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Left ventricular non compaction

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https://www.readbyqxmd.com/read/28706583/cardiac-damage-in-athlete-s-heart-when-the-supernormal-heart-fails
#1
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
#2
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
#3
https://www.readbyqxmd.com/read/28691389/fetal-left-ventricular-non-compaction-cardiomyopathy-with-ascites-a-case-report
#4
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...
July 10, 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
#5
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...
July 7, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28567093/cardiomyopathies-with-mixed-and-inapparent-morphological-features-in-cardiac-troponin-i3-mutation
#6
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
#7
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...
May 16, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28510120/obscurin-variants-and-inherited-cardiomyopathies
#8
REVIEW
Steven Marston
The inherited cardiomyopathies, hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM) and left ventricular non-compaction (LVNC), have been frequently associated with mutations in sarcomeric proteins. In recent years, advances in DNA sequencing technology has allowed the study of the giant proteins of the sarcomere, such as titin and nebulin. Obscurin has been somewhat neglected in these studies, largely because its functional role is far from clear, although there was an isolated report in 2007 of obscurin mutations associated with HCM...
June 2017: Biophysical Reviews
https://www.readbyqxmd.com/read/28458700/echocardiographic-characteristics-of-isolated-left-ventricular-noncompaction
#9
Afsoon Fazlinezhad, Mohammad Vojdanparast, Shadi Sarafan, Pouya Nezafati
BACKGROUND: Although isolated left ventricular noncompaction (ILVNC) has been described almost two decades ago, our knowledge about its diagnosis, presentation, echocardiographic features and clinical outcome is sparse. We aimed to assess the echocardiographic and clinical characteristics of ILVNC in a group of patients referred to our center. METHODS: Patients who were referred to a tertiary referral center, affiliated with Mashhad University of Medical Sciences, with primary diagnosis of dilated cardiomyopathy underwent comprehensive echocardiographic evaluation...
September 2016: ARYA Atherosclerosis
https://www.readbyqxmd.com/read/28421585/left-ventricular-non-compaction-associated-with-hypertrophic-cardiomyopathy-in-the-same-patient
#10
Lobna Laaroussi, Afef Ben Halima, Marouane Boukhris, Faouzi Addad, Salem Kachboura
No abstract text is available yet for this article.
2017: Kardiologia Polska
https://www.readbyqxmd.com/read/28381694/aortopathy-in-an-adult-with-tricuspid-atresia-and-left-ventricular-non-compaction-after-fontan-procedure
#11
Tomotaka Murakami, Yoshiki Mori, Nao Inoue, Sachie Kaneko, Yasumi Nakashima
No abstract text is available yet for this article.
April 5, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28379356/decreased-glycolytic-metabolism-in-non-compaction-cardiomyopathy-by-18f-fluoro-2-deoxyglucose-positron-emission-tomography-new-insights-into-pathophysiological-mechanisms-and-clinical-implications
#12
Marcelo Dantas Tavares de Melo, Maria Clementina Pinto Giorgi, Antonildes Nascimento Assuncao, Roberto Nery Dantas, Jose de Arimateia Araujo Filho, Jose Rodrigues Parga Filho, Ana Luiza de Souza Bierrenbach, Camila Rocon de Lima, José Soares, José Claudio Meneguetti, Charles Mady, Ludhmila Abrahão Hajjar, Roberto Kalil Filho, Edimar Alcides Bocchi, Vera Maria Cury Salemi
Aims: The pathophysiological mechanisms of left ventricular non-compaction cardiomyopathy (LVNC) remain controversial. This study performed combined 18F-fluoro-2-deoxyglucose dynamic positron emission tomography (FDG-PET) and 99mTc-sestamibi single-photon emission computed tomography (SPECT) studies to evaluate myocardial glucose metabolism and perfusion in patients with LVNC and their clinical implications. Methods and results: Thirty patients (41 ± 12 years, 53% male) with LVNC, diagnosed by cardiovascular magnetic resonance (CMR) criteria, and eight age-matched healthy controls (42 ± 12 years, 50% male) were prospectively recruited to undergo FDG-PET with measurement of the myocardial glucose uptake rate (MGU) and SPECT to investigate perfusion-metabolism patterns...
March 30, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28369760/clinical-genetic-testing-in-pediatric-cardiomyopathy-is-bigger-better
#13
A C Ouellette, J Mathew, A K Manickaraj, G Manase, L Zahavich, J Wilson, K George, L Benson, S Bowdin, S Mital
BACKGROUND: For clinical genetic testing of cardiomyopathy (CMP), current guidelines do not address which gene panels to use: targeted panels specific to a CMP phenotype or expanded (panCMP) panels that include genes associated with multiple phenotypic subtypes. AIM: Our objective was to assess the clinical utility of targeted versus panCMP panel testing in pediatric CMPs. METHODS: 151 pediatric patients with primary hypertrophic (n = 66), dilated (n = 64), restrictive (n = 8), or left-ventricular non-compaction (n = 13) CMP who underwent clinical genetic panel testing at a single centre were included...
March 30, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28343948/left-ventricular-non-compaction-a-cardiomyopathy-with-acceptable-prognosis-in-children
#14
Tao Tian, Yankun Yang, Lanlan Zhou, Fang Luo, Yuehua Li, Peng Fan, Xueqi Dong, Yaxin Liu, Jingang Cui, Xianliang Zhou
BACKGROUND: Data on children with left ventricular non-compaction (LVNC) is sparse. The purpose of this study was to evaluate its clinical profiles in a population of Chinese children. METHODS: From January 2010 to March 2016, consecutive Chinese children (aged <18 years) with LVNC diagnosed by cardiovascular magnetic resonance (CMR) were prospectively recruited at Fuwai Hospital. RESULTS: A total of 41 Chinese children (male: 28%; mean age: 14±4years) were included in this study...
March 1, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28315121/untangling-the-biology-of-genetic-cardiomyopathies-with-pluripotent-stem-cell-disease-models
#15
REVIEW
Jan W Buikema, Sean M Wu
PURPOSE OF REVIEW: Recently, the discovery of strategies to reprogram somatic cells into induced pluripotent stem (iPS) cells has led to a major paradigm change in developmental and stem cell biology. The application of iPS cells and their cardiac progeny has opened novel directions to study cardiomyopathies at a cellular and molecular level. This review discusses approaches currently undertaken to unravel known inherited cardiomyopathies in a dish. RECENT FINDINGS: With improved efficiency for mutation correction by genome editing, human iPS cells have now provided a platform to untangle the biology of cardiomyopathies...
April 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28268080/prognostic-power-of-nt-probnp-in-left-ventricular-non-compaction-cardiomyopathy
#16
Simon F Stämpfli, Ladina Erhart, Niels Hagenbuch, Barbara E Stähli, Christiane Gruner, Matthias Greutmann, Markus Niemann, Beat A Kaufmann, Rolf Jenni, Leonhard Held, Felix C Tanner
BACKGROUND: The risk of adverse events in patients with left ventricular non-compaction cardiomyopathy (LVNC) is substantial. This study was designed to determine the prognostic value of NT-proBNP, left ventricular ejection fraction (LVEF), NYHA class, and exercise capacity in LVNC patients. METHODS: Cox regression analyses were performed for evaluating the prognostic value of NT-proBNP, LVEF, NYHA class, and exercise capacity on the occurrence of death or heart transplantation...
February 24, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28258649/cardiac-manifestations-of-inherited-metabolic-disease-in-children
#17
REVIEW
David Fa Lloyd, Roshni Vara, Sujeev Mathur
Inborn errors of metabolism (IEM) are responsible for around 5% of all cases of cardiomyopathy (CM) and for 15% of non-idiopathic cases. Storage disorders such as Pompe disease (glycogen storage disease type II) typically cause hypertrophic CM, whereas the accumulation of toxic metabolites, as seen in the organic acidurias, is associated with dilated cardiomyopathy (DCM). Mixed pathology is also possible, particularly in late presentations. IEM such as Barth syndrome, a disorder of cardiolipin stability usually associated with DCM, have been associated with rarer types of CM such as endocardial fibroelastosis and left ventricular non-compaction...
May 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28110572/-cardiomyopathy-and-ion-channel-diseases-registry-the-szeged-cardiogen-registry
#18
Péter Blazsó, Kornél Kákonyi, Tamás Forster, Róbert Sepp
The Szeged cardiomyopathy and ion channel diseases registry aims to establish a representative disease-specific registry based on the recruitment of patients with different cardiomyopathies and ion channel diseases followed at the Cardiology Center, University of Szeged. The registry collects patient data on the main forms of primary cardiomyopathies (hypertrophic, dilated, restrictive, arrhythmogenic right ventricular, left ventricular non-compact, tako-tsubo cardiomyopathy) and ion channel diseases (long QT syndrome, short QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia)...
January 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28104484/sick-sinus-syndrome-with-hcn4-mutations-shows-early-onset-and-frequent-association-with-atrial-fibrillation-and-left-ventricular-noncompaction
#19
Taisuke Ishikawa, Seiko Ohno, Takashi Murakami, Kentaro Yoshida, Hiroyuki Mishima, Tetsuya Fukuoka, Hiroki Kimoto, Risa Sakamoto, Takafumi Ohkusa, Takeshi Aiba, Akihiko Nogami, Naokata Sumitomo, Wataru Shimizu, Koh-Ichiro Yoshiura, Hitoshi Horigome, Minoru Horie, Naomasa Makita
BACKGROUND: Familial sick sinus syndrome (SSS) is often attributable to mutations in genes encoding the cardiac Na channel SCN5A and pacemaker channel HCN4. We previously found that SSS with SCN5A mutations shows early onset of manifestations and male predominance. Despite recent reports on the complications of atrial fibrillation (AF) and left ventricular noncompaction (LVNC) in patients with SSS caused by HCN4 mutations, their overall clinical spectrum remains unknown. OBJECTIVE: The purpose of this study was to investigate the clinical and demographic features of SSS patients carrying HCN4 mutations...
May 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28102598/novel-findings-of-left-ventricular-non-compaction-cardiomyopathy-microform-cleft-lip-and-poor-vision-in-patient-with-smc1a-associated-cornelia-de-lange-syndrome
#20
Tara L Wenger, Penny Chow, Stephanie C Randle, Anna Rosen, Craig Birgfeld, Joanna Wrede, Patrick Javid, Darcy King, Vivian Manh, Anne V Hing, Erin Albers
Relatively few patients with Cornelia de Lange syndrome (CdLS) due to SMC1A mutation have been reported, limiting understanding of the full extent of the phenotype. Compared to children with classic NIPBL-associated CdLS, patients with SMC1A-associated CdLS have a milder physical phenotype with prominent intellectual disability, high rate of cleft palate and absence of limb reductions. We present a patient with SMC1A-associated CdLS who had typical features including developmental delay, seizure disorder, feeding difficulties, hirsutism, and cleft palate...
February 2017: American Journal of Medical Genetics. Part A
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