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congenital cardiac defects

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https://www.readbyqxmd.com/read/27909479/atrial-fibrillation-ablation-in-adults-with-repaired-congenital-heart-disease
#1
REVIEW
Marta Acena Md, Ignasi Anguera Md PhD, Paolo D Dallaglio Md, Marcos Rodriguez Md, Xavier Sabaté Md PhD
The incidence of atrial fibrillation (AF) in congenital heart disease (CHD) adults has increased in the past decades due to a longer life expectancy of this population where the subjects are exposed to cardiac overflow, overpressure and structural changes for years. The literature regarding AF ablation in repaired CHD adults emphasizes the importance of intracardiac echocardiography (ICE) to perform the transseptal puncture and the ablation procedure in the left atrium (LA), both effectively and safely. In small case control studies, where the predominant congenital cardiomyopathy was the atrial septal defect, the most common strategy for ablation was antral isolation of the pulmonary veins showing results, at one year follow-up, similar to those in the general population...
February 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27906085/tissue-engineered-cardiac-patch-seeded-with-human-induced-pluripotent-stem-cell-derived-cardiomyocytes-promoted-the-regeneration-of-host-cardiomyocytes-in-a-rat-model
#2
Tadahisa Sugiura, Narutoshi Hibino, Christopher K Breuer, Toshiharu Shinoka
BACKGROUND: Thousands of babies are born with congenital heart defects that require surgical repair involving a prosthetic implant. Lack of growth in prosthetic grafts is especially detrimental in pediatric surgery. Cell seeded biodegradable tissue engineered grafts are a novel solution to this problem. The purpose of the present study is to evaluate the feasibility of seeding human induced pluripotent stem cell derived cardiomyocytes (hiPS-CMs) onto a biodegradable cardiac patch. METHODS: The hiPS-CMs were cultured on a biodegradable patch composed of a polyglycolic acid (PGA) and a 50:50 poly (l-lactic-co-ε-caprolactone) copolymer (PLCL) for 1 week...
December 1, 2016: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/27904843/cardiac-surgery-in-africa-a-thirty-five-year-experience-on-open-heart-surgery-in-cote-d-ivoire
#3
Koffi Herve Yangni-Angate, Christophe Meneas, Florent Diby, Manga Diomande, Anicet Adoubi, Yves Tanauh
BACKGROUND: Few centers for open heart surgery (OHS) are in Sub-Saharan Africa. Lack of OHS results is also noted. By reporting our African experience on OHS, the aim of this study was to fill the gap. METHODS: It is a retrospective study on 2,612 patients who were subject to an OHS between 1978 and 2013. Data were collected from demographical, clinical, investigative studies, surgical and outcomes parameters. RESULTS: There were 1,475 cases of rheumatic heart diseases (RHD), 126 endomyocardial fibrosis (EMF), 741 congenital heart diseases (CHDs) and 270 various affections...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27899864/right-ventricle-mimics-right-atrium-at-first-glance-a-rare-case-of-congenital-right-sided-partial-pericardial-defect
#4
Meitzu Wang, Tien-Yu Chang, Wei-Hsian Yin, Yung-Nien Yang
Among heart irregularities, congenital pericardial defect is an unusual anomaly, and is typically left dominant. However, cases of right pericardial defect with heart herniation are extremely rare. This is a case of congenital right pericardial defect with herniation of the right ventricle free wall and right ventricular outflow tract. The patient is asymptomatic and refused further intervention but even indolent discomfort underscores the risks of iatrogenic injuries to the heart and sudden death caused by mechanical pathogenesis due to changes in anatomical positions of the cardiac structures...
November 2016: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/27893194/incidence-morphology-and-progression-of-bicuspid-aortic-valve-in-pediatric-and-young-adult-subjects-with-coexisting-congenital-heart-defects
#5
Talha Niaz, Joseph T Poterucha, Jonathan N Johnson, Cecilia Craviari, Thomas Nienaber, Jared Palfreeman, Frank Cetta, Donald J Hagler
BACKGROUND: Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV. METHODS: The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015...
November 28, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27885814/trends-microbiology-and-outcomes-of-infective-endocarditis-in-children-during-2000-2010-in-the-united-states
#6
Shipra Gupta, Ankit Sakhuja, Eric McGrath, Basim Asmar
BACKGROUND: We studied the incidence, trend, underlying conditions, microbiology, and outcomes of infective endocarditis (IE) in children during 11 years using Nationwide Inpatient Sample (NIS) database. This is the largest all-payer inpatient care database in the United States containing data for more than 8 million hospital stays from over 1000 hospitals. METHODS: NIS data from 2000 to 2010 of primary discharge diagnosis of IE in children aged ≤19 years old were studied...
November 25, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27876287/mid-term-outcomes-of-common-congenital-heart-defects-corrected-through-a-right-subaxillary-thoracotomy
#7
Guoying An, Hongyu Zhang, Shanguang Zheng, Weixin Wang, Liming Ma
OBJECTIVE: To evaluate the mid-term outcomes of the correction of various congenital heart defects through a right subaxillary thoracotomy. METHODS: Between June 2004 and April 2014, all eligible patients were those with a common congenital heart defect corrected via an approach through a right subaxillary thoracotomy. There were 836 patients (male 417, female 419; median age, 3.5 years; median weight 13.6kg) with ventricular septal defect (VSD) closure (523 patients), atrial septal defect (ASD) closure (235 patients), partial atrioventricular canal (PAVC) correction (55 patients), mild tetralogy of Fallot (TOF) (23 patients)...
February 17, 2016: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/27872050/experimental-systems-and-computational-approaches-to-understanding-the-microrna-mediated-reparative-potential-of-cardiac-progenitor-cell-derived-exosomes-from-pediatric-patients
#8
Udit Agarwal, Alex George, Srishti Bhutani, Shohini Ghosh-Choudhary, Josh T Maxwell, Milton E Brown, Yash Mehta, Manu O Platt, Yaxuan Liang, Susmita Sahoo, Michael E Davis
RATIONALE: Studies have demonstrated that exosomes can repair cardiac tissue post myocardial infarction (MI) and recapitulate the benefits of cellular therapy. OBJECTIVE: We evaluated the role of donor age and hypoxia of human pediatric cardiac progenitor cell (CPC)-derived exosomes, in a rat model of ischemia reperfusion (IR) injury. METHODS AND RESULTS: Human CPCs from the right atrial appendages from children of different ages undergoing cardiac surgery for congenital heart defects were isolated and cultured under hypoxic or normoxic conditions...
November 21, 2016: Circulation Research
https://www.readbyqxmd.com/read/27866675/hemodynamics-of-patient-specific-aorta-pulmonary-shunt-configurations
#9
Senol Piskin, H Firat Altin, Okan Yildiz, Ihsan Bakir, Kerem Pekkan
Optimal hemodynamics in aorta-pulmonary shunt reconstruction is essential for improved post-operative recovery of the newborn congenital heart disease patient. However, prior to in vivo execution, the prediction of post-operative hemodynamics is extremely challenging due to the interplay of multiple confounding physiological factors. It is hypothesized that the post-operative performance of the surgical shunt can be predicted through computational blood flow simulations that consider patient size, shunt configuration, cardiac output and the complex three-dimensional disease anatomy...
November 11, 2016: Journal of Biomechanics
https://www.readbyqxmd.com/read/27864598/impact-of-fetal-counseling-on-outcome-of-antenatal-congenital-surgical-anomalies
#10
Shilpa Sharma, Ranjana Bhanot, Dipika Deka, Minu Bajpai, Devendra K Gupta
AIM: To analyze the impact of counseling on antenatal congenital surgical anomalies (ACSA). METHODS: Cases presenting with ACSA for fetal counseling and those presenting in post-natal period following diagnosis of ACSA (PACSA) for surgical opinion were analyzed for spectrum, presentation and outcome. RESULTS: 117 cases including ACSA(68);PACSA(49) were analyzed. Gestational age at diagnosis of ACSA;PACSA was 17-37;17-39 weeks (median 24;32 weeks)...
November 18, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27861436/the-association-between-cyanosis-and-thromboelastometry-rotem-in-children-with-congenital-heart-defects-a-retrospective-cohort-study
#11
Marie-Laure Laskine-Holland, Walter H A Kahr, Lynn Crawford-Lean, Tilman Humpl, Osami Honjo, Celeste Foreman, Mehr Jain, James D O'Leary
BACKGROUND: Children with congenital heart defects (CHD) have quantitative and qualitative differences in coagulation compared with healthy children. Secondary to polycythemia and increased deformability of red blood cells, cyanosis may be an important confounding factor for altered whole-blood coagulation in this population with potential implications for interpreting intraoperative thromboelastometry (TEM) for children with CHD undergoing major surgery. The primary aim of the study was to evaluate the association between cyanosis in children with CHD and measures of whole-blood coagulation determined using TEM (ROTEM [Tem International, GmbH, Munich, Germany])...
November 17, 2016: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/27853695/aplasia-cutis-congenita-of-the-scalp-with-a-familial-pattern-a-case-report
#12
Waleed AlShehri, Sara AlFadil, Alhanouf AlOthri, Abdulaziz O Alabdulkarim, Shabeer A Wani, Sari M Rabah
Aplasia Cutis Conginita (ACC) is a condition characterized by congenital absence of skin, usually on the scalp. ACC can occur as an isolated condition or in the presence of other congenital anomalies. Here we describe a case of a 16 days old baby girl with an isolated ACC of the scalp. Her elder two siblings have been diagnosed with ACC with concomitant cardiac or limb anomalies. The patient was managed conservatively until the defect has scarred 6 months later.
September 2016: World Journal of Plastic Surgery
https://www.readbyqxmd.com/read/27837304/utility-and-scope-of-rapid-prototyping-in-patients-with-complex-muscular-ventricular-septal-defects-or-double-outlet-right-ventricle-does-it-alter-management-decisions
#13
Puneet Bhatla, Justin T Tretter, Achi Ludomirsky, Michael Argilla, Larry A Latson, Sujata Chakravarti, Piers C Barker, Shi-Joon Yoo, Doff B McElhinney, Nicole Wake, Ralph S Mosca
Rapid prototyping facilitates comprehension of complex cardiac anatomy. However, determining when this additional information proves instrumental in patient management remains a challenge. We describe our experience with patient-specific anatomic models created using rapid prototyping from various imaging modalities, suggesting their utility in surgical and interventional planning in congenital heart disease (CHD). Virtual and physical 3-dimensional (3D) models were generated from CT or MRI data, using commercially available software for patients with complex muscular ventricular septal defects (CMVSD) and double-outlet right ventricle (DORV)...
November 11, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27837301/early-complications-after-percutaneous-closure-of-atrial-septal-defect-in-infants-with-procedural-weight-less-than-15-kg
#14
Gustaf Tanghöj, Michal Odermarsky, Estelle Naumburg, Petru Liuba
Atrial septal defect (ASD) is the most common congenital cardiac lesion accounting for 10-15% of all cardiac malformations. In the majority of cases, the secundum type of the ASD is closed percutaneously in the catheterization laboratory. Although transcatheter closure of ASD is considered safe and effective in pediatric patients, there are limited data regarding the efficacy and safety of device ASD closure in smaller infants. The aim of this study was to determine risk of complications within 72 h following device closure of ASD in children of body weight <15 kg compared to larger children...
November 11, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27836357/does-the-ex-utero-intrapartum-treatment-to-extracorporeal-membrane-oxygenation-procedure-change-morbidity-outcomes-for-high-risk-congenital-diaphragmatic-hernia-survivors
#15
Hester F Shieh, Jay M Wilson, Catherine A Sheils, C Jason Smithers, Virginia S Kharasch, Ronald E Becker, Mollie Studley, Donna Morash, Terry L Buchmiller
PURPOSE: In high-risk congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before extracorporeal membrane oxygenation (ECMO) can be initiated. We previously examined ex utero intrapartum treatment (EXIT)-to-ECMO in our most severe CDH patients, but demonstrated no survival advantage. We now report morbidity outcomes in survivors of this high-risk cohort to determine whether EXIT-to-ECMO conferred any benefit. METHODS: All CDH survivors with <15% predicted lung volume (PPLV) from September 1999 to December 2010 were included...
October 25, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27834760/surgical-treatment-for-double-outlet-right-ventricle-with-pulmonary-outflow-tract-obstruction
#16
Qingyu Wu, Yongqiang Jin, Hongyin Li, Mingkui Zhang
BACKGROUND: Double outlet right ventricle (DORV) is a conotruncal anomaly that is a defining element of many types of complex congenital heart disease. Because of a big variety of pathology, there are still some controversies with respect to the definition, classification, and surgical treatment. We report our experience with surgical treatment for DORV (as defined by the "90% rule") with pulmonary outflow tract obstruction (POTO). METHODS: From July 2005 to July 2015, 90 patients underwent surgical treatment of DORV with POTO at the First Hospital of Tsinghua University...
November 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27829775/ellis-van-creveld-syndrome-in-siblings-a-rare-case-report
#17
Sabitha Gokulraj, N Mohan, J Babususai Raj, S Yasmeen Ahamed, C J Stephen Arokiaraj, A Cicilia Subbulakshmi
Ellis-Van Creveld syndrome or chondroectodermal dysplasia is a rare autosomal recessive disorder presenting several skeletal manifestations and congenital heart malformations. Ellis-Van Creveld syndrome comprises of a tetrad of clinical manifestations of chondrodysplasia, polydactyly, ectodermal dysplasia, and cardiac defects. Here, we are presenting a very rare case of Ellis-Van Creveld syndrome in siblings.
October 2016: Journal of Pharmacy & Bioallied Sciences
https://www.readbyqxmd.com/read/27828833/percutaneous-management-of-failed-bioprosthetic-pulmonary-valves-in-patients-with-congenital-heart-defects
#18
Pierpaolo Tarzia, Erika Conforti, Alessandro Giamberti, Alessandro Varrica, Luca Giugno, Angelo Micheletti, Diana Negura, Luciane Piazza, Antonio Saracino, Mario Carminati, Massimo Chessa
AIMS: We reviewed our center experience in the field of transcatheter pulmonary valve-in-valve implantation (TPViV), that is emerging as a treatment option for patients with pulmonary bioprosthetic valve (BPV) dysfunction. METHODS: Between April 2008 and September 2015, a total of six patients with congenital heart disease (four men) underwent TPViV due to stenosis of preexisting BPV. Four patients received a Melody Medtronic Transcatheter Pulmonary Valve and two an Edward Sapien Valve...
November 8, 2016: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/27814918/four-tiered-echocardiographic-analysis-approach-for-congenital-mitral-valve-malformations-four-years-of-experience
#19
Feifei Sun, Yixin Chen, Weidong Ren, Ying Zhang, Dan Wu, Xin Chen, Chunyan Ma, Dongyu Li
BACKGROUND: Traditional methods of describing and classifying congenital mitral valve malformations (CMVMs) often lack specificity and scientificity. Thus, documentation is incomplete, especially in terms of ultrasound findings. METHODS: Data were collected from 436 patients (mean age, 36.6±26.8years; male 47.9%), each subjected to echocardiographic evaluation of CMVM. Valvar characteristics were studied and analyzed via a four-tiered echocardiographic analysis (FTEA) approach: (1) supravalvular region and annulus, (2) valvar leaflets and commissures, (3) chordae tendineae, and (4) papillary muscles...
October 29, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27809680/in-vivo-functional-consequences-of-human-thra-variants-expressed-in-the-zebrafish
#20
Federica Marelli, Silvia Carra, Giuditta Rurale, Franco Cotelli, Luca Persani
Heterozygous mutations in the thyroid hormone receptor alpha (THRA) gene cause Resistance to Thyroid Hormone alpha (RTHα), a disease characterized by variable manifestations reminiscent of untreated congenital hypothyroidism but a raised T3/T4 ratio and normal TSH levels. We recently described that zebrafish embryos expressing a dominant negative (DN) form of thraa recapitulate the key features of RTHα, and that zebrafish and human receptors are functionally interchangeable. In this study we expressed several human TRα variants in zebrafish embryos and analyzed the resulting phenotypes...
November 3, 2016: Thyroid: Official Journal of the American Thyroid Association
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