keyword
https://read.qxmd.com/read/38545080/left-ventricular-function-assessment-including-or-excluding-trabeculations-in-left-ventricular-non-compaction-a-preliminary-case-control-cardiac-magnetic-resonance-study
#1
JOURNAL ARTICLE
Jean-Pierre Laissy, Ahmed Ben Driss
BACKGROUND: Functional assessment of compact myocardium and hypertrabeculations in left ventricular non-compaction (LVNC) is underestimated with regards to the morphological spectrum of disease. We aimed to assess whether measuring concurrently left ventricular (LV) volume, mass and ejection fraction (LVEF) with and without trabeculation inclusion on cine magnetic resonance (cineMR) could help diagnose patients with LVNC by comparison to normal individuals with an excess of myocardial trabeculations...
March 15, 2024: Quantitative Imaging in Medicine and Surgery
https://read.qxmd.com/read/38535015/congenital-heart-defects-in-patients-with-molecularly-confirmed-sotos-syndrome
#2
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/38496124/when-hearts-and-minds-collide-a-case-report-of-left-ventricle-noncompaction-syndrome-as-a-precursor-to-acute-ischemic-stroke-in-a-15-year-old-along-with-a-literature-review
#3
Yousif Alkhafaji, Omar Farooq Al-Nahhas, Khaled Alaboud Alkheder, Jamal A Alkoteesh
Pediatric stroke, though uncommon, is often underdiagnosed due to subtle symptoms and delayed recognition. Cardiac diseases, accounting for up to 33% of pediatric ischemic strokes, play a significant role. This case report explores the rare occurrence of ischemic stroke in a 15-year-old boy with left ventricular non-compaction syndrome (LVNC). It underscores the complexity of managing pediatric ischemic stroke, particularly in the context of LVNC, emphasizing the challenges in timely diagnosis and management...
February 2024: Curēus
https://read.qxmd.com/read/38492683/left-ventricular-trabeculation-arrhythmogenic-and-clinical-significance-in-elite-athletes
#4
JOURNAL ARTICLE
Giuseppe Di Gioia, Simone Pasquale Crispino, Sara Monosilio, Viviana Maestrini, Antonio Nenna, Alessandro Spinelli, Erika Lemme, Maria Rosaria Squeo, Antonio Pelliccia
INTRODUCTION: Left ventricular trabeculations (LVTs) are common findings in athletes. Limited information exists regarding clinical significance, management, and outcome. OBJECTIVES: To study the prevalence and morphologic characteristics of LVTs in elite athletes, with a focus on clinical correlates and prognostic significance. METHODS: We enrolled 1.492 Olympic elite athletes of different sports disciplines with ECG, echocardiogram, and exercise stress test...
March 14, 2024: Journal of the American Society of Echocardiography
https://read.qxmd.com/read/38382423/preeclampsia-eclampsia-impacts-the-structure-and-function-of-neonatal-hearts-probably-by-reducing-myocardial-compaction
#5
JOURNAL ARTICLE
Zexin Li, Jinxiu Zhu, Yequn Chen, Fengxiang Wei, Jinying Yang, Xuerui Tan
PURPOSE: Preeclampsia/Eclampsia (PE/E) poses significant risks to neonatal cardiac health. Traditional echocardiographic methods have limitations in detailing these impacts. This study hypothesized that echocardiographic radiomics could provide a more comprehensive assessment of the cardiac changes in neonates affected by PE/E. METHOD: In a comprehensive analysis, 2594 neonates underwent echocardiographic screening. From these, 556 were selected for detailed radiomics analysis, focusing on cardiac shape, movement, and texture features...
February 17, 2024: European Journal of Radiology
https://read.qxmd.com/read/38341315/ebstein-s-anomaly-of-the-tricuspid-valve-mitral-valve-prolapse-and-left-ventricular-non-compaction-a-triple-trouble
#6
JOURNAL ARTICLE
Swasthi S Kumar, Sudipta Mondal, Saurav Banerjee
No abstract text is available yet for this article.
February 9, 2024: Heart, Lung & Circulation
https://read.qxmd.com/read/38316882/cardiac-manifestations-of-human-acta2-variants-recapitulated-in-a-zebrafish-model
#7
JOURNAL ARTICLE
Wulan Apridita Sebastian, Masanori Inoue, Nobuyuki Shimizu, Ryosuke Sato, Saori Oguri, Tomoyo Itonaga, Shintaro Kishimoto, Hiroshi Shiraishi, Toshikatsu Hanada, Kenji Ihara
The ACTA2 gene encodes actin α2, a major smooth muscle protein in vascular smooth muscle cells. Missense variants in the ACTA2 gene can cause inherited thoracic aortic diseases with characteristic symptoms, such as dysfunction of smooth muscle cells in the lungs, brain vessels, intestines, pupils, bladder, or heart. We identified a heterozygous missense variant of Gly148Arg (G148R) in a patient with a thoracic aortic aneurysm, dissection, and left ventricular non-compaction. We used zebrafish as an in vivo model to investigate whether or not the variants might cause functional or histopathological abnormalities in the heart...
February 5, 2024: Journal of Human Genetics
https://read.qxmd.com/read/38298349/left-ventricular-noncompaction-cardiomyopathy-a-scoping-review
#8
JOURNAL ARTICLE
O S Ogah, E P Iyawe, K F Okwunze, C A Nwamadiegesi, F E Obiekwe, M O Fabowale, M Okeke, O A Orimolade, O V Olalusi, A Aje, A Adebiyi
INTRODUCTION: There has been an upsurge in the reporting of cases of Left Ventricular Noncompaction (LVNC) cardiomyopathy in medical literature in the last 35 years due to advances in medical imaging.The condition was first described in 1926 and the first reported case by echocardiography was in 1984. The American Heart Association considers LVNC a primary cardiomyopathy of genetic origin, while the European Society of Cardiology and the World Health Organization grouped it as an unclassified cardiomyopathy...
December 2023: Annals of Ibadan Postgraduate Medicine
https://read.qxmd.com/read/38270365/-clinical-and-genetic-manifestations-of-left-ventricular-non-compaction-in-children
#9
JOURNAL ARTICLE
Emanuele Monda, Gaetano Diana, Federica Verrillo, Marta Rubino, Annapaola Cirillo, Adelaide Fusco, Chiara Cirillo, Alfredo Mauriello, Ippolita Altobelli, Martina Caiazza, Francesca Dongiglio, Giuseppe Palmiero, Maria Giovanna Russo, Giuseppe Limongelli
Left ventricular non-compaction (LVNC) is a myocardial disease characterized by a two-layered structure typically seen at the apical and lateral left portions of the ventricular myocardium, distal to the papillary muscles. While considered a rare disease, its prevalence in children is increasing due to the increased awareness of this condition and improved resolution of imaging techniques. The etiology is heterogeneous, ranging from inherited conditions to acquired diseases. Although many patients are asymptomatic, some patients may experience adverse events, including heart failure, arrhythmias, or thromboembolic events...
February 2024: Giornale Italiano di Cardiologia
https://read.qxmd.com/read/38237796/comparison-of-multi-point-pacing-and-conventional-crt-through-non-invasive-hemodynamics-measurement-short-and-long-term-results-of-the-compact-mpp-study
#10
JOURNAL ARTICLE
Francesco Passafaro, Antonio Rapacciuolo, Antonio Ruocco, Giuseppe Ammirati, Salvatore Crispo, Eugenia Pasceri, Giuseppe Santarpia, Ciro Mauro, Giovanni Esposito, Ciro Indolfi, Antonio Curcio
Invasive hemodynamic studies have demonstrated improved left ventricular (LV) performances when cardiac resynchronization therapy/defibrillator (CRT-D) is delivered through multi-point pacing (MPP). Nowadays, strategies that allow to study the same hemodynamic parameters at a non-invasive level have become available. The aim of the present study was to evaluate the clinical implication of using a patient-tailored approach for CRT programming based on non-invasively assessed LV hemodynamics to identify the best biventricular pacing modality between standard single-site pacing (STD) and MPP for each patient...
January 16, 2024: American Journal of Cardiology
https://read.qxmd.com/read/38223010/a-novel-tpm1-mutation-causes-familial-hypertrophic-cardiomyopathy-in-an-indian-family-genetic-and-clinical-correlation
#11
Prabodh Kumar, Ganesh Paramasivam, Tom Devasia, Mukund Prabhu, Maneesh K Rai, K Prakashini, Sandeep Mallya, Dinesh Reghunathan, A Megha, Krishnananda Nayak, Rajasekhar Moka
Hypertrophic cardiomyopathy (HCM) is a common inherited cardiac disorder characterised by unexplained left ventricular hypertrophy in the absence of abnormal loading conditions. The global prevalence of HCM is estimated to be 1 in 250 in the general population. It is caused due to mutations in genes coding for sarcomeric proteins. α-tropomyosin (TPM1) is an important protein in the sarcomeric thin filament which regulates sarcomere contraction. Mutations in TPM1 are known to cause hypertrophic cardiomyopathy, dilated cardiomyopathy and left ventricular non-compaction...
January 2024: Indian Journal of Clinical Biochemistry: IJCB
https://read.qxmd.com/read/38202085/myocardial-mechanics-and-associated-valvular-and-vascular-abnormalities-in-left-ventricular-noncompaction-cardiomyopathy
#12
REVIEW
Attila Nemes
Left ventricular (LV) non-compaction (LVNC) is a rare genetic cardiomyopathy due to abnormal intra-uterine arrest of compaction of the myocardial fibers during endomyocardial embryogenesis. Due to the partial or complete absence of LV compaction, the structure of the LV wall shows characteristic abnormalities, including a thin compacted epicardium and a thick non-compacted endocardium with prominent trabeculations and deep intertrabecular recesses. LVNC is frequently associated with chronic heart failure, life-threatening ventricular arrhythmias, and systemic embolic events...
December 22, 2023: Journal of Clinical Medicine
https://read.qxmd.com/read/38202009/concealed-inherited-cardiomyopathies-detected-in-cardio-oncology-screening
#13
JOURNAL ARTICLE
Rebeca Lorca, Isaac Pascual, Maria Fernandez, Rut Alvarez-Velasco, Santiago Colunga, Maria Muñiz, Marta Izquierdo, Yolanda Fernandez, Emilio Esteban, Juan Gomez, Pablo Avanzas, Teresa Lopez-Fernandez
INTRODUCTION: Basal cardiovascular risk assessment in cardio-oncology is essential. Integrating clinical information, ECG and transthoracic echocardiogram can identify concealed inherited cardiomyopathies (ICMPs) with potential added risk of cardiotoxicity. We aimed to evaluate the impact of our Cardio-Oncology Unit design in detecting concealed ICMPs. METHODS: We carried out a retrospective study of all consecutive breast cancer patients referred to the Cardio-Oncology Unit for cardiac evaluation (2020-2022)...
December 19, 2023: Journal of Clinical Medicine
https://read.qxmd.com/read/38201424/left-ventricular-non-compaction-in-children-aetiology-and-diagnostic-criteria
#14
REVIEW
Emanuele Monda, Gianantonio De Michele, Gaetano Diana, Federica Verrillo, Marta Rubino, Annapaola Cirillo, Adelaide Fusco, Federica Amodio, Martina Caiazza, Francesca Dongiglio, Giuseppe Palmiero, Pietro Buono, Maria Giovanna Russo, Giuseppe Limongelli
Left ventricular non-compaction (LVNC) is a heterogeneous myocardial disorder characterized by prominent trabeculae protruding into the left ventricular lumen and deep intertrabecular recesses. LVNC can manifest in isolation or alongside other heart muscle diseases. Its occurrence among children is rising due to advancements in imaging techniques. The origins of LVNC are diverse, involving both genetic and acquired forms. The clinical manifestation varies greatly, with some cases presenting no symptoms, while others typically manifesting with heart failure, systemic embolism, and arrhythmias...
January 4, 2024: Diagnostics
https://read.qxmd.com/read/38141360/generation-of-an-rbm20-mutation-associated-left-ventricular-non-compaction-cardiomyopathy-ipsc-line-umgi255-a-into-a-dcm-genetic-background-to-investigate-monogenetic-cardiomyopathies
#15
JOURNAL ARTICLE
Hanna Eberl, Sabine Rebs, Stefanie Hoppe, Farbod Sedaghat-Hamedani, Elham Kayvanpour, Benjamin Meder, Katrin Streckfuss-Bömeke
RBM20 mutations account for 3 % of genetic cardiomypathies and manifest with high penetrance and arrhythmogenic effects. Numerous mutations in the conserved RS domain have been described as causing dilated cardiomyopathy (DCM), whereas a particular mutation (p.R634L) drives development of a different cardiac phenotype: left-ventricular non-compaction cardiomyopathy. We generated a mutation-induced pluripotent stem cell (iPSC) line in which the RBM20-LVNC mutation p.R634L was introduced into a DCM patient line with rescued RBM20-p...
December 16, 2023: Stem Cell Research
https://read.qxmd.com/read/38137702/improving-a-deep-learning-model-to-accurately-diagnose-lvnc
#16
JOURNAL ARTICLE
Jaime Rafael Barón, Gregorio Bernabé, Pilar González-Férez, José Manuel García, Guillem Casas, Josefa González-Carrillo
Accurate diagnosis of Left Ventricular Noncompaction Cardiomyopathy (LVNC) is critical for proper patient treatment but remains challenging. This work improves LVNC detection by improving left ventricle segmentation in cardiac MR images. Trabeculated left ventricle indicates LVNC, but automatic segmentation is difficult. We present techniques to improve segmentation and evaluate their impact on LVNC diagnosis. Three main methods are introduced: (1) using full 800 × 800 MR images rather than 512 × 512; (2) a clustering algorithm to eliminate neural network hallucinations; (3) advanced network architectures including Attention U-Net, MSA-UNet, and U-Net++...
December 12, 2023: Journal of Clinical Medicine
https://read.qxmd.com/read/38072092/a-fetal-rat-model-of-ventricular-non-compaction-caused-by-intrauterine-hyperglycemia
#17
JOURNAL ARTICLE
Fanglu Wang, Songbo Han, Ligang Fang, Xue Lin
BACKGROUND: This study aims to develop a fetal rat model of ventricular non-compaction (NVM) using streptozotocin (STZ)-induced gestational hyperglycemia and compare it with a retinoic acid (RA) model. METHODS: Female SD rats were categorized into STZ, RA, and normal control (NC) groups. The STZ group was given a high-fat diet pre-pregnancy and 35 mg/kg of 2% STZ post-pregnancy. The RA group received a 90 mg/kg dose of RA on day 13 post-pregnancy. Embryonic myocardial morphology was analyzed through HE staining, and embryonic cardiomyocyte ultrastructures were studied using electron microscopy...
December 8, 2023: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://read.qxmd.com/read/38050525/overlapping-phenotypes-of-alcoholic-cardiomyopathy-and-left-ventricular-non-compaction-a-case-report-and-discussion-of-converging-cardiomyopathies
#18
Vaidehi Mendpara, Jaya Krishna Reddy Endreddy, Sahini Gajula, Pratyusha Ravulapalli, Mahendra Kumar, Parvinder Kaur, Meet Thakkar
Left ventricular non-compaction cardiomyopathy, often known as LVNC, is a form of congenital cardiomyopathy that is extremely uncommon. It is a condition that may be identified by an elevated number of endomyocardial trabeculations as well as an increase in their prominence. Alcoholic cardiomyopathy, also known as ACM, is a non-ischemic form of dilated cardiomyopathy that is characterized by contractile failure and an enlargement of the heart ventricles. It is not entirely known whether or not there is a clinically significant overlap in phenotypic characteristics between the two illnesses...
November 2023: Curēus
https://read.qxmd.com/read/38021952/left-ventricular-non-compaction-cardiomyopathy-the-key-to-its-diagnosis-and-implications-for-management
#19
Angela E Gallucci, Meghan R Grewal, Brooke T Alexander, Arianna M Heyer, Yvonne M Diaz
Left ventricular non-compaction (LVNC) cardiomyopathy is a condition with increasing prevalence as cardiac imaging technology improves, although there is currently no diagnostic gold standard. Characterized by the presence of a bilayered myocardium with prominent trabeculations, LVNC cardiomyopathy has a wide range of presentations, from asymptomatic to severe heart failure, thromboembolism, and sudden cardiac death. We present the case of a 62-year-old male who was admitted for a heart failure exacerbation with a worsening ejection fraction and signs of increased trabeculations of the left ventricle on an echocardiogram...
October 2023: Curēus
https://read.qxmd.com/read/38019448/the-trouble-with-trabeculation-how-genetics-can-help-to-unravel-a-complex-and-controversial-phenotype
#20
REVIEW
Roddy Walsh
Excessive trabeculation of the cardiac left ventricular wall is a complex phenotypic substrate associated with various physiological and pathological processes. There has been considerable conjecture as to whether hypertrabeculation contributes to disease and whether left ventricular non-compaction (LVNC) cardiomyopathy is a distinct pathology. Building on recent insights into the genetic basis of LVNC cardiomyopathy, in particular three meta-analysis studies exploring genotype-phenotype associations using different methodologies, this review examines how genetic research can advance our understanding of trabeculation...
November 29, 2023: Journal of Cardiovascular Translational Research
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