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Ventricular noncompaction

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https://www.readbyqxmd.com/read/28400861/revision-of-echocardiographic-indications-and-findings-in-neurologically-ill-patients
#1
Claudia Stöllberger, Christian Wegner, Josef Finsterer
BACKGROUND AND OBJECTIVE: Little is known about the general indications for echocardiography and the prevalence of abnormalities detected by echocardiography in patients who are referred from a neurological department. Left ventricular hypertrabeculation/noncompaction (LVHT) is associated with neuromuscular disorders and embolism. The aim of the study was to assess the indications for echocardiography in patients from a neurological department and to review the cine-loops of the examinations in order to assess the frequency of abnormal echocardiographic findings with special regard to LVHT...
2017: Open Cardiovascular Medicine Journal
https://www.readbyqxmd.com/read/28395867/key-questions-relating-to-left-ventricular-noncompaction-cardiomyopathy-is-the-emperor-still-wearing-any-clothes
#2
REVIEW
Robert H Anderson, Bjarke Jensen, Timothy J Mohun, Steffen E Petersen, Nay Aung, Filip Zemrak, R Nils Planken, David H MacIver
The evidence is increasing that left ventricular noncompaction cardiomyopathy as it is currently defined does not represent a failure of compaction of pre-existing trabecular myocardium found during embryonic development to form the compact component of the ventricular walls. Neither is there evidence of which we are aware to favour the notion that the entity is a return to a phenotype seen in cold-blooded animals. It is also known that when seen in adults, the presence of excessive ventricular trabeculations does not portend a poor prognosis when the ejection fraction is normal, with the risks of complications such as arrhythmia and stroke being rare in this setting...
February 1, 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28373784/left-ventricular-noncompaction-a-rare-form-of-cardiomyopathy-revelation-modes-and-predictors-of-mortality-in-adults-through-23-cases
#3
Iliyasse Asfalou, Sanae Boulaamayl, Maha Raissouni, Najat Mouine, Mohamed Sabry, Jamal Kheyi, Nawal Doghmi, Aatif Benyass
OBJECTIVES: To describe modes of clinical presentation and echocardiographic, angiographic, and rhythmic features, and prognostic characteristics of left ventricular noncompaction cardiomyopathy (LVNC) in North African adults, through one of the first series in Morocco. BACKGROUND: LVNC is a rare congenital disorder, described for the first time by Engberding in 1984. The suspected diagnosis in thromboembolic, hemodynamic, or rhythm events requires both echocardiography and cardiovascular magnetic resonance (CMR)...
April 2017: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/28330612/association-between-mutations-in-the-nkx2-5-homeobox-atrial-septal-defects-ventricular-noncompaction-and-sudden-cardiac-death
#4
Julián Palomino Doza, Rafael Salguero-Bodes, María de la Parte, Fernando Arribas-Ynsaurriaga
No abstract text is available yet for this article.
March 16, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28290856/-chronic-heart-failure-with-preserved-systolic-function-and-reversible-dilatation-of-cardiac-chambers
#5
O V Blagova, S V Volkov, A V Nedostup, A O Korobkov, I V Mostovoy, N G Sergushina, N V Gagarina, E A Mershina
Diagnosis of dilation (D) cardiomyopathy (CMP) requires exclusion not only of inflammatory and genetically determined forms but also of some rare diseases. This 51 year old patient with history of moderate arterial hypertension approached a cardiologist because of new onset atrial fibrillation and dyspnea. Echocardiography detected dilation of all cardiac chambers with relatively preserved ejection fraction, causing suspicion of DCMP. Among conditions excluded were coronary atherosclerosis, congenital heart defect with left to right shunt, primary pulmonary hypertension, pulmonary embolism, hypertensive heart, tachycardia induced CMP, arrhythmogenic right ventricular dysplasia, noncompaction myocardium...
June 2016: Kardiologiia
https://www.readbyqxmd.com/read/28267213/echocardiography-fails-to-detect-left-ventricular-noncompaction-in-a-cohort-of-patients-with-noncompaction-on-cardiac-magnetic-resonance-imaging
#6
Sachin Diwadkar, Leelakrishna Nallamshetty, Carlos Rojas, Alexia Athienitis, Chris Declue, Chad Cox, Aarti Patel, Sanders H Chae
BACKGROUND: Left ventricular noncompaction (LVNC) is a rare disorder characterized by increased left ventricular trabeculation, deep intertrabecular recesses, and a thin compacted myocardial layer with associated clinical sequelae. Cardiac imaging with echocardiogram and cardiac magnetic resonance (CMRI) can detect variable myocardial morphology including excessive trabeculations. Multiple CMRI and echocardiographic criteria have been offered that attempt to identify LVNC morphology. The aim of this study was to assess the utility of echocardiogram in identifying LVNC in a cohort of patients with LVNC detected on CMRI...
March 7, 2017: Clinical Cardiology
https://www.readbyqxmd.com/read/28249770/long-qt-syndrome-and-left-ventricular-noncompaction-in-4-family-members-across-2-generations-with-kcnq1-mutation
#7
Mira Kharbanda, Amanda Hunter, Stephen Tennant, David Moore, Stephanie Curtis, Jules C Hancox, Victoria Murday
The association of long QT syndrome and left ventricular noncompaction is uncommon, with only a handful of previous reports, and only one reported case in association with a mutation in KCNQ1. Here we present genetic and phenotypic data for 4 family members across 2 generations who all have evidence of prolonged QT interval and left ventricular noncompaction in association with a pathogenic mutation in KCNQ1, and discuss the potential mechanisms of this association. In conclusion, we suggest that it may be helpful to consider looking for mutations in KCNQ1 in similar patients...
February 27, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28238123/challenging-mitral-valve-repair-for-double-orifice-mitral-valve-with-noncompaction-of-left-ventricular-myocardium
#8
Manabu Yamasaki, Hiroyasu Misumi, Kohei Abe, Kohei Kawazoe
Double-orifice mitral valve (DOMV) is a relatively rare cardiac anomaly. Although usually associated with various cardiac anomalies, co-presence of DOMV and noncompaction of left ventricular myocardium (NCLVM) is extremely rare. Here, we present a 24-year-old male who underwent mitral valve repair using artificial chordae and annuloplasty at the posterior commissure for severe mitral regurgitation (MR), resulting from flail anterior leaflet of the larger postero-medial orifice and dilatation of left ventricle with NCLVM...
February 25, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28183324/identification-of-taz-mutations-in-pediatric-patients-with-cardiomyopathy-by-targeted-next-generation-sequencing-in-a-chinese-cohort
#9
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
#10
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...
March 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
#11
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
#12
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
#13
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...
April 2017: Nature Reviews. Cardiology
https://www.readbyqxmd.com/read/28076895/implantation-of-left-ventricular-assist-device-in-a-patient-with-left-ventricular-non-compaction
#14
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/28067668/adaptor-proteins-numb-and-numbl-promote-cell-cycle-withdrawal-by-targeting-erbb2-for-degradation
#15
Maretoshi Hirai, Yoh Arita, C Jane McGlade, Kuo-Fen Lee, Ju Chen, Sylvia M Evans
Failure of trabecular myocytes to undergo appropriate cell cycle withdrawal leads to ventricular noncompaction and heart failure. Signaling of growth factor receptor ERBB2 is critical for myocyte proliferation and trabeculation. However, the mechanisms underlying appropriate downregulation of trabecular ERBB2 signaling are little understood. Here, we have found that the endocytic adaptor proteins NUMB and NUMBL were required for downregulation of ERBB2 signaling in maturing trabeculae. Loss of NUMB and NUMBL resulted in a partial block of late endosome formation, resulting in sustained ERBB2 signaling and STAT5 activation...
February 1, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27989498/recent-advancements-in-the-molecular-genetics-of-left-ventricular-noncompaction-cardiomyopathy
#16
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
#17
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
#18
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
#19
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
#20
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
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