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https://www.readbyqxmd.com/read/29224255/the-highest-mortality-rates-in-childhood-dilated-cardiomyopathy-occur-during-the-first-year-after-diagnosis
#1
Shalan Fadl, Håkan Wåhlander, Katja Fall, Yang Cao, Jan Sunnegårdh
AIM: The aim of the study was to assess the incidence, mortality and morbidity of dilated cardiomyopathy (DCM) and non-compaction of the left ventricle (LVNC) in Swedish children. METHODS: We reviewed hospital records of all children with dilated cardiomyopathy (DCM) or left ventricular non-compaction cardiomyopathy (LVNC) up to the age of 18 in the healthcare region of western Sweden from 1991 to 2015. RESULTS: In total, 69 cases (61% males) were identified...
December 9, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/29214778/supplementary-diagnostic-landmarks-of-left-ventricular-non-compaction-on-magnetic-resonance-imaging
#2
Marko Boban, Vladimir Pesa, Natko Beck, Sime Manola, Marinko Zulj, Ante Rotim, Aleksandar Vcev
PURPOSE: Diagnostic criteria for left ventricular non-compaction (LVNC) are still a matter of dispute. The aim of our present study was to test the diagnostic value of two novel diagnostic cardiac magnetic resonance (CMR) parameters: proof of non-compact (NC) myocardium blood flow using T2 sequences and changes in geometry of the left ventricle. MATERIALS AND METHODS: The study included cases with LVNC and controls, from a data base formed in a period of 3.5 years (n=1890 exams), in which CMR protocol included T2 sequences...
January 2018: Yonsei Medical Journal
https://www.readbyqxmd.com/read/29207943/auxiliary-diagnostic-potential-of-ventricle-geometry-and-late-gadolinium-enhancement-in-left-ventricular-non-compaction-non-randomized-case-control-study
#3
Marko Boban, Vladimir Pesa, Ivo Darko Gabric, Sime Manola, Viktor Persic, Helena Antic-Kauzlaric, Marinko Zulj, Aleksandar Vcev
BACKGROUND: There are still ambiguities existing in regard to left ventricular non-compaction (LVNC) diagnostic imaging. The aim of our study was to analyze diagnostic potential of late gadolinium enhancement (LGE) and ventricle geometry in patients with LVNC and controls. METHODS: Data on cardiac magnetic resonance imaging (CMR) studies for LVNC were reassessed from the hospital's database (3.75 years; n=1975 exams). Matching sample of controls included cases with no structural heart disease, hypertrophic or dilative cardiomyopathy, arrhythmogenic right ventricular dysplasia or subacute myocarditis...
December 6, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/29203757/variant-of-myocardial-infarction-course-in-the-patient-with-left-ventricular-non-compaction
#4
Natalia Y Osovska, Oleksandr I Datsyuk, Natalia V Kuzminova, Yevhen V Shaprynskyi, Hennadii V Bevz, Yaroslav M Pashynskyy, Oleksandr D Bondarchuk, Olena V Sergiichuk, Yaroslav V Karyi, Galina M Mazur
Non-compacted left ventricle in adults is a rare occurrence, though it is diagnosed even more rarely. As a rule in patients with non-compacted left ventricle (LVNC) other pathologic condition is diagnosed, notably hypertrophic or dilated cardiomyopathy. The majority of LVNC cases are diagnosed in early infancy but currently there are asymptomatic cases detected by means of echocardiographic examination. Real prevalence of LVNC is unknown. According to many authors LVNC occurs in 9.2-9.5% of children with diagnosed cardiomyopathies...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29188317/genetic-testing-in-pediatric-cardiomyopathy
#5
Chalani D Ellepola, Linda M Knight, Peter Fischbach, Shriprasad R Deshpande
Genetic testing is recommended in patients with dilated cardiomyopathy (DCM); however, limited studies demonstrate high yields of genetic testing in non-hypertrophic (HCM) patients. Furthermore, there is sparse genotype-phenotype data in pediatric DCM patients. We performed a retrospective review of 70 consecutive probands with cardiomyopathy (non-HCM) who underwent genetic evaluation. Mean age at presentation was 5.48 years. Echocardiography revealed mean ejection fraction of 32.4%. The LVEDd z score ranged from - 5...
November 29, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29173988/non-compaction-cardiomyopathy-a-short-review-of-a-controversial-entity
#6
REVIEW
Rebeca Lorca, José Rozado, María Martín
Non-compaction cardiomyopathy is a heterogeneous and complex entity concerning which there are still many doubts to be resolved. While the American Heart Association includes it among genetic cardiomyopathies, the European Society of Cardiology treats it as an unclassified cardiomyopathy. It may present in a sporadic or familial form, isolated or associated with other heart diseases, affecting only the left ventricle or both and can sometimes appear as a mixed phenotype in patients with other cardiomyopathies...
November 22, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/29140423/ventricular-assist-device-implantation-in-a-young-patient-with-non-compaction-cardiomyopathy-and-hereditary-spherocytosis
#7
Katharina Huenges, Bernd Panholzer, Jochen Cremer, Assad Haneya
A case of a 15-year-old female patient with acute heart failure due to non-compaction cardiomyopathy and hereditary anaemia (hereditary spherocytic elliptocytosis) requiring ventricular assist device implantation as a bridge to transplantation is presented. The possible effects of mechanical stress on erythrocytes potentially induced by mechanical circulatory support remains unclear, but it may lead to haemolytic crisis in patients suffering from hereditary anaemia. In our case, ventricular assist device therapy was feasible, and haematological complications did not occur within 6 weeks of bridging our patient to heart transplantation...
November 10, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29124903/relationship-between-the-left-ventricular-size-and-the-amount-of-trabeculations
#8
Bruno Paun, Bart Bijnens, Constantine Butakoff
Contemporary imaging modalities offer non-invasive quantification of myocardial deformation; however, they make gross assumptions about internal structure of the cardiac walls. Our aim is to study the possible impact of the trabeculations on the stroke volume, strain and capacity of differently sized ventricles. The cardiac left ventricle is represented by an ellipsoid and the trabeculations by a tissue occupying a fixed volume. The ventricular contraction is modelled by scaling the ellipsoid whereupon the measurements of longitudinal strain, end-diastolic, end-systolic and stroke volume are derived and compared...
November 9, 2017: International Journal for Numerical Methods in Biomedical Engineering
https://www.readbyqxmd.com/read/29118297/phenotype-and-functional-analyses-in-a-transgenic-mouse-model-of-left-ventricular-noncompaction-caused-by-a-dtna-mutation
#9
Qing Cao, Yang Shen, Xin Liu, Xin Yu, Ping Yuan, Rong Wan, Xiuxia Liu, Xiaogang Peng, Wenfeng He, Jielin Pu, Kui Hong
DTNA encoding dystrobrevin-α (α-DB) is a putative causal gene associated with left ventricular noncompaction cardiomyopathy (LVNC). The aim of the study was to investigate the causal role of DTNA in LVNC using a transgenic mouse model.A missense mutation (c.146A > G, p.N49S) of DTNA was identified in a patient with LVNC by Sanger sequencing. Six independent lines of transgenic mice expressing the mutant DTNA under a myosin heavy chain 6 (Myh6) promoter were generated (Myh6:Dtna(N49S)). Phenotypic characteristics of DTNA-p...
November 8, 2017: International Heart Journal
https://www.readbyqxmd.com/read/29102365/pregnancy-in-women-with-a-cardiomyopathy-outcomes-and-predictors-from-a-retrospective-cohort
#10
Gilles Billebeau, Martin Etienne, Riadh Cheikh-Khelifa, Daniele Vauthier-Brouzes, Estelle Gandjbakhch, Richard Isnard, Jacky Nizard, Michel Komajda, Marc Dommergues, Philippe Charron
BACKGROUND: Pregnancies in women with pre-existing cardiomyopathies are considered at high risk for complications. However, few data are available to characterize their natural history and predict the outcome. AIMS: Our aim was to evaluate the prevalence and predictors of acute cardiac and obstetric events in women with a cardiomyopathy during pregnancy, excluding peripartum cardiomyopathy. METHODS: In this retrospective study in a referral centre for cardiomyopathies, we included 43 consecutive pregnancies in 36 women with dilated, hypertrophic, arrhythmogenic right ventricular or tachycardia-induced cardiomyopathy, or left ventricular non-compaction...
October 25, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/29037169/left-ventricular-non-compaction-cardiomyopathy-with-coronary-artery-anomaly-complicated-by-ventricular-tachycardia
#11
Gustav Mattsson, Abdullah Baroudi, Hoshmand Tawfiq, Peter Magnusson
BACKGROUND: Non-compaction cardiomyopathy (NCCM) is characterized by prominent trabeculations, deep intertrabecular recesses, and a thick non-compacted endocardial myocardium. Prevalence in the general population remains unclear, but echocardiography series report 0.05%. During fetal development muscle fibers and trabeculae should compact into a solid myocardium and when this fails, NCCM occurs. The condition is genetic, even though acquired forms have been described. Worsening myocardial dysfunction may lead to heart failure and/or arrhythmias...
October 16, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/29030363/isolated-left-ventricular-non-compaction-lvnc-and-recurrent-strokes-to-anticoagulate-or-not-to-anticoagulate-that-is-the-question
#12
Ahmed Subahi, Abubaker A I Hassan, Hossam Abubakar, Walid Ibrahim
Isolated left ventricular non-compaction (LVNC) is an uncommon primary cardiomyopathy associated with significant risk of thromboembolic stroke. We report a case of a 69-year-old man with a medical history of ischaemic stroke who presented with a stroke for the second time, and during stroke workup transthoracic echo was suggestive of increased apical trabeculation. He underwent cardiac MRI study to evaluate the left ventricular structure, which revealed LVNC cardiomyopathy, which we believe is the main culprit of his recurrent strokes...
October 13, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29029073/clinical-genetics-and-outcome-of-left-ventricular-non-compaction-cardiomyopathy
#13
Farbod Sedaghat-Hamedani, Jan Haas, Feng Zhu, Christian Geier, Elham Kayvanpour, Martin Liss, Alan Lai, Karen Frese, Regina Pribe-Wolferts, Ali Amr, Daniel Tian Li, Omid Shirvani Samani, Avisha Carstensen, Diana Martins Bordalo, Marion Müller, Christine Fischer, Jing Shao, Jing Wang, Ming Nie, Li Yuan, Sabine Haßfeld, Christine Schwartz, Min Zhou, Zihua Zhou, Yanwen Shu, Min Wang, Kai Huang, Qiutang Zeng, Longxian Cheng, Tobias Fehlmann, Philipp Ehlermann, Andreas Keller, Christoph Dieterich, Katrin Streckfuß-Bömeke, Yuhua Liao, Michael Gotthardt, Hugo A Katus, Benjamin Meder
Aims: In this study, we aimed to clinically and genetically characterize LVNC patients and investigate the prevalence of variants in known and novel LVNC disease genes. Introduction: Left ventricular non-compaction cardiomyopathy (LVNC) is an increasingly recognized cause of heart failure, arrhythmia, thromboembolism, and sudden cardiac death. We sought here to dissect its genetic causes, phenotypic presentation and outcome. Methods and results: In our registry with follow-up of in the median 61 months, we analysed 95 LVNC patients (68 unrelated index patients and 27 affected relatives; definite familial LVNC = 23...
October 6, 2017: European Heart Journal
https://www.readbyqxmd.com/read/29024827/left-ventricular-non-compaction-with-ebstein-anomaly-attributed-to-a-tpm1-mutation
#14
Aleksandra Nijak, Maaike Alaerts, Cuno Kuiperi, Anniek Corveleyn, Bert Suys, Bernard Paelinck, Johan Saenen, Emeline Van Craenenbroeck, Lut Van Laer, Bart Loeys, Aline Verstraeten
Left ventricular non-compaction (cardiomyopathy) (LVN(C)) is a rare hereditary cardiac condition, resulting from abnormal embryonic myocardial development. While it mostly occurs as an isolated condition, association with other cardiovascular manifestations such as Ebstein anomaly (EA) has been reported. This congenital heart defect is characterized by downward displacement of the tricuspid valve and leads to diminished ventricular size and function. In an autosomal dominant LVN(C) family consisting of five affected individuals, of which two also presented with EA and three with mitral valve insufficiency, we pursued the genetic disease cause using whole exome sequencing (WES)...
October 9, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29024504/high-validity-of-cardiomyopathy-diagnoses-in-western-sweden-1989-2009
#15
Carmen Basic, Annika Rosengren, Sandra Lindström, Maria Schaufelberger
AIM: Hospital discharges with a diagnosis of cardiomyopathy have more than doubled in Sweden since 1987. We validated the cardiomyopathy diagnoses over this time period to investigate that the increase was real and not a result of improved recognition of the diagnosis and better diagnostic methods. METHODS AND RESULTS: Every fifth year from 1989 to 2009, records for all patients with a cardiomyopathy diagnosis were identified by searching the local registers in three hospitals in Västra Götaland, Sweden...
October 11, 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28957389/a-new-insight-into-the-three-dimensional-architecture-of-the-golgi-complex-characterization-of-unusual-structures-in-epididymal-principal-cells
#16
Narcisa Martínez-Martínez, Emma Martínez-Alonso, Mónica Tomás, Josef Neumüller, Margit Pavelka, José A Martínez-Menárguez
Principal epididymal cells have one of the largest and more developed Golgi complex of mammalian cells. In the present study, we have used this cell as model for the study of the three-dimensional architecture of the Golgi complex of highly secretory and endocytic cells. Electron tomography demonstrated the presence in this cell type of some unknown or very unusual Golgi structures such as branched cisternae, pocket-like cisternal invaginations or tubular connections. In addition, we have used this methodology and immunoelectron microscopy to analyze the close relationship between this organelle and both the endoplasmic reticulum and microtubules, and to describe in detail how these elements interact with compact and non-compact regions of the ribbon...
2017: PloS One
https://www.readbyqxmd.com/read/28867022/cardiac-magnetic-resonance-evaluation-of-left-ventricular-functional-morphological-and-structural-features-in-children-and-adolescents-vs-young-adults-with-isolated-left-ventricular-non-compaction
#17
Gaetano Nucifora, Karthigesh Sree Raman, Daniele Muser, Ranjit Shah, Rebecca Perry, Kama A Awang Ramli, Joseph B Selvanayagam
AIM: To investigate the left ventricular (LV) functional, morphological, and structural features revealed by cardiac magnetic resonance (CMR) in children/adolescents with isolated LV non-compaction (iLVNC), and to compare them with those observed in young adults with iLVNC and healthy controls. METHODS: 56 subjects were included: 12 children/adolescents (mean age 15±3years, 75% male) and 20 young adults (mean age 35±7years, 75% male) with first diagnosis of iLVNC, 12 healthy children/adolescents (mean age 15±3years, 75% male) and 12 healthy young adults (mean age 34±8years, 75% male)...
November 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28706583/cardiac-damage-in-athlete-s-heart-when-the-supernormal-heart-fails
#18
REVIEW
Andreina Carbone, Antonello D'Andrea, Lucia Riegler, Raffaella Scarafile, Enrica Pezzullo, Francesca Martone, Raffaella America, Biagio Liccardo, Maurizio Galderisi, Eduardo Bossone, Raffaele Calabrò
Intense exercise may cause heart remodeling to compensate increases in blood pressure or volume by increasing muscle mass. Cardiac changes do not involve only the left ventricle, but all heart chambers. Physiological cardiac modeling in athletes is associated with normal or enhanced cardiac function, but recent studies have documented decrements in left ventricular function during intense exercise and the release of cardiac markers of necrosis in athlete's blood of uncertain significance. Furthermore, cardiac remodeling may predispose athletes to heart disease and result in electrical remodeling, responsible for arrhythmias...
June 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28703272/left-ventricular-non-compaction-and-hypertrophic-cardiomyopathy-two-overlapping-diseases-or-two-manifestations-of-the-same-cardiomyopathy-response-to-the-letter-concerning-the-article-left-ventricular-non-compaction-associated-with-hypertrophic%C3%A2
#19
COMMENT
https://www.readbyqxmd.com/read/28703265/noncompaction-may-not-only-be-non-isolated-but-also-myopathic-commentary-to-the-article-left-ventricular-non-compaction-associated-with-hypertrophic-cardiomyopathy-in-the-same-patient
#20
COMMENT
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