous-ventricular-septal-defect-using-the-amplatzer-duct-occluder-2
#5
Li-Jian Zhao, Bo Han, Jian-Jun Zhang, Ying-Chun Yi, Dian-Dong Jiang, Jian-Li Lyu
OBJECTIVE: The objective of this study was to explore the clinical effect of the transcatheter closure of congenital perimembranous ventricular septal defect using the Amplatzer duct occluder 2. METHODS: Between February 2012 and December 2016, 51 patients were subjected to Amplatzer duct occluder 2 for transcatheter closure of perimembranous ventricular septal defect. A total of 51 patients with perimembranous ventricular septal defect who underwent transcatheter closure by the conventional membranous ventricular septal occluder comprised the control group...
December 13, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/29232303/totally-endoscopic-cardiac-surgery-for-atrial-septal-defect-repair-on-beating-heart-without-robotic-assistance-in-25-patients
#6
Quang-Huy Dang, Ngoc-Thanh Le, Cong-Huu Nguyen, Dac-Dai Tran, Do-Hung Nguyen, Trung-Hieu Nguyen, Thi-Hai-Linh Ngo
OBJECTIVE: The aim of the study was to investigate the effectivity and safety of totally endoscopic cardiac surgery without robotic assistance for atrial septal defect (ASD) closure on beating hearts. METHODS: Twenty-five patients (adults/children: 15/10) underwent ASD closure using nonrobotically assisted totally endoscopic approach on beating heart. Three 5-mm trocars and one 12-mm trocar were used, only the superior vena cava is snared, filling the pleural and pericardial cavities with CO2, and the heart was beating during the surgery...
December 7, 2017: Innovations: Technology and Techniques in Cardiothoracic and Vascular Surgery
https://www.readbyqxmd.com/read/29230261/early-experience-with-open-heart-surgery-in-a-pioneer-private-hospital-in-west-africa-the-biket-medical-centre-experience
#7
Uvie Ufuoma Onakpoya, Adebisi David Adenle, Anthony Taiwo Adenekan
Introduction: More than forty years after the first open heart surgery in Nigeria, all open heart surgeries were carried out in government-owned hospitals before the introduction of such surgeries in 2013 at Biket Medical Centre, a privately owned hospital in Osogbo, South-western Nigeria. The aim of this paper is to review our initial experience with open heart surgery in this private hospital. Methods: All patients who underwent open heart surgery between August 2013 and January 2014 were included in this prospective study...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/29229899/phenotypic-variation-in-patients-with-homozygous-c-1678g-t-mutation-in-evc-gene-report-of-two-mexican-families-with-ellis-van-creveld-syndrome
#8
Marisol Ibarra-Ramirez, Luis Daniel Campos-Acevedo, Jose Lugo-Trampe, Laura E Martínez-Garza, Víctor Martinez-Glez, María Valencia-Benitez, Pablo Lapunzina, Víctor Ruiz-Peréz
BACKGROUND Ellis-van Creveld syndrome is an autosomal recessive chondro-ectodermal dysplasia characterized by disproportionate short stature, limb shortening, narrow chest, postaxial polydactyly and dysplastic nails and teeth. In addition, 60% of cases present congenital heart defects. Ellis-van Creveld syndrome is predominantly caused by mutations in the EVC or EVC2 (4p16) genes, with only a few cases caused by mutations in WDR35.  CASE REPORT Here, we report on two Mexican families with patients diagnosed with Ellis-van Creveld syndrome...
December 12, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/29226900/diffuse-hypoplasia-of-the-aortic-arch-and-isthmus-in-a-patient-with-williams-syndrome
#9
İsmihan Selen Onan, Erkut Öztürk, Aylin Demirel Başgöze, Ayse Çicek, Burak Onan
Williams syndrome is a rare neurodevelopmental disorder characterized by mental retardation, growth deficiency, hypercalcemia, cardiac defects, and a distinctive facial appearance. Cardiovascular abnormalities are present in approximately 80% of Williams syndrome patients. Surgical treatment is generally performed for supravalvular aortic stenosis, aortic coarctation, pulmonary artery stenosis, or ventricular septal defect. In rare cases, diffuse hypoplasia of the aortic arch with a normal left ventricular outflow tract and ascending aorta may be diagnosed in early childhood...
December 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/29226520/a-novel-homozygous-slc25a1-mutation-with-impaired-mitochondrial-complex-v-possible-phenotypic-expansion
#10
Idan Cohen, Orna Staretz-Chacham, Ohad Wormser, Yonatan Perez, Ann Saada, Rotem Kadir, Ohad S Birk
SLC25A1 mutations are associated with combined D,L-2-hydroxyglutaric aciduria (DL- 2HGA; OMIM #615182), characterized by muscular hypotonia, severe neurodevelopmental dysfunction and intractable seizures. SLC25A1 encodes the mitochondrial citrate carrier (CIC), which mediates efflux of the mitochondrial tricarboxylic acid (TCA) cycle intermediates citrate and isocitrate in exchange for cytosolic malate. Only a single family with an SLC25A1 mutation has been described in which mitochondrial respiratory chain dysfunction was documented, specifically in complex IV...
December 11, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29225981/tricuspid-valve-endocarditis-due-to-streptococcus-bovis-in-a-patient-with-ventricular-septal-defect-a-rare-manifestation-case-report-and-review-of-the-literature
#11
J Pushpakumara, G Mudiyanse, A Jayawardana, J Siriwardana, N L A Shyamali, J Indrakumar
Streptococcus bovis endocarditis has 18%-62% association with colonic neoplasms with multivalvular involvement and affects mainly elderly males leading to severe cardiac dysfunction, septic embolization, and neurological complications. The aortic valve is the commonest valve to be affected followed by aortic and mitral valves together. However, involvement of tricuspid valve is extremely rare. There are no reported cases of Streptococcus bovis endocarditis affecting the tricuspid valve in the presence of ventricular septal defect with left to right shunt...
2017: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/29224925/incidence-and-clinical-characteristics-of-sudden-cardiac-death-in-adult-congenital-heart-disease
#12
Benjamin Moore, Christopher Yu, Irina Kotchetkova, Rachael Cordina, David S Celermajer
BACKGROUND: The life expectancy of adults with congenital heart disease (CHD) has significantly improved in recent decades, with non-cardiovascular causes of death now competing with traditional cardiovascular causes. The risk of sudden cardiac death (SCD), a devastating event, still remains elevated above that of the general population. METHODS: We reviewed 2935 patients in our adult CHD database (age≥16years, seen at least once in our centre) and documented all cases of SCD between 2000-2015...
December 5, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29224761/direct-tracheobronchopexy-via-left-lateral-thoracotomy-for-severe-tracheobronchomalacia
#13
Evan J Propst, Faisal Zawawi, Roxanne E Kirsch, Osami Honjo
An infant with pulmonary atresia/ventricular septal defect/major aortopulmonary collateral arteries underwent unifocalization, ventriculoseptal defect closure and placement of a right ventricle to pulmonary artery conduit via median sternotomy. Aortopexy and pulmonary arteriopexy via redo sternotomy were insufficient to allow weaning of continuous positive airway pressure and he required direct tracheobronchopexy via left lateral thoracotomy to alleviate posterior trachealis intrusion along the length of the trachea and left main bronchus...
December 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29224269/-surgical-treatment-and-early-mid-follow-up-results-of-complete-atrioventricular-septal
#14
H J Cui, J Zhuang, J M Chen, J Z Cen, G Xu, S S Wen
Objective: To discuss the surgical treatment and early-mid follow-up results of complete atrioventricular septal defect (CAVSD). Methods: The clinic data of 235 cases of CAVSD underwent surgical treatment at Department of Cardiac Surgery, Guangdong General Hospital from June 2009 to June 2016 was analyzed retrospectively (104 male and 131 female patients). The patients were aged 18 days to 58 years (mid age 8.9 months) at surgery, and 2 cases of newborns (0.9%), 24 cases of 1 to 3 months (10.2%), 56 cases of 4 to 6 months (23...
December 1, 2017: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/29222010/a-novel-nr2f2-loss-of-function-mutation-predisposes-to-congenital-heart-defect
#15
Xiao-Hui Qiao, Qian Wang, Juan Wang, Xing-Yuan Liu, Ying-Jia Xu, Ri-Tai Huang, Song Xue, Yan-Jie Li, Min Zhang, Xin-Kai Qu, Ruo-Gu Li, Xing-Biao Qiu, Yi-Qing Yang
Congenital heart defect (CHD) is the most common type of birth defect in humans and a leading cause of infant morbidity and mortality. Previous studies have demonstrated that genetic defects play a pivotal role in the pathogenesis of CHD. However, the genetic basis of CHD remains poorly understood due to substantial genetic heterogeneity. In this study, the coding exons and splicing boundaries of the NR2F2 gene, which encodes a pleiotropic transcription factor required for normal cardiovascular development, were sequenced in 168 unrelated patients with CHD, and a novel mutation (c...
December 5, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29221351/extracorporeal-membrane-oxygenation-system-as-a-bridge-to-reparative-surgery-in-ventricular-septal-defect-complicating-acute-inferoposterior-myocardial-infarction
#16
Jose Rozado, Isaac Pascual, Pablo Avanzas, Daniel Hernandez-Vaquero, Ruben Alvarez, Rocio Díaz, Beatriz Díaz, María Martín, Amelia Carro, Guillermo Muñiz, Jacobo Silva, Cesar Moris
Post-infarction ventricular septal defect (VSD) is a rare but potentially lethal complication of acute myocardial infarction. Medical management is usually futile, so definitive surgery remains the treatment of choice but the risk surgery is very high and the optimal timing for surgery is still under debate. A 55-year-old man with no previous medical history attended the emergency-room for 12 h evolution of oppressive chest pain and strong anginal pain 7 days ago. On physical examination, blood pressure was 96/70 mmHg, pansystolic murmur over left sternal border without pulmonary crackles...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29218069/down-syndrome-genetics-and-cardiogenetics
#17
Vasilica Plaiasu
During the last years, Down syndrome has been the focus of special attention. Down syndrome is a genetic disorder characterized by distinct physical features and some degree of cognitive disability. Patients with Down syndrome also present many other congenital anomalies. The mapping for phenotypes to specific regions of chromosome 21 permits to identify which genes (or small regions) contribute to the phenotypic features of Down syndrome and thus, to understand its pathogenesis. Mainly there are three cytogenetic forms of Down syndrome: free trisomy 21, mosaic trisomy 21 and robertsonian translocation trisomy 21...
September 2017: Mædica
https://www.readbyqxmd.com/read/29216945/-expression-and-meaning-of-t-bet-and-gata3-mrna-in-t-lymphocyte-of-patients-during-the-operation-with-cardiopulmonary-bypass
#18
Haifeng Ni, Yingbin Xiao
OBJECTIVE: To investigate the effects of cardiopulmonary bypass (CPB) on the differentiation of T lymphocyte subsets and the expression of specific transcription regulator T-bet/GATA binding protein 3 (GATA3). METHODS: A prospective double-blind study was conducted. Patients with CPB pulmonary repair of ventricular septal defect (observation group) or off-pump ligation of ductus arteriosus (control group) with 20 cases each in the 150th Military Hospital from February 2015 to February 2016 were enrolled...
December 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
https://www.readbyqxmd.com/read/29216221/copy-number-variants-in-ebstein-anomaly
#19
Andreas Giannakou, Robert J Sicko, Wei Zhang, Paul Romitti, Marilyn L Browne, Michele Caggana, Lawrence C Brody, Laura Jelliffe-Pawlowski, Gary M Shaw, Denise M Kay, James L Mills
BACKGROUND: Ebstein anomaly (EA) is a rare congenital defect characterized by apical displacement of the septal tricuspid leaflets and atrialization of the right ventricle. The etiology of EA is unclear; however, recurrence in families and the association of EA with genetic syndromes and copy number variants (CNVs) suggest a genetic component. OBJECTIVE: We performed a population-based study to search for recurrent and novel CNVs in a previously unreported set of EA cases...
2017: PloS One
https://www.readbyqxmd.com/read/29214833/living-with-functionally-univentricular-heart-beating-the-odds
#20
Safak Alpat, Metin Demircin
An unoperated 45-year-old man with double-inlet single left ventricle, transposed great arteries, ventricular septal defect, and pulmonary stenosis presented with mitral and pulmonary valvular vegetations unresponsive to antibiotic therapy. The initial diagnosis was made with echocardiography, and the morphology was delineated with computed tomography and magnetic resonance imaging. The patient underwent mitral valve replacement with a mechanical valve and pulmonary valve vegetectomy. He recovered well and he is currently well two years after the operation...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
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