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Noncompaction cardiomyopathy

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https://www.readbyqxmd.com/read/28079110/left-ventricular-noncompaction-cardiomyopathy-cardiac-neuromuscular-and-genetic-factors
#1
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/28067668/adaptor-proteins-numb-and-numbl-promote-cell-cycle-withdrawal-by-targeting-erbb2-for-degradation
#2
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...
January 9, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28058741/noncompaction-cardiomyopathy-the-role-of-advanced-multimodality-imaging-techniques-in-diagnosis-and-assessment
#3
REVIEW
Lakshmi H Chebrolu, Anjlee M Mehta, Navin C Nanda
Noncompaction cardiomyopathy (NCCM) is a unique cardiomyopathy with a diverse array of genotypic and phenotypic manifestations. Its hallmark morphology consists of a bilayered myocardium with a compact epicardial layer and prominent trabeculations that comprise the noncompacted endocardial layer. The controversial diagnostic criteria for NCCM have been frequently discussed in the literature. This review touches on those diagnostic criteria, delves further into the evolving use of advanced imaging techniques within the major imaging modalities (echocardiography, computed tomography, and cardiac magnetic resonance imaging), and proposes an alternative algorithm incorporating these techniques for aiding with the diagnosis of NCCM...
January 6, 2017: Echocardiography
https://www.readbyqxmd.com/read/28039183/spongious-ischemic-myocardium-dealing-with-morphological-criteria-of-noncompaction-cardiomyopathy
#4
Gonzalo Navarrete, Eduardo Pozo, Pablo Díez-Villanueva, María José Olivera, Paloma Caballero, Luis Jesús Jiménez-Borreguero, Fernando Alfonso
No abstract text is available yet for this article.
January 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/27989498/recent-advancements-in-the-molecular-genetics-of-left-ventricular-noncompaction-cardiomyopathy
#5
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...
December 15, 2016: Clinica Chimica Acta; International Journal of Clinical Chemistry
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
#6
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
#7
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
#8
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
#9
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
#10
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/27801604/noncompaction-cardiomyopathy-and-stem-cell-therapy-from-pathogenesis-to-targeted-therapy
#11
Ozcan Ozeke, Dursun Aras, Omac Tufekcioglu, Sinan Aydogdu
No abstract text is available yet for this article.
January 2017: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/27773363/-heart-transplantation-for-the-treatment-of-isolated-left-ventricular-myocardial-noncompaction-first-case-in-mexico
#12
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
#13
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/27749293/left-ventricular-assist-device-insertion-in-a-patient-with-biventricular-noncompaction-cardiomyopathy-ebstein-anomaly-and-a-left-atrial-mass-a-case-report
#14
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/27688916/comment-on-a-rare-case-of-renal-infarct-due-to-noncompaction-cardiomyopathy-a-case-report-and-literature-review
#15
Josef Finsterer, Sinda Zarrouk-Mahjoub
No abstract text is available yet for this article.
2016: Case Reports in Cardiology
https://www.readbyqxmd.com/read/27688059/primary-cardiac-hydatid-cyst-presenting-as-noncompaction-cardiomyopathy
#16
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.
September 30, 2016: Echocardiography
https://www.readbyqxmd.com/read/27667386/isolated-left-ventricular-noncompaction-cardiomyopathy-a-transient-disease
#17
Juan M Vinardell, Maria D Avila, Orlando Santana
Isolated left ventricular noncompaction is either a distinct cardiomyopathy or a morphologic trait shared by several different types of cardiomyopathies. Although there is no current gold standard for its diagnosis, cardiac imaging is the most commonly accepted modality. Described is a case of left ventricular noncompaction that resolved 2 years after the initial diagnosis.
2016: Reviews in Cardiovascular Medicine
https://www.readbyqxmd.com/read/27609553/right-atrial-deformation-analysis-in-isolated-left-ventricular-noncompaction-insights-from-the-three-dimensional-speckle-tracking-echocardiographic-magyar-path-study
#18
Attila Nemes, Péter Domsik, Anita Kalapos, Henriette Gavallér, Mónika Oszlánczi, Tamás Forster
OBJECTIVE: Isolated left ventricular noncompaction (ILVNC) is a rare cardiomyopathy characterized by a prominent trabecular meshwork and deep intertrabecular recesses. The present study aimed to examine right atrial (RA) volumes, volume-based functional properties and strains by three-dimensional speckle-tracking echocardiography (3DSTE) in ILVNC patients. METHODS: The study group consisted of 13 patients with ILVNC (mean age: 54.6±14.1 years, six male) and 23 healthy age- and gender-matched volunteers (mean age: 50...
October 2016: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/27568851/mismatch-between-the-origin-of-premature-ventricular-complexes-and-the-noncompacted-myocardium-in-patients-with-noncompaction-cardiomyopathy-patients-involvement-of-the-conduction-system
#19
Sophie Van Malderen, Sip Wijchers, Ferdi Akca, Kadir Caliskan, Tamas Szili-Torok
BACKGROUND: Noncompaction cardiomyopathy (NCCM) is considered to be the result of an arrest in the normal myocardial embryogenesis. The histological, developmental, and electrophysiological explanation of ventricular arrhythmias in NCCM is still unknown. The aim of this study was to determine the origin of premature ventricular contractions (PVCs) in NCCM and to identify any predominant arrhythmic foci. METHODS: Retrospective data from our NCCM registry including 101 patients were analyzed...
August 29, 2016: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/27568117/lv-noncompaction-cardiomyopathy-or%C3%A2-just-a-lot-of-trabeculations
#20
Nay Aung, Filip Zemrak, Saidi A Mohiddin, Steffen E Petersen
No abstract text is available yet for this article.
August 20, 2016: JACC. Cardiovascular Imaging
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