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left ventricular noncompaction

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https://www.readbyqxmd.com/read/28183324/identification-of-taz-mutations-in-pediatric-patients-with-cardiomyopathy-by-targeted-next-generation-sequencing-in-a-chinese-cohort
#1
Jian Wang, Ying Guo, Meirong Huang, Zhen Zhang, Junxue Zhu, Tingliang Liu, Lin Shi, Fen Li, Huimin Huang, Lijun Fu
BACKGROUND: Barth syndrome (BTHS) is a rare X-linked recessive disease characterized by cardiomyopathy, neutropenia, skeletal myopathy and growth delay. Early diagnosis and appropriate treatment may improve the prognosis of this disease. The purpose of this study is to determine the role of targeted next-generation sequencing (NGS) in the early diagnosis of BTHS in children with cardiomyopathy. METHODS: During the period between 2012 and 2015, a gene panel-based NGS approach was used to search for potentially disease-causing genetic variants in all patients referred to our institution with a clinical diagnosis of primary cardiomyopathy...
February 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28158907/naturally-occurring-biventricular-noncompaction-in-an-adult-domestic-cat
#2
M D Kittleson, P R Fox, C Basso, G Thiene
A definitively diagnosed case of left ventricular noncompaction (LVNC) has not been previously reported in a non-human species. We describe a Maine Coon cross cat with echocardiographically and pathologically documented LVNC. The cat was from a research colony and was heterozygous for the cardiac myosin binding protein C mutation associated with hypertrophic cardiomyopathy (HCM) in Maine Coon cats (A31P). The cat had had echocardiographic examinations performed every 6 months until 6 years of age at which time the cat died of an unrelated cause...
February 3, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28154298/long-term-prognosis-of-patients-with-left-ventricular-noncompaction%C3%A3-comparison-between-infantile-and-juvenile-types
#3
Ce Wang, Asami Takasaki, Sayaka Watanabe Ozawa, Hideyuki Nakaoka, Mako Okabe, Nariaki Miyao, Kazuyoshi Saito, Keijiro Ibuki, Keiichi Hirono, Naoki Yoshimura, Xianyi Yu, Fukiko Ichida
BACKGROUND: The natural history of left ventricular noncompaction (LVNC) is largely unsolved, so the aim of the present study was to clarify the clinical features and long-term prognosis of children with LVNC until adulthood.Methods and Results:We conducted a nationwide survey over 20 years and compared the clinical features, anatomical characteristics and long-term prognosis of 205 patients divided into 2 classifications: infantile type (diagnosed at <1 year of age: 108 cases) and juvenile type (diagnosed 1-15 years of age: 97 cases)...
February 2, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28108756/left-ventricular-noncompaction-cardiomyopathy-in-pediatric-patients-a-case-series-of-a-clinically-heterogeneous-disease
#4
Umang Gupta, Pooja Makhija
Left ventricular noncompaction is a rare form of cardiomyopathy, which results from multiple trabeculations in the left ventricular myocardium. The clinical presentation is highly variable, and spectrum includes asymptomatic patients diagnosed during family screening on one end to patients with depressed systolic function, heart failure, thromboembolic complications, and cardiac arrhythmias on the other (Kim et al in J Am Coll Cardiol 53: 2009, 2009). Further, the progression of the condition is highly variable...
January 21, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28079110/left-ventricular-noncompaction-cardiomyopathy-cardiac-neuromuscular-and-genetic-factors
#5
REVIEW
Josef Finsterer, Claudia Stöllberger, Jeffrey A Towbin
Left ventricular hypertrabeculation (LVHT) or noncompaction is a myocardial abnormality of unknown aetiology, frequently associated with monogenic disorders, particularly neuromuscular disorders, or with chromosomal defects. LVHT is diagnosed usually by echocardiography by the presence of a bilayered myocardium consisting of a thick, spongy, noncompacted endocardial layer and a thin, compacted, epicardial layer. The pathogenesis of LVHT is unsolved, and the diagnostic criteria, prognosis, and optimal treatment of patients with LVHT are under debate...
January 12, 2017: Nature Reviews. Cardiology
https://www.readbyqxmd.com/read/28076895/implantation-of-left-ventricular-assist-device-in-a-patient-with-left-ventricular-non-compaction
#6
Keki R Balsara, Andrew Bierhals, Justin Vader, Michael K Pasque, Aki Itoh
Left ventricular noncompaction (LVNC) may result in systolic left ventricular (LV) failure resulting in the need for heart transplantation. LV assist devices (LVAD) have been used to bridge these patients to transplantation; however, the extensive trabeculations found in these patients predispose them to thromboembolic events and pump thrombosis. We describe a patient with LVNC in whom an aggressive surgical approach was used to debride the LV cavity of trabeculations to successfully implant an LVAD.
January 11, 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/27989498/recent-advancements-in-the-molecular-genetics-of-left-ventricular-noncompaction-cardiomyopathy
#7
REVIEW
Xueqi Dong, Peng Fan, Tao Tian, Yankun Yang, Yan Xiao, Kunqi Yang, Yaxin Liu, Xianliang Zhou
Left ventricular noncompaction cardiomyopathy (LVNC) is a myocardial disorder characterized by prominent and excessive trabeculations with deep recesses in the ventricular wall. Clinical manifestations of LVNC are highly variable, ranging from no symptoms to arrhythmias, heart failure, thromboembolism, or even sudden cardiac death. It is a heterogenetic disease which can be presented as an autosomal, X-linked or mitochondrial disorder. A series of candidate mutations have been identified in LVNC patients or murine models...
February 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/27855807/left-ventricular-noncompaction-or-is-it
#8
EDITORIAL
Nay Aung, Filip Zemrak, Steffen E Petersen
No abstract text is available yet for this article.
November 15, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27855806/long-term-prognostic-value-of-cardiac%C3%A2-magnetic-resonance-in-left%C3%A2-ventricle-noncompaction-a-prospective-multicenter-study
#9
Daniele Andreini, Gianluca Pontone, Jan Bogaert, Alberto Roghi, Andrea Barison, Juerg Schwitter, Saima Mushtaq, Georgios Vovas, Paola Sormani, Giovanni D Aquaro, Pierre Monney, Chiara Segurini, Marco Guglielmo, Edoardo Conte, Laura Fusini, Antonio Dello Russo, Massimo Lombardi, Paola Gripari, Andrea Baggiano, Cesare Fiorentini, Federico Lombardi, Antonio L Bartorelli, Mauro Pepi, Pier Giorgio Masci
BACKGROUND: Cardiac magnetic resonance (CMR) is useful for the diagnosis of left ventricular noncompaction (LVNC). However, there are limited data regarding its prognostic value. OBJECTIVES: The goal of this study was to evaluate the prognostic relevance of CMR findings in patients with LVNC. METHODS: A total of 113 patients with an echocardiographic diagnosis of LVNC underwent CMR at 5 referral centers. CMR diagnostic criterion of LVNC (noncompacted/compacted ratio >2...
November 15, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27855805/left-ventricular-noncompaction-anatomical-phenotype-or-distinct-cardiomyopathy
#10
Jonathan R Weir-McCall, Phey Ming Yeap, Carla Papagiorcopulo, Kerrie Fitzgerald, Stephen J Gandy, Matthew Lambert, Jill J F Belch, Ian Cavin, Roberta Littleford, Jennifer A Macfarlane, Shona Z Matthew, R Stephen Nicholas, Allan D Struthers, Frank Sullivan, Shelley A Waugh, Richard D White, J Graeme Houston
BACKGROUND: There is considerable overlap between left ventricular noncompaction (LVNC) and other cardiomyopathies. LVNC has been reported in up to 40% of the general population, raising questions about whether it is a distinct pathological entity, a remodeling epiphenomenon, or merely an anatomical phenotype. OBJECTIVES: The authors determined the prevalence and predictors of LVNC in a healthy population using 4 cardiac magnetic resonance imaging diagnostic criteria...
November 15, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27843800/left-ventricular-noncompaction-diagnosed-following-graves-disease
#11
Habib Habib, Amer Hawatmeh, Upamanyu Rampal, Fayez Shamoon
Isolated left ventricular noncompaction (LVNC) is a rare genetic cardiomyopathy. Clinical manifestations are variable; patients may present with heart failure symptoms, arrhythmias, and systemic thromboembolism. However, it can also be asymptomatic. When asymptomatic, LVNC can manifest later in life after the onset of another unrelated condition. We report a case of LVNC which was diagnosed following a hyperthyroid state secondary to Graves' disease. The association of LVNC with other noncardiac abnormalities including neurological, hematological, and endocrine abnormalities including hypothyroidism has been described in isolated case reports before...
October 2016: Avicenna Journal of Medicine
https://www.readbyqxmd.com/read/27842951/suspected-malignant-hyperthermia-during-biventricular-assist-device-implantation-in-a-patient-with-left-ventricular-noncompaction-cardiomyopathy
#12
Karsten Schmidt, Anke Missler, Christoph Lichtenstern, Thorsten Brenner, Bastian Schmack, Arjang Ruhparwar, Markus A Weigand, Stefan Hofer
No abstract text is available yet for this article.
August 21, 2016: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/27830773/grey-zones-in-cardiomyopathies-defining-boundaries-between-genetic-and-iatrogenic-disease
#13
REVIEW
Giovanni Quarta, Michael Papadakis, Paolo Di Donna, Niccolò Maurizi, Attilio Iacovoni, Antonello Gavazzi, Michele Senni, Iacopo Olivotto
Genetic cardiomyopathies are complex diseases with heterogeneous clinical presentation and phenotypes. Early descriptions of cardiomyopathies originated from case studies involving individuals with severe, paradigmatic presentation, which provided insight into the worst-case scenarios of these conditions. With time, improved diagnostic sensitivity and awareness of cardiomyopathies has uncovered a more heterogeneous disease spectrum, including mild phenotypes overlapping with physiological variation. This diagnostic 'grey area' poses important dilemmas, particularly in athletes...
November 10, 2016: Nature Reviews. Cardiology
https://www.readbyqxmd.com/read/27798758/incremental-value-of-contrast-echocardiography-in-the-diagnosis-of-left-ventricular-noncompaction
#14
Xiaoxiao Zhang, Li Yuan, Linli Qiu, Yali Yang, Qing Lv, Lin Li, Jing Wang, Lin He, Li Zhang, Xinfang Wang, Mingxing Xie, Xu Yu Jin
Contrast echocardiography with left ventricular opacification (LVO) improves the definition of endocardium in two-dimensional echocardiography (2DE). This study was aimed to determine whether LVO offered added diagnostic value in noncompaction of left ventricular myocardium (NCVM). A total of 85 patients (40 ± 20 years, 54 males) with suspected NCVM were subjected to transthoracic 2DE and LVO, and 40 healthy volunteers were examined with 2DE and assigned as control subjects. The location of NCVM, the thickness ratio of noncompacted to compacted myocardium (NCR), and the cavity size and ejection fraction of LV were quantified...
October 25, 2016: Frontiers of Medicine
https://www.readbyqxmd.com/read/27773363/-heart-transplantation-for-the-treatment-of-isolated-left-ventricular-myocardial-noncompaction-first-case-in-mexico
#15
Hugo Jesús Zetina-Tun, Guillermo Careaga-Reyna, José Galván-Díaz, Magdalena Sánchez-Uribe
BACKGROUND: Myocardial noncompaction of the left ventricle is a congenital cardiomyopathy characterised by left ventricular hypertrabeculation and prominent intertrabecular recesses. The incidence ranges from 0.15% to 2.2%. Clinical manifestations include heart failure, arrhythmias, and stroke. Prognosis is fatal in most cases. Heart transplantation is a therapeutic option for this cardiomyopathy, and few had been made worldwide. CLINICAL CASE: The case is presented of a 20 year-old male with noncompacted myocardium of the left ventricle, who had clinical signs of heart failure...
October 20, 2016: Cirugia y Cirujanos
https://www.readbyqxmd.com/read/27751310/a-rare-malady-with-a-rarer-complication
#16
Arun Gopi, Placid Sebastian, C D Ramakrishna
Isolated ventricular noncompaction (IVNC) is an unclassified cardiomyopathy characterized by prominent intraventricular trabeculations separated by deep intertrabecular recesses. Although microvascular dysfunction is known, myocardial infarction is rare and usually seen as a consequence of coincidental coronary artery disease. We report the case of a 19-year-old male patient who presented to us with symptoms and signs of heart failure. Echocardiography revealed IVNC with severe left ventricular dysfunction...
September 2016: Indian Heart Journal
https://www.readbyqxmd.com/read/27751274/a-cortical-stroke-secondary-to-an-isolated-left-ventricular-noncompaction-in-a-29-year-old-female
#17
Hany Younan, Raed Alroughani, Raouf Guindi
No abstract text is available yet for this article.
September 2016: Indian Heart Journal
https://www.readbyqxmd.com/read/27749293/left-ventricular-assist-device-insertion-in-a-patient-with-biventricular-noncompaction-cardiomyopathy-ebstein-anomaly-and-a-left-atrial-mass-a-case-report
#18
Nikhil Kumar, Christopher A Troianos, Joshua S Baisden
In this report, we present the case of a patient with biventricular noncompaction cardiomyopathy, Ebstein anomaly, and a left atrial mass who required emergent placement of a left ventricular assist device. The noncompaction cardiomyopathy complicated the left ventricular assist device implantation procedure because the thickened, trabeculated myocardium made it difficult to place the inflow cannula. We discuss our perioperative management strategy, in which transesophageal echocardiography was used, to help the surgical team identify the proper cannula placement and provide a bridge to transplantation...
December 15, 2016: A & A Case Reports
https://www.readbyqxmd.com/read/27728033/a-case-of-isolated-left-ventricular-noncompaction
#19
Tejaswi Jallepalli
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27688059/primary-cardiac-hydatid-cyst-presenting-as-noncompaction-cardiomyopathy
#20
Gonenc Kocabay, Serdar Yilmazer, Yetkin Korkmaz, Asli Karadeniz, Nilufer Eksi Duran
We report a case of cyst was initially labeled as left ventricular noncompaction cardiomyopathy. An accurate diagnosis is essential to establish the most effective treatment strategy. In particular, echocardiographic examination assists in identifying the correct diagnosis. In this case, two-dimensional and three-dimensional echocardiography and computed tomography were used for definitive diagnosis of cardiac hydatid cyst.
January 2017: Echocardiography
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