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https://www.readbyqxmd.com/read/28732025/hand-factor-ablation-causes-defective-left-ventricular-chamber-development-and-compromised-adult-cardiac-function
#1
Joshua W Vincentz, Kevin P Toolan, Wenjun Zhang, Anthony B Firulli
Coordinated cardiomyocyte growth, differentiation, and morphogenesis are essential for heart formation. We demonstrate that the bHLH transcription factors Hand1 and Hand2 play critical regulatory roles for left ventricle (LV) cardiomyocyte proliferation and morphogenesis. Using an LV-specific Cre allele (Hand1LV-Cre), we ablate Hand1-lineage cardiomyocytes, revealing that DTA-mediated cardiomyocyte death results in a hypoplastic LV by E10.5. Once Hand1-linage cells are removed from the LV, and Hand1 expression is switched off, embryonic hearts recover by E16...
July 21, 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28728260/-clinical-analysis-of-pacing-therapy-and-treatment-of-complications-during-follow-up-in-children
#2
P J Zhao, Y W Chen, F Li, Y Li, J P Yang, J J Wu
Objective: To analyze characteristics of permanent pacemaker implantation in children as well as treatment and prognosis of complications. Method: Clinical data of children who underwent endocardial or epicardial permanent pacemaker implantation between April 2000 and June 2016 in Shanghai Xinhua Hospital and Shanghai Children's Medical Center were analyzed retrospectively. These patients were discharged 5 days after implantation. Electrocardiogram, chest X ray, echocardiography and pacemaker programming were performed during follow-up...
July 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28725996/improvement-of-tricuspid-regurgitation-after-transcatheter-asd-closure-in-older-patients
#3
L Chen, J Shen, X Shan, F Wang, T Kan, X Tang, X Zhao, Y Qin
BACKGROUND: Adult patients with undiagnosed atrial septal defect (ASD) may have right heart cavity enlargement and functional tricuspid valve insufficiency. Moderate or more severe tricuspid regurgitation has been associated with a worse prognosis, and more serious complications are typically seen in older patients. This study aimed to evaluate the improvement in functional tricuspid regurgitation and heart geometry after transcatheter ASD closure in older patients. PATIENTS AND METHODS: The data of 111 patients over 60 years of age with moderate or severe tricuspid regurgitation before ASD closure were analyzed...
July 19, 2017: Herz
https://www.readbyqxmd.com/read/28723882/altered-carnitine-homeostasis-in-children-with-increased-pulmonary-blood-flow-due-to-ventricular-septal-defects
#4
Stephen M Black, Aida Field-Ridley, Shruti Sharma, Sanjiv Kumar, Roberta L Keller, Rebecca Kameny, Emin Maltepe, Sanjeev A Datar, Jeffrey R Fineman
OBJECTIVES: Congenital heart disease with increased pulmonary blood flow results in progressive pulmonary vascular endothelial dysfunction and associated increased perioperative morbidity. Using our ovine model of congenital heart disease with increased pulmonary blood flow, we have previously demonstrated progressive endothelial dysfunction associated with disruption in carnitine homeostasis, mitochondrial dysfunction, decreased nitric oxide signaling, and enhanced reactive oxygen species generation...
July 18, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28722171/use-of-antibiotics-during-pregnancy-and-the-risk-of-major-congenital-malformations-a-population-based-cohort-study
#5
Flory T Muanda, Odile Sheehy, Anick Bérard
INTRODUCTION: Few studies have investigated the link between individual antibiotics and major congenital malformations (MCMs) including specific malformations owing to small sample size. We aimed to quantify the association between exposure to gestational antibiotic and the risk of MCMs. METHODS: Using the Quebec pregnancy cohort (1998 -2008), we included a total of 139,938 liveborn singleton alive whose mothers were covered by the «Régie de l'assurance maladie du Québec" drug plan for at least 12 months before and during pregnancy...
July 19, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28721547/the-effect-on-somatic-growth-of-surgical-and-catheter-treatment-of-secundum-atrial-septal-defects
#6
Meghan M Chlebowski, Hongying Dai, Stephen F Kaine
Historical studies suggest an association between atrial septal defect (ASD) and impaired growth with inconsistent improvement following closure. Limited data exist regarding the impact on growth in the era of transcatheter therapy. To evaluate the effect of closure on growth, we conducted a retrospective review of patients undergoing surgical or transcatheter closure during two time periods. Four hundred patients with isolated secundum ASD were divided into three cohorts: early surgical, contemporary surgical, and transcatheter...
July 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28719142/changing-prevalence-of-severe-congenital-heart-disease-results-from-the-national-register-for-congenital-heart-defects-in-germany
#7
Constanze Pfitzer, Paul C Helm, Hannah Ferentzi, Lisa-Maria Rosenthal, Ulrike M M Bauer, Felix Berger, Katharina R L Schmitt
OBJECTIVE: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender. DESIGN: Cross-sectional registry study. SETTING: We analyzed data from patients with CHD born between 1996 and 2015. PATIENTS: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic capability for earlier identifying minor lesions)...
July 18, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28713619/functional-and-clinical-importance-of-a-large-sized-ostium-secundum-defect-in-a-middle-aged-female-cadaver-a-case-report
#8
Satheesha B Nayak
Atrial septal defect (ASD) is one of the common congenital anomalies of the heart in humans. Its complications depend on the size of the defect and can manifest at any age. The common symptoms of ASD include dyspnea and fatigue. Most of the ASDs are associated with morbidity and mortality, Earlier the treatment, it is better to the patient. I saw a large ostium secundum defect in the heart of an adult female cadaver during dissection classes for undergraduate medical students. The interatrial septum had large defect at the region where fossa ovalis should have been located...
June 2017: Anatomy & Cell Biology
https://www.readbyqxmd.com/read/28711966/modified-single-patch-technique-versus-two-patch-technique-for-the-repair-of-complete-atrioventricular-septal-defect-a-meta-analysis
#9
Dongxu Li, Qiang Fan, Tomoyuki Iwase, Yasutaka Hirata, Qi An
Technical selection for surgical repair of complete atrioventricular septal defect (CAVSD) still remains controversial. This meta-analysis aimed to compare the modified single-patch (MP) technique with the two-patch (TP) technique for patients with CAVSD. Relevant studies comparing the MP technique with the TP technique were identified through a literature search using MEDLINE, EMBASE, Google Scholar, Cochrane Library, and the China National Knowledge Infrastructure databases. The variables were ventricular septal defect (VSD) size, cardiopulmonary bypass (CBP) time, aortic cross-clamp (ACC) time, intensive care unit stay, hospital stay, and other outcomes involving mortality, left ventricular outflow tract obstruction, atrioventricular valve regurgitation, residual septal shunt, atrioventricular block, and reoperation...
July 15, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28711543/clival-defect-with-primary-csf-rhinorrhea-a-very-rare-presentation-with-challenging-management
#10
Sheikh Asad, Jens Peters-Willke, Warwick Brennan, Saima Asad
Primary spontaneous cerebrospinal fluid (CSF) rhinorrhea due to clival defect is an extremely rare presentation and only six cases have been reported in the literature so far(17). We present a unique case of a 64-year-old lady who presented with 2 years history of ongoing CSF rhinorrhea. She had similar episode at the age of 40 and suffered from meningitis. She had MRI, CT cisternogram as well as CT of brain and that showed clival defect with CSF leak into the sphenoid sinus. She underwent trans-sphenoidal repair of the clival defect with concurrent lumbar drain insertion for CSF drainage...
July 12, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28711428/-congenital-heart-disease-mortality-in-spain-during-a-10-year-period-2003-2012
#11
Javier Pérez-Lescure Picarzo, Margarita Mosquera González, Pello Latasa Zamalloa, David Crespo Marcos
INTRODUCTION AND OBJECTIVES: Congenital heart disease is a major cause of infant mortality in developed countries. In Spain, there are no publications at national level on mortality due to congenital heart disease. The aim of this study is to analyse mortality in infants with congenital heart disease, lethality of different types of congenital heart disease, and their variation over a ten-year period. METHODS: A retrospective observational study was performed to evaluate mortality rate of children under one year old with congenital heart disease, using the minimum basic data set, from 2003 to 2012...
July 12, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28709918/unroofed-coronary-sinus-syndrome-an-easily-corrected-congenital-anomaly-but-more-diagnostic-suspicions-are-needed
#12
Changcheng Chen, Lili Xu, Yi Xu, Ping Li, Shuo Liu, Bin You
BACKGROUND: Unroofed coronary sinus syndrome (URCSS) is a spectrum of cardiac anomalies in which part (partial type) or the entire common wall (complete type) between the coronary sinus (CS) and left atrium is absent. It is commonly associated with a persistent left superior vena cava (PLSVC). The PLSVC can even anomalously connect to the left atrium in complete type anomaly. URCSS has been reported to be associated with delayed diagnosis and life-threatening cerebral injury. The purpose of the present study was to review our experience with surgical correction of this often-concealed malformation and discuss methods for reducing diagnostic omission...
June 22, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28709589/initial-experience-of-transcatheter-closure-of-subarterial-vsd-with-the-amplatzer-duct-occluder
#13
Tsung-Cheng Shyu, Ming-Chih Lin, Yeak-Wun Quek, Sheng-Je Lin, Hean-Pat Saw, Sheng-Ling Jan, Yun-Ching Fu
BACKGROUND: The traditional treatment of subarterial ventricular septal defect (VSD) is open heart surgery. This study aimed to evaluate the feasibility, safety and outcome of transcatheter closure with the Amplatzer duct occluder (ADO). METHODS: Between March 2012 and June 2015, a total of 16 patients (8 male and 8 female) with subarterial VSD who underwent transcatheter closure with the ADO were enrolled retrospectively. Their age ranged from 3.0 to 65.6 years, with the median of 35...
July 11, 2017: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/28707608/atrial-septal-defect-and-an-unusual-anatomical-variant-of-double-chambered-right-ventricle-presenting-with-cyanosis
#14
Abraham Rothman, Gagandeep Singh, William N Evans
We treated two patients with unexplained cyanosis, an atrial septal defect and an unusual form of non-obstructive double-chambered right ventricle, with device closure of the atrial septal defect.
July 14, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28707408/complete-heart-block-following-transcatheter-closure-of-perimembranous-vsd-using-amplatzer-duct-occluder-ii
#15
Sanjiban Ghosh, Anuradha Sridhar, Muthukumaran Sivaprakasam
Long term follow up studies have shown that transcatheter closure of perimembranous Ventricular Septal Defect (PmVSD) can be done safely and successfully. One of the most serious complications of VSD device closure is complete atrioventricular block which has been reported in 3-18% is various studies following VSD device closure. Amplatzar Duct Occluder II (ADO-II) device is now commonly being used for closure of small to moderate sized PmVSD. Complete heart block has not been reported with ADO-II device. We are reporting 2 cases of complete heart block following transcatheter closure of PmVSD using ADO-II device where sinus rhythm was established following early surgical removal of the device...
July 14, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28706620/adams-oliver-syndrome-with-unusual-central-nervous-system-findings-and-an-extrahepatic-portosystemic-shunt
#16
Carlos Pérez-García, Yolanda Ruíz Martín, Alejandra Aguado Del Hoyo, Carlos Marín Rodríguez, Minia Campos Domínguez
We report a case of a premature neonate girl with scalp and skull defects and brachydactyly of the feet consistent with an Adams-Oliver syndrome (AOS). The patient had central nervous system abnormalities, such as periventricular calcifications, hypoplastic corpus callosum, and bilateral hemispheric corticosubcortical hemorrhagic lesions. A muscular ventricular septal defect and a portosystemic shunt were diagnosed. To our knowledge, this is the first report of congenital supratentorial grey-white matter junction lesions without dural sinus thrombosis in association with AOS...
June 26, 2017: Pediatric Reports
https://www.readbyqxmd.com/read/28705664/complete-atrioventricular-canal-defect-towards-a-more-physiological-repair
#17
A Boutayeb
We describe a more physiological technique of complete atrioventricular septal defect repair which restores normal heart anatomy with the offset between the insertions of the mitral and tricuspid valves. This technique overcomes the drawbacks of the previous approaches, and may improve surgical outcomes, particularly in small infants with high ventricular septal defect component or dextroposed aorta.
June 15, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28705031/barium-exposure-increases-the-risk-of-congenital-heart-defects-occurrence-in-offspring
#18
Nannan Zhang, Zhen Liu, Xiaoxian Tian, Ming Chen, Ying Deng, Yixiong Guo, Nana Li, Ping Yu, Jiaxiang Yang, Jun Zhu
CONTEXT: Several studies have investigated the association between heavy metal exposure and congenital heart defects (CHDs). However, there are limited data regarding the relationship between barium exposure and the occurrence of CHDs. The objective of this study was to analyze the association between barium exposure in mothers and the risk of CHD in offspring. MATERIALS AND METHODS: We developed a case-control study with 399 cases and 490 controls with normal live births in China...
July 14, 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28705019/a-rare-case-of-pediatric-primary-cardiac-tumor-in-a-patient-with-down-syndrome
#19
Kimiaki Okada, Ayumu Masuoka, Kentaro Hotoda, Yoshimasa Uno, Takaaki Suzuki
Although hematological malignancies are a known complication of Down syndrome, few reports have described cases involving solid tumors. We describe the case of a 3-year-old Down syndrome girl with a primary solid cardiac tumor. Outpatient echocardiography after intracardiac repair of a ventricular septal defect at 6 months of age revealed a highly mobile pedunculated mass (8 × 9 mm) on the free wall of the right atrium. Due to potential incarceration of the mass in the tricuspid orifice, it was excised under extracorporeal circulation and cardiac arrest...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/28704916/three-dimensional-echocardiography-in-adult-congenital-heart-disease
#20
REVIEW
Hyun Suk Yang
Congenital heart disease (CHD) is now more common in adults than in children due to improvements in fetal echo, neonatal and pediatric care, and surgical techniques leading to dramatically increased survivability into adulthood. Adult patients with CHD, regardless of prior cardiac surgery, experience further cardiac problems or therapeutic challenges; therefore, a non-invasive, easily accessible echocardiographic examination is an essential follow-up tool. Among echocardiographic modalities, three-dimensional (3D) echocardiography provides better delineation of spatial relationships in complex cardiac geometries and more accurate volumetric information without geometric assumptions...
July 2017: Korean Journal of Internal Medicine
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