keyword
https://read.qxmd.com/read/38535111/rna-binding-proteins-in-cardiomyopathies
#1
REVIEW
De-Li Shi
The post-transcriptional regulation of gene expression plays an important role in heart development and disease. Cardiac-specific alternative splicing, mediated by RNA-binding proteins, orchestrates the isoform switching of proteins that are essential for cardiomyocyte organization and contraction. Dysfunctions of RNA-binding proteins impair heart development and cause the main types of cardiomyopathies, which represent a heterogenous group of abnormalities that severely affect heart structure and function...
March 5, 2024: Journal of Cardiovascular Development and Disease
https://read.qxmd.com/read/38533951/impact-of-an-impaired-maternal-fetal-environment-on-death-in-children-with-congenital-heart-defects-undergoing-surgery-in-denmark-from-1994-to-2018
#2
JOURNAL ARTICLE
Rasmus Kristensen, Camilla Omann, Charlotte K Ekelund, J William Gaynor, Vibeke E Hjortdal
BACKGROUND: Studies show that an impaired maternal-fetal environment (iMFE) increases the mortality risk in children with single-ventricle congenital heart defects (CHDs). We investigated the impact of an iMFE on death in children with various surgically corrected CHDs. METHODS AND RESULTS: In this nationwide register-based study, we examined the association between an iMFE (including preeclampsia, gestational hypertension, gestational diabetes, maternal smoking during pregnancy) and death in a large cohort of children with surgically corrected CHDs in Denmark (1994-2018)...
March 27, 2024: Journal of the American Heart Association
https://read.qxmd.com/read/38532293/how-to-protect-the-right-ventricle-in-hypoplastic-left-heart-syndrome
#3
JOURNAL ARTICLE
Shunji Sano
No abstract text is available yet for this article.
March 26, 2024: European Journal of Cardio-thoracic Surgery
https://read.qxmd.com/read/38522869/from-surgical-to-total-transcatheter-stage-i-palliation-exploring-evidence-and-perspectives
#4
REVIEW
Rodrigo Zea-Vera, Francesca Sperotto, Pirooz Eghtesady, Nicola Maschietto
Neonates with single ventricle physiology and ductal-dependent systemic circulation, such as those with hypoplastic left heart syndrome, undergo palliation in the first days of life. Over the past decades, variations on the traditional Stage 1 palliation, also known as Norwood operation, have emerged. These include the hybrid palliation and the total transcatheter approach. Here, we review the current evidence and data on different Stage 1 approaches, with a focus on their advantages, challenges, and future perspectives...
2024: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://read.qxmd.com/read/38522867/endocardial-fibroelastosis-resection-when-it-works-and-when-it-does-not
#5
REVIEW
Gregor Gierlinger, Sitaram M Emani
Endocardial fibroelastosis (EFE) is a thickening of the endocardial layer by accumulation of collagen and elastic fibers. Endothelial to mesenchymal transformation is proposed to be the underlying mechanism of formation. Although EFE can occur in both right and left ventricles, this article will focus on management of left ventricular EFE. Through its fibrous, nonelastic manifestation EFE restricts the myocardium leading to diastolic and systolic ventricular dysfunction and prevents ventricular growth in neonates and infants...
2024: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://read.qxmd.com/read/38519623/prognostic-value-of-rv-function-analysis-during-the-interstage-period-in-patients-with-hypoplastic-left-heart-syndrome
#6
JOURNAL ARTICLE
Alan P Wang, Nazia Husain, Jamie Penk, Christina Laternser, Defne Magnetta, Kae Watanabe, Amanda Hauck
Cardiac dysfunction is associated with mortality in children with hypoplastic left heart syndrome (HLHS). We evaluated the ability of qualitative and quantitative RV functional parameters to predict outcomes in HLHS patients. In this retrospective, single-center study, echocardiograms from 3 timepoints (pre-stage 1 palliation, 4-8 weeks post-stage 1 palliation, and pre-Glenn) were analyzed in infants with HLHS. Patients were stratified into two groups based on outcome of transplant-free survival post-Glenn (survivors) versus mortality or transplantation prior to Fontan (non-survivors)...
March 22, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38516005/hybrid-stage-1-palliation-for-hlhs-the-experience-of-a-tertiary-center-in-a-developing-country
#7
JOURNAL ARTICLE
Fouad Bitar, Issam M El-Rassi, Rana Zareef, Yehya Jassar, Jennifer Abboud, Ziad Bulbul, Fadi Bitar, Mariam Arabi
BACKGROUND: Hypoplastic left heart syndrome (HLHS) accounts for 2.6% of congenital heart disease and is an invariably fatal cardiac anomaly if left untreated. Approximately 33,750 babies are born annually with HLHS in developing countries. Unfortunately, the majority will not survive due to the scarcity of resources and the limited availability of surgical management. AIM: To describe and analyze our experience with the hybrid approach in the management of HLHS in a developing country...
2024: Frontiers in Cardiovascular Medicine
https://read.qxmd.com/read/38514154/cardiac-fibroma-presenting-as-hypoplastic-left-heart-syndrome-in-a-foetus-causal-or-coincidental
#8
JOURNAL ARTICLE
Abinaya Sundari, Umamaheshwari Gurusamy
Primary cardiac tumours are very rare. Cardiac tumours in the perinatal period are even more uncommon with a prevalence of 0.0017% to 0.28% in autopsy series. The majority of benign cardiac tumours are cardiac rhabdomyomas, followed by cardiac fibromas. Another rare congenital heart disease is hypoplastic left heart syndrome (HLHS). Here we present a 21-week-old foetus diagnosed antenatally with HLHS on foetal echocardiogram. An autopsy done on the foetus following medical termination of pregnancy revealed a cardiac fibroma in the ventricular septum...
March 21, 2024: BMJ Case Reports
https://read.qxmd.com/read/38499270/cmr-reference-values-of-right-ventricular-volumetric-variables-in-patients-with-hypoplastic-left-heart-syndrome
#9
JOURNAL ARTICLE
Andrik Ballenberger, Amke Caliebe, Sylvia Krupickova, Anselm Uebing, Dominik Daniel Gabbert, Inga Voges
BACKGROUND: Cardiovascular magnetic resonance (CMR) has established itself as the gold standard for serial assessment of systemic right ventricular (RV) performance but due to the lack of standardized RV reference values for HLHS patients, the interpretation of RV volumetric data in HLHS remains difficult. Therefore, this study aimed to close this gap by providing CMR reference values for the systemic RV in HLHS patients. METHODS: CMR scans of 160 children, adolescents and young adults (age range 2...
March 16, 2024: Journal of Cardiovascular Magnetic Resonance
https://read.qxmd.com/read/38497454/long-term-survival-after-single-ventricle-palliation-a-swedish-nationwide-cohort-study
#10
JOURNAL ARTICLE
Magnus Dalén, Michal Odermarsky, Petru Liuba, Jens Johansson Ramgren, Mats Synnergren, Jan Sunnegårdh
BACKGROUND: Long-term survival after single-ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported. METHODS AND RESULTS: This nationwide cohort study included all children undergoing operation with single-ventricle palliation during their first year of life in Sweden between January 1994 and December 2019. Data were obtained from institutional records and assessment of underlying cardiac anomaly and dominant ventricular morphology was based on complete review of medical records, surgical reports, and echocardiographic examinations...
March 19, 2024: Journal of the American Heart Association
https://read.qxmd.com/read/38497437/predicting-high-risk-fetal-cardiac-disease-anticipated-to-need-immediate-postnatal-stabilization-and-intervention-with-planned-pediatric-cardiac-operating-room-delivery
#11
REVIEW
Amol Moray, Proscovia M Mugaba, Chloe Joynt, Angela McBrien, Luke G Eckersley, Ernest Phillipos, Paula Holinski, Lindsay Ryerson, James Yashu Coe, Sujata Chandra, Billy Wong, Michele Derbyshire, Maria Lefebvre, Mohammed Al Aklabi, Lisa K Hornberger
BACKGROUND: Distances between delivery and cardiac services can make the care of fetuses with cardiac disease at risk of acute cardiorespiratory instability at birth a challenge. In 2013 we implemented a fetal echocardiography-based algorithm targeting fetuses considered high risk for acute cardiorespiratory instability at ≤2 hours of birth for delivery in our pediatric cardiac operating room of our children's hospital, and, herein, examine our experience. METHODS AND RESULTS: We reviewed maternal and postnatal medical records of all fetuses with cardiac disease encountered January 2013 to March 2022 considered high risk for acute cardiorespiratory instability...
March 19, 2024: Journal of the American Heart Association
https://read.qxmd.com/read/38494938/identifying-predictors-for-hypoplastic-aortic-arch-haa-in-pediatric-patients-with-complex-coarctation-of-the-aorta-coa
#12
JOURNAL ARTICLE
Ying Lyu, Lu Tian, Bin Qin, Xiao Fan, Ling He
OBJECTIVE: HAA is a significant risk factor in complex CoA patients. We conducted a retrospective study to explore the relationship between HAA and other cardiovascular factors. METHODS: We analyzed 103 patients diagnosed with complex CoA using CT angiography and echocardiography. Aortic diameter was measured at six levels, and severe coarctation was defined as coarctation site to diaphragmatic level ratio (CDR) < 50%. Correlations between non-HAA and HAA groups were assessed...
March 15, 2024: Current medical imaging
https://read.qxmd.com/read/38489836/configuration-of-the-neo-aortic-root-after-chimney-reconstruction-in-the-norwood-procedure
#13
JOURNAL ARTICLE
Satoshi Asada, Shinichiro Oda, Yoshinobu Maeda, Shuhei Fujita, Hisayuki Hongu, Eijiro Yamashita, Hiroki Nakatsuji, Takashi Nagase, Rie Nakai, Takaaki Hayashi, Jin Ikarashi, Yasutaka Goto, Masaaki Yamagishi
OBJECTIVES: After staged reconstruction for hypoplastic left heart syndrome (HLHS), the neoaortic root tends to dilate, and the incidence of significant neoaortic valve insufficiency increases with time. This study aimed to evaluate the mid-term outcomes of the neoaortic root geometries and valve function after chimney reconstruction in the Norwood procedure. METHODS: Between 2013 and 2021, 20 consecutive patients who underwent chimney reconstruction during the Norwood procedure for HLHS and its variants in our institution were enrolled...
March 15, 2024: European Journal of Cardio-thoracic Surgery
https://read.qxmd.com/read/38485103/sudden-unexpected-intrapartum-death-and-left-ventricular-noncompaction-involving-the-right-ventricle
#14
Giulia Ottaviani, Tobia Tomasello, Francesca Boggio, Letterio Runza, Alessandro Del Gobbo, L Maximilian Buja
Left ventricular noncompaction (LVNC), involving mainly the right ventricle, is a rare form of congenital heart disorder characterized by a developmental arrest in myocardial compaction, resulting in a spongy appearance of the myocardium, mainly of the right ventricle, rarely detected in fetuses. We report the case of a female fetus with a gestational age of 41+4 weeks who came to our attention for intrapartum sudden unexpected death, resulting in stillbirth. The ventricular walls, particularly the right ventricular wall, appeared thick, hypertrabeculated and spongy, leading to the diagnosis of LVNC involving mainly the right ventricle...
March 12, 2024: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://read.qxmd.com/read/38480572/differential-regulation-of-immune-related-genes-in-the-developing-heart
#15
JOURNAL ARTICLE
Mathieu Garand, Susie S Y Huang, Brian Dineen, Ian A Glass, Pirooz Eghtesady
In many congenital heart defects, it can be difficult to ascertain primary pathology from secondary consequences from altered flow through the developing heart. The molecular differences between the growing right and left ventricles (RV and LV, respectively) following the completion of septation and the impact of sex on these mechanisms have not been investigated. We analyzed RNA-seq data derived from twelve RV and LVs, one with Hypoplastic Left Heart Syndrome (HLHS), to compare the transcriptomic landscape between the ventricles during development...
March 13, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38477687/what-is-the-hypoplastic-left-heart-syndrome
#16
JOURNAL ARTICLE
Robert H Anderson, Diane E Spicer, Adrian Crucean
No abstract text is available yet for this article.
March 13, 2024: World Journal for Pediatric & Congenital Heart Surgery
https://read.qxmd.com/read/38465590/total-aortic-arch-and-neoaortic-root-replacement-following-stage-iii-palliation-for-hypoplastic-left-heart-syndrome
#17
JOURNAL ARTICLE
Sayar Kumar Munshi, Tommaso Generali, Arul Narayanan, Raul Jose Correia, Ramesh Kutty, Ram Dhannapuneni
Progressive dilatation of the neoaortic root and reconstructed aortic arch is a serious complication after the Norwood procedure. There are no clear guidelines on the management of this complex anatomy in the setting of single ventricle physiology, and the surgical treatment of such an entity remains anecdotal. We describe a successful surgical repair in a 15-year-old girl presenting with a severely dilated neoaortic root and aortic arch causing compression and narrowing of the left pulmonary artery after successful three-stage palliation for hypoplastic left heart syndrome...
March 11, 2024: World Journal for Pediatric & Congenital Heart Surgery
https://read.qxmd.com/read/38464057/mitotic-block-and-epigenetic-repression-underlie-neurodevelopmental-defects-and-neurobehavioral-deficits-in-congenital-heart-disease
#18
George C Gabriel, Hisato Yagi, Tuantuan Tan, Abha S Bais, Benjamin J Glennon, Margaret C Stapleton, Lihua Huang, William T Reynolds, Marla G Shaffer, Madhavi Ganapathiraju, Dennis Simon, Ashok Panigrahy, Yijen L Wu, Cecilia W Lo
Poor neurodevelopment is often observed with congenital heart disease (CHD), especially with mutations in chromatin modifiers. Here analysis of mice with hypoplastic left heart syndrome (HLHS) arising from mutations in Sin3A associated chromatin modifier Sap130 , and adhesion protein Pcdha9, revealed neurodevelopmental and neurobehavioral deficits reminiscent of those in HLHS patients. Microcephaly was associated with impaired cortical neurogenesis, mitotic block, and increased apoptosis. Transcriptional profiling indicated dysregulated neurogenesis by REST, altered CREB signaling regulating memory and synaptic plasticity, and impaired neurovascular coupling modulating cerebral blood flow...
February 26, 2024: bioRxiv
https://read.qxmd.com/read/38459240/surgical-modulation-of-pulmonary-artery-shear-stress-a-patient-specific-cfd-analysis-of-the-norwood-procedure
#19
JOURNAL ARTICLE
Simbarashe G Chidyagwai, Michael S Kaplan, Christopher W Jensen, James S Chen, Reid C Chamberlain, Kevin D Hill, Piers C A Barker, Timothy C Slesnick, Amanda Randles
PURPOSR: This study created 3D CFD models of the Norwood procedure for hypoplastic left heart syndrome (HLHS) using standard angiography and echocardiogram data to investigate the impact of shunt characteristics on pulmonary artery (PA) hemodynamics. Leveraging routine clinical data offers advantages such as availability and cost-effectiveness without subjecting patients to additional invasive procedures. METHODS: Patient-specific geometries of the intrathoracic arteries of two Norwood patients were generated from biplane cineangiograms...
March 8, 2024: Cardiovascular Engineering and Technology
https://read.qxmd.com/read/38447986/labyrinthine-cor-triatriatum-sinister-in-fetal-hypoplastic-left-heart-syndrome-is-associated-with-poor-outcomes
#20
JOURNAL ARTICLE
Amna Qasim, Tam T Doan, Betul Yilmaz Furtun, Ziyad Binsalamah, Iki Adachi, Shaine A Morris
OBJECTIVES: A subset of hypoplastic-left-heart-syndrome (HLHS) fetuses have a complex cor-triatriatum sinister that we named "labyrinthine-cor (L-cor)". We sought to determine the prevalence of L-cor in HLHS fetuses and hypothesized that it is associated with increased mortality. METHODS: This single-center retrospective cohort study included all HLHS fetuses from January 2010-December 2020. Fetuses with other hypoplastic-left-heart variants, inadequate images, lack of follow-up and fetal atrial-septal interventions were excluded...
March 6, 2024: Prenatal Diagnosis
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