keyword
https://read.qxmd.com/read/38546282/paediatric-endoscopic-endonasal-middle-skull-base-reconstruction-retrospective-analysis-of-78-patients-treated-in-a-single-tertiary-care-paediatric-center
#1
JOURNAL ARTICLE
Danilo Di Giorgio, Filippo Giovannetti, Paolo Priore, Mirko Scagnet, Federico Mussa, Ingrid Raponi, Valentino Valentini, Lorenzo Genitori
INTRODUCTION: Skull base pathologies in the paediatric population are rare and require treatment by multiple qualified specialists. The endoscopic endonasal approach has revolutionized surgical treatment because it is less invasive than existing treatments.The goal of this study was to retrospectively review our experience with the reconstruction of paediatric skull middle base defects and associated complications. MATERIALS AND METHODS: We analysed medical records from patients aged ≤ 18 years who were treated at our centre between 2013 and 2021...
March 28, 2024: British Journal of Neurosurgery
https://read.qxmd.com/read/38544878/congenital-tracheal-stenosis-patients-undergoing-modified-slide-tracheoplasty-single-centre-technique-and-long-term-morbidity-and-mortality
#2
JOURNAL ARTICLE
Ryaan El-Andari, Rami Zibdawi, Paula Holinski, John Koller, Chloe Joynt, Nee Khoo, Laurance Lequier, Hamdy El-Hakim, Mohammed Al Aklabi
BACKGROUND: Congenital malformations of the trachea are rare but often life-threatening. Limited data have been published on the outcomes of tracheal reconstruction for congenital tracheal stenosis. We sought to describe the outcomes of patients undergoing tracheal reconstruction over 10 years at our centre. METHODS: All paediatric patients who underwent long-segment tracheal or bronchial reconstruction from January 1, 2012, to August 31, 2022, were included. The primary outcome was mortality, and secondary outcomes included reoperation and postoperative morbidity...
February 2024: CJC Pediatr Congenit Heart Dis
https://read.qxmd.com/read/38541848/predicting-long-term-childhood-survival-of-newborns-with-congenital-heart-defects-a-population-based-prospective-cohort-study-epicard
#3
JOURNAL ARTICLE
Makan Rahshenas, Nathalie Lelong, Damien Bonnet, Lucile Houyel, Babak Choodari-Oskooei, Mithat Gonen, Francois Goffinet, Babak Khoshnood
Backgroud: Congenital heart defects (CHDs) are the most frequent group of major congenital anomalies, accounting for almost 1% of all births. They comprise a very heterogeneous group of birth defects in terms of their severity, clinical management, epidemiology, and embryologic origins. Taking this heterogeneity into account is an important imperative to provide reliable prognostic information to patients and their caregivers, as well as to compare results between centers or to assess alternative diagnostic and treatment strategies...
March 12, 2024: Journal of Clinical Medicine
https://read.qxmd.com/read/38539038/cleft-closure-and-other-predictors-of-contemporary-outcomes-after-atrioventricular-canal-repair-in-patients-with-parachute-left-atrioventricular-valve
#4
JOURNAL ARTICLE
Patrick McGeoghegan, Minmin Lu, Lynn A Sleeper, Sitaram Emani, Chris Baird, Eric Feins, Laura A Gellis, Kevin G Friedman
OBJECTIVES: Parachute left atrioventricular valve (LAVV) complicates atrioventricular septal defect (AVSD) repair. We evaluate outcomes of AVSD patients with parachute LAVV and identify risk factors for adverse outcomes. METHODS: We evaluated all patients undergoing repair of AVSD with parachute LAVV from 2012-2021. The primary outcome was a composite of time-to death, LAVV reintervention, and development of ≥ moderate LAVV dysfunction (≥ moderate LAVV stenosis [LAVVS] and/or LAVV regurgitation [LAVVR])...
March 27, 2024: Interdiscip Cardiovasc Thorac Surg
https://read.qxmd.com/read/38537425/real-world-safety-of-levetiracetam-mining-and-analysis-of-its-adverse-drug-reactions-based-on-faers-database
#5
JOURNAL ARTICLE
Zhimin He, Cuimin Liu, Lin Lin, Guowen Feng, Gang Wu
INTRODUCTION: Levetiracetam is a relatively new and widely utilized anti-seizure medication; however, limited information is available regarding its adverse effects. This study aims to thoroughly investigate, evaluate, and present evidence on the safety profile of Levetiracetam, relying on data from the FDA Adverse Event Reporting System (FAERS) database to facilitate informed clinical decision-making. METHODS: We employed various statistical measures, including Reporting Odds Ratio (ROR), Proportionate Reporting Ratio (PRR), and analysis by the Medicines and Healthcare Products Regulatory Agency (MHRA), to identify signals of adverse reactions associated with Levetiracetam...
March 21, 2024: Seizure: the Journal of the British Epilepsy Association
https://read.qxmd.com/read/38535015/congenital-heart-defects-in-patients-with-molecularly-confirmed-sotos-syndrome
#6
JOURNAL ARTICLE
Giulio Calcagni, Federica Ferrigno, Alessio Franceschini, Maria Lisa Dentici, Rossella Capolino, Lorenzo Sinibaldi, Chiara Minotti, Alessia Micalizzi, Viola Alesi, Antonio Novelli, Anwar Baban, Giovanni Parlapiano, Domenico Coviello, Paolo Versacci, Carolina Putotto, Marcello Chinali, Fabrizio Drago, Andrea Bartuli, Bruno Marino, Maria Cristina Digilio
Sotos syndrome is an autosomal dominant condition characterized by overgrowth with advanced bone age, macrodolicocephaly, motor developmental delays and learning difficulties, and characteristic facial features caused by heterozygous pathogenetic variants in the NSD1 gene located on chromosome 5q35. The prevalence of heart defects (HDs) in individuals with Sotos syndrome is estimated to be around 15-40%. Septal defects and patent ductus arteriosus are the most commonly diagnosed malformations, but complex defects have also been reported...
March 11, 2024: Diagnostics
https://read.qxmd.com/read/38532329/structure-predictions-and-functional-insights-into-amidase_3-domain-containing-n-acetylmuramyl-l-alanine-amidases-from-deinococcus-indicus-dr1
#7
JOURNAL ARTICLE
Malvika Modi, Menaka Thambiraja, Archana Cherukat, Ragothaman M Yennamalli, Richa Priyadarshini
BACKGROUND: N-acetylmuramyl-L-alanine amidases are cell wall modifying enzymes that cleave the amide bond between the sugar residues and stem peptide in peptidoglycan. Amidases play a vital role in septal cell wall cleavage and help separate daughter cells during cell division. Most amidases are zinc metalloenzymes, and E. coli cells lacking amidases grow as chains with daughter cells attached to each other. In this study, we have characterized two amidase enzymes from Deinococcus indicus DR1...
March 26, 2024: BMC Microbiology
https://read.qxmd.com/read/38530796/automated-isochronal-late-activation-mapping-for-substrate-characterization-in-patients-with-repaired-tetralogy-of-fallot
#8
JOURNAL ARTICLE
Eduardo Arana-Rueda, Juan Acosta, Manuel Frutos-López, Juan-Antonio Sánchez-Brotons, Carmen González de la Portilla-Concha, Pastora Gallego, Alonso Pedrote
AIMS: Slow conduction (SC) anatomical isthmuses (AIs) are the dominant substrate for monomorphic ventricular tachycardia (VT) in patients with repaired tetralogy of Fallot (rTF). This study aimed to evaluate the utility of automated propagational analysis for the identification of SC-AI in patients with rTF. METHODS AND RESULTS: Consecutive rTF patients undergoing VT substrate characterization were included. Automated isochronal late activation maps (ILAM) were obtained with multielectrode HD Grid Catheter...
March 1, 2024: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://read.qxmd.com/read/38529899/correction-of-absent-pulmonary-valve-syndrome-using-hilum-to-hilum-internal-pulmonary-artery-plication-plasty
#9
Fabian A Kari, Sebastian Michel, Jürgen Hörer
A 12-month-old girl (8.4 kg) with absent pulmonary valve syndrome and enlarged bilateral pulmonary arteries underwent trans-tricuspid ventricular septal defect closure, placement of a right ventricular-to-pulmonary artery conduit and hilum-to-hilum internal pulmonary artery plication plasty.
March 26, 2024: Multimedia Manual of Cardiothoracic Surgery: MMCTS
https://read.qxmd.com/read/38529454/congenital-heart-defects-in-pregnancies-conceived-by-assisted-reproductive-technology-comparing-functional-and-structural-defects
#10
JOURNAL ARTICLE
Saeid Rasouli, Mohammad Radgoodarzi, Reza Azarbad, Azim Ghazvini, Mohammadjavad Sotoudeheian, Mehdi Taghizadeh, Mohammad Sedigh Dakkali
Introduction Congenital heart defects (CHD) are one of the most common congenital anomalies, and their association with assisted reproductive technology (ART) is controversial in different populations. The purpose of this study was to evaluate this association and to provide information about the necessity of specialized echocardiography during pregnancy with ART. Methods This retrospective study was performed on all pregnancies conceived by ART and referred for fetal echocardiography to the Rasoul Akram and Akbar Abadi hospitals in Tehran, Iran...
February 2024: Curēus
https://read.qxmd.com/read/38528794/bicuspidisation-of-unicuspid-stenotic-pulmonary-valve-in-a-nine-year-old-male
#11
JOURNAL ARTICLE
Florin Anghel, Catalin Constantin Badiu, Alain J Poncelet
We report the case of a 9-year-old male with severe congenital pulmonary valve stenosis referred to our centre for percutaneous valvotomy. On admission, trans-thoracic echocardiogram confirmed a unicuspid pulmonary valve with a peak/mean pulmonary valve gradient of 91/53 mmHg and a pulmonary annulus of 13.8 mm (-0.8 Z Score). It also showed an enlarged RV (RV/LV ratio 0,9). During cardiac catheterisation, an additional atrial septal defect (secundum) with significant left to right shunt (Qp/Qs > 2) was diagnosed, which was not amenable to percutaneous closure...
March 26, 2024: Cardiology in the Young
https://read.qxmd.com/read/38524994/multimodality-imaging-in-the-diagnosis-of-unroofed-coronary-sinus-in-the-adult
#12
Prashanth Venkatesh, Ruchira Garg
• Unroofed CS is a rare cause of atrial-level shunting and right heart dilatation. • Unroofed CS is commonly diagnosed in adulthood. • TTE is the primary modality to recognize and diagnose unroofed CS. • CCT and/or magnetic resonance imaging should be done as complementary studies before CS repair.
March 2024: CASE: Cardiovascular Imaging Case Reports
https://read.qxmd.com/read/38524970/novel-transcatheter-approach-to-treat-primum-atrial-septal-defects
#13
Gregory J Condos, James M McCabe, Denise C Joffe, Richard D Sheu
•Degenerative common AVC defect can mimic rheumatic MV stenosis.•Closure of primum ASD can be achieved percutaneously.•Live 3D multiplanar TEE is crucial for procedural guidance.
March 2024: CASE: Cardiovascular Imaging Case Reports
https://read.qxmd.com/read/38524968/large-secundum-atrial-septal-defect-managed-conservatively-in-an-adult-dog
#14
Carl Toborowsky, Lauren E Markovic
• ASDs can be medically managed in dogs if interventional options are not selected. • 3D modeling can be used for canine congenital heart disease. • 3D imaging helps with preprocedural planning for ASD.
March 2024: CASE: Cardiovascular Imaging Case Reports
https://read.qxmd.com/read/38523790/high-paternal-homocysteine-causes-ventricular-septal-defects-in-mouse-offspring
#15
JOURNAL ARTICLE
Lian Liu, Xuan Zhang, Hao-Ran Geng, Ya-Nan Qiao, Yong-Hao Gui, Jian-Yuan Zhao
Maternal hyperhomocysteinemia is widely considered as an independent risk of congenital heart disease (CHD). However, whether high paternal homocysteine causes CHD remains unknown. Here, we showed that increased homocysteine levels of male mice caused decreased sperm count, sperm motility defect and ventricular septal defect of the offspring. Moreover, high levels of paternal homocysteine decrease sperm DNMT3A/3B, accompanied with changes in DNA methylation levels in the promoter regions of CHD-related genes...
April 19, 2024: IScience
https://read.qxmd.com/read/38522876/hypertrophic-cardiomyopathy-preadolescence-mitral-valve-disease-and-midventricular-obstruction
#16
REVIEW
Elaine M Griffeth, Elizabeth H Stephens, Joseph A Dearani
Septal myectomy is indicated in patients with obstructive hypertrophic cardiomyopathy (HCM) who have persistent symptoms despite medical therapy, intolerance of medication side effects, or severe resting or provocable gradients. Septal myectomy at high volume centers is safe, with low operative mortality (1%) and low rates of complications such as complete heart block or ventricular septal defect (3% and 0.5%, respectively). Additionally, improved survival following myectomy has been observed when compared to patients with obstructive HCM managed medically or those with nonobstructive HCM...
2024: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://read.qxmd.com/read/38522875/en-bloc-rotation-of-the-outflow-tracts-double-root-translocation
#17
REVIEW
Rudolf Mair, Michaela Kreuzer, Eva Sames-Dolzer
En bloc rotation of the outflow tracts or double root translocation offers an anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction and closely related forms of double outlet right ventricle. The technical principle is to excise aortic and pulmonary root en bloc, rotate them as a whole by 180°, and reimplant them. The left ventricular outflow tract is enlarged with the patch closing the ventricular septal defect. In our experience, two thirds of the pulmonary valves could be preserved...
2024: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://read.qxmd.com/read/38521975/diagnostic-yield-after-next-generation-sequencing-in-pediatric-cardiovascular-disease
#18
JOURNAL ARTICLE
Anne M Slavotinek, Michelle L Thompson, Lisa J Martin, Bruce D Gelb
Genetic testing with exome sequencing and genome sequencing is increasingly offered to infants and children with cardiovascular diseases. However, the rates of positive diagnoses after genetic testing within the different categories of cardiac disease and phenotypic subtypes of congenital heart disease (CHD) has been little studied. We report the diagnostic yield after next-generation sequencing in 500 patients with CHD from diverse population subgroups that were enrolled at three different sites in the Clinical Sequencing Evidence-Generating Research consortium...
March 22, 2024: HGG advances
https://read.qxmd.com/read/38521846/off-label-use-of-muscular-vsd-device-for-closure-of-a-rare-congenital-portosystemic-shunt
#19
JOURNAL ARTICLE
Hala Mounir Agha, Shady Mashoor, Mohamed Ghobashy, Antoine AbdelMassih, Hanya Gaber, Hanaa El-Karaksy
BACKGROUND: Congenital portosystemic shunt (CPSS) is a vascular malformation in which portal blood drains toward the systemic circulation, leading to pulmonary hypertension. CASE PRESENTATION: A 10-year-old patient was brought for evaluation because of dyspnea on exertion. Echocardiography revealed a pulmonary hypertension of 75 mmHg, and multi-slice CT angiography revealed the presence of a CPSS. Closure was finally implemented using a muscular ventricular septal defect device...
March 23, 2024: Egyptian Heart Journal: EHJ
https://read.qxmd.com/read/38521547/one-year-postprocedural-quality-of-life-following-mitral-valve-surgery-data-from-the-netherlands-heart-registration
#20
JOURNAL ARTICLE
Samuel Heuts, Jules R Olsthoorn, Saskia Houterman, Maaike M Roefs, Jos G Maessen, Peyman Sardari Nia
OBJECTIVES: The aim of surgical treatment of mitral valve disease is to reverse heart failure and to restore life expectancy and quality of life (QoL). In mitral valve surgery, QoL has not been studied extensively, especially regarding the surgical approach. The current study aimed to evaluate QoL after mitral valve surgery through full sternotomy and a minimally invasive approach (MIMVS). METHODS: All patients undergoing mitral valve surgery between 2013-2018 through sternotomy or a MIMVS approach (right anterolateral mini-thoracotomy, sternal-sparing), with or without concomitant tricuspid valve surgery, surgical ablation, or atrial septal defect closure were eligible for inclusion in this multicentre nationwide registry in the Netherlands...
March 23, 2024: Interdiscip Cardiovasc Thorac Surg
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