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Left ventricular non compaction

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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/29124903/relationship-between-the-left-ventricular-size-and-the-amount-of-trabeculations
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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/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
#13
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
#14
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
#15
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
#16
COMMENT
https://www.readbyqxmd.com/read/28691389/fetal-left-ventricular-non-compaction-cardiomyopathy-with-ascites-a-case-report
#17
Junsuke Muraoka, Yuki Kodama, Hiroshi Sameshima, Kaori Michikata, Satoshi Matsuzawa, Oohashi Masanao, Masatoki Kaneko, Mayumi Akaki, Yuichiro Sato
Here we report a case of fetal left ventricular non-compaction cardiomyopathy with ascites and cardiac dysfunction at a gestational age of 34(+5)  weeks. Laboratory tests did not reveal any sign of viral infection in utero. A female neonate weighing 2436 g was delivered by emergency cesarean section due to non-reassuring fetal status. Postnatal echocardiography confirmed left ventricular non-compaction cardiomyopathy with severe cardiac failure. Although she was treated effectively during the acute period by continuous flow peritoneal dialysis, surgical ligation of a patent ductus arteriosus, and inhaled nitric oxide, she died on day 41 of life...
September 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28687944/prolonged-qtc-indicates-the-clinical-severity-and-poor-prognosis-in-patients-with-isolated-left-ventricular-non-compaction
#18
Hongmei Zhou, Xue Lin, Ligang Fang, Wenlin Zhu, Xihai Zhao, Haiyan Ding, Meng Jiang, Heng Ge, Quan Fang, Ben He
Isolated left ventricular non-compaction (LVNC) is a rare cardiomyopathy that leads to severe clinical complications. This study is to investigate whether or not prolonged QTc is a good indicator for evaluating the severity of fibrosis and predicting the prognosis of LVNC, and if native T1 can be used to quantify the fibrosis. 32 LVNC patients and 14 healthy controls with matched age and sex were examined by CMR and ECG to acquire native T1, QTc interval, and ECG abnormalities. 18 LVNC patients had normal QTc and 14 LVNC patients had prolonged QTc...
December 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28567093/cardiomyopathies-with-mixed-and-inapparent-morphological-features-in-cardiac-troponin-i3-mutation
#19
Dae-Won Sohn, Hyung-Kwan Kim, Yong-Jin Kim, Seil Oh, Moon-Woo Seong, Sung-Sup Park
The fact that different types of cardiomyopathies can be manifested by the same sarcomere protein gene mutation in a single family is well known. However, mixed features of different types of cardiomyopathies in a single patient have not been well appreciated. We identified a novel mutation in cardiac troponin I3 (Arg186Gly) in the present case, and two of the family members showed mixed morphologic features of hypertrophic cardiomyopathy and left ventricular non-compaction. Moreover, both the features of cardiomyopathies were not apparent for each type of cardiomyopathy...
May 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28510647/epicardial-cardioverter-defibrillator-implantation-in-a-4-month-old-infant-bridged-to-heart-transplantation
#20
Cristina Carro, Alberto Francesco Cereda, Giuseppe Annoni, Stefano Maria Marianeschi
Implantable cardioverter-defibrillator (ICD) is the gold standard therapy for the prevention of sudden cardiac death. Nevertheless, ICD placement in the paediatric population is still limited because of several technical difficulties. Several implantation techniques have been proposed but experience in infants with very low weight and less than 6 months is very limited. We herein describe a case of a minimally invasive ICD epicardial implantation in a 4-month-old infant weighing 5 kg. A diagnosis of arrhythmic cardiomyopathy with left ventricular non-compaction disease with ventricular tachycardia storms, QT prolongation and Wolff-Parkinson-White pattern was made...
November 1, 2017: Interactive Cardiovascular and Thoracic Surgery
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