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Noncompaction cardiomyopathy

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https://www.readbyqxmd.com/read/29661525/a-preliminary-study-of-left-ventricular-rotational-mechanics-in-children-with-noncompaction-cardiomyopathy-do-they-influence-ventricular-function
#1
Hythem M Nawaytou, Andrea E Montero, Putri Yubbu, Renzo J C Calderón-Anyosa, Tomoyuki Sato, Matthew J O'Connor, Kelley D Miller, Philip C Ursell, Julien I E Hoffman, Anirban Banerjee
BACKGROUND: Current diagnostic criteria for noncompaction cardiomyopathy (NCC) lack specificity, and the disease lacks prognostic indicators. Reverse apical rotation (RAR) with abnormal rotation of the cardiac apex in the same clockwise direction as the base has been described in adults with NCC. The aim of this study was to test the hypothesis that RAR might differentiate between symptomatic NCC and benign hypertrabeculations and might be associated with ventricular dysfunction. METHODS: Echocardiograms from 28 children with NCC without cardiac malformations were prospectively compared with those from 29 age-matched normal control subjects...
April 13, 2018: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29629254/multimodality-imaging-and-clinical-significance-of-congenital-ventricular-outpouchings-recesses-diverticula-aneurysms-clefts-and-crypts
#2
REVIEW
Alberto Cresti, Pierpaolo Cannarile, Elena Aldi, Marco Solari, Bruno Sposato, Luca Franci, Ugo Limbruno
The high spatial resolution of cardiac computed tomography (CT) and cardiac magnetic resonance (CMR) permit the diagnosis of congenital ventricular outpouchings (CVOs), including congenital ventricular diverticula (CVD), congenital ventricular aneurysms (CVA), clefts, and crypts. A unique classification has not been established, and these terms are used interchangeably with confounding terminology. Moreover, their significance is not univocal. A research was performed using PubMed on six subjects: (1) congenital left ventricular outpouchings; (2) congenital ventricular diverticulum; (3) congenital ventricular aneurysm; (4) ventricular clefts; (5) ventricular crypts; and (6) ventricular crevices...
January 2018: Journal of Cardiovascular Echography
https://www.readbyqxmd.com/read/29622970/sudden-cardiac-death-with-triple-pathologies-a-case-report
#3
R Razuin, F Nurquin, M N Shahidan, M N Julina
Sudden cardiac death in young adults may be associated with rare cardiomyopathies such as left ventricular noncompaction (LVNC) and arrhythmogenic right ventricular (ARVC) cardiomyopathies. LVNC is characterised by hypertrabeculations and deep recesses of the left ventricle. ARVC presents with thin myocardium as a result of extensive fibro-fatty infiltrations. In both conditions, death may be due to arrhythmia, thromboembolic events or heart failure. We report a case of a 21-year old athletic young man who collapsed at the futsal court right after the game...
June 2017: Egypt Heart J
https://www.readbyqxmd.com/read/29577407/echocardiographic-and-clinical-markers-of-left-ventricular-ejection-fraction-and-moderate-or-greater-systolic-dysfunction-in-left-ventricular-noncompaction-cardiomyopathy
#4
Ivan A Arenas, Christos G Mihos, Doreen DeFaria Yeh, Evin Yucel, Hany M Elmahdy, Orlando Santana
BACKGROUND: Left ventricular noncompaction (LVNC) is associated with progressive LV systolic dysfunction and dilated cardiomyopathy. We aimed to investigate the echocardiographic and clinical characteristics associated with LV ejection fraction (LVEF) and moderate or greater systolic dysfunction in patients with LVNC. METHODS: Our institutional echocardiography database was retrospectively reviewed between 2008 and 2014, and 62 patients with LVNC were identified...
March 25, 2018: Echocardiography
https://www.readbyqxmd.com/read/29568952/whole-exome-sequencing-identifies-novel-candidate-mutations-in-a-chinese-family-with-left-ventricular-noncompaction
#5
Ye Zhou, Zhiyong Qian, Jing Yang, Meng Zhu, Xiaofeng Hou, Yao Wang, Hongping Wu, Jiangang Zou
Left ventricular noncompaction (LVNC) is an inherited cardiomyopathy involving numerous genes. To identify novel candidate causal mutations, a whole exome sequencing study was performed on a Chinese LVNC family. Exons of the most prevalent pathogenic genes of LVNC (myosin heavy chain 7 and actin, α‑cardiac muscle 1) were sequenced, although no mutations were identified. Following this, Burrows‑Wheeler Aligner, PICARD and Genome Analysis Toolkit (v.2.8) were used to analyze the exome sequencing data. Non‑silent single nucleotide variants (SNVs) that were identified in patients with LVNC, although not in the healthy individual, were investigated further using SNV prioritization via the integration of genomic data (SPRING) based on P‑values...
March 19, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29563375/two-patients-with-coincident-noncompacted-myocardium-and-hypertrophic-cardiomyopathy
#6
Xuepei Tang, Sisi Yu, Liang Yin, Lianggeng Gong
To our knowledge, left ventricular noncompaction (LVNC) and hypertrophic cardiomyopathy (HCM) commonly occur as separate disorders in different patients; however, LVNC associated with HCM, which is called hypertrophic LVNC, is relatively rare.1) Here we report two sporadic cases of hypertrophic LVNC which were diagnosed by echocardiography and cardiac magnetic resonance (CMR).
March 20, 2018: International Heart Journal
https://www.readbyqxmd.com/read/29558024/predictors-of-poor-outcome-in-patients-with-left-ventricular-noncompaction-review-of-the-literature
#7
REVIEW
Marcin Kubik, Alicja Dąbrowska-Kugacka, Ewa Lewicka, Ludmiła Daniłowicz-Szymanowicz, Grzegorz Raczak
Left ventricular noncompaction (LVNC) is a unique inherited cardiomyopathy, characterized by an increased risk of adverse cardiovascular events such as heart failure, arrhythmia or sudden cardiac death. Although in comparison to dilated cardiomyopathy, the number of clinical studies concerning LVNC is still small, it is quickly increasing, which reflects a huge effort of the cardiovascular society to develop data to improve understanding of this cardiomyopathy. However, the predictors of adverse outcomes in LVNC are not well established...
March 16, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/29447732/left-ventricular-noncompaction-from-physiologic-remodeling-to-noncompaction-cardiomyopathy
#8
EDITORIAL
Erwin Oechslin, Rolf Jenni
No abstract text is available yet for this article.
February 20, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29447731/genetics-clinical-features-and-long-term-outcome-of-noncompaction-cardiomyopathy
#9
Jaap I van Waning, Kadir Caliskan, Yvonne M Hoedemaekers, Karin Y van Spaendonck-Zwarts, Annette F Baas, S Matthijs Boekholdt, Joost P van Melle, Arco J Teske, Folkert W Asselbergs, Ad P C M Backx, Gideon J du Marchie Sarvaas, Michiel Dalinghaus, Johannes M P J Breur, Marijke P M Linschoten, Laura A Verlooij, Isabella Kardys, Dennis Dooijes, Ronald H Lekanne Deprez, Arne S IJpma, Maarten P van den Berg, Robert M W Hofstra, Marjon A van Slegtenhorst, Jan D H Jongbloed, Danielle Majoor-Krakauer
BACKGROUND: The clinical outcomes of noncompaction cardiomyopathy (NCCM) range from asymptomatic to heart failure, arrhythmias, and sudden cardiac death. Genetics play an important role in NCCM. OBJECTIVES: This study investigated the correlations among genetics, clinical features, and outcomes in adults and children diagnosed with NCCM. METHODS: A retrospective multicenter study from 4 cardiogenetic centers in the Netherlands classified 327 unrelated NCCM patients into 3 categories: 1) genetic, with a mutation in 32% (81 adults; 23 children) of patients; 2) probably genetic, familial cardiomyopathy without a mutation in 16% (45 adults; 8 children) of patients; or 3) sporadic, no family history, without mutation in 52% (149 adults; 21 children) of patients...
February 20, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29445263/noncompaction-cardiomyopathy-and-heterotaxy-syndrome
#10
Hugo R Martinez, Stephanie M Ware, Marcus S Schamberger, John J Parent
Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by compact and trabecular layers of the left ventricular myocardium. This cardiomyopathy may occur with congenital heart disease (CHD). Single cases document co-occurrence of LVNC and heterotaxy, but no data exist regarding the prevalence of this association. This study sought to determine whether a non-random association of LVNC and heterotaxy exists by evaluating the prevalence of LVNC in patients with heterotaxy. In a retrospective review of the Indiana Network for Patient Care, we identified 172 patients with heterotaxy (69 male, 103 female)...
September 2017: Progress in Pediatric Cardiology
https://www.readbyqxmd.com/read/29440008/novel-%C3%AE-actin-gene-mutation-p-ala21val-causing-familial-hypertrophic-cardiomyopathy-myocardial-noncompaction-and-transmural-crypts-clinical-pathologic-correlation
#11
Andrea Frustaci, Alessandro De Luca, Valentina Guida, Tommaso Biagini, Tommaso Mazza, Carlo Gaudio, Claudio Letizia, Matteo Antonio Russo, Nicola Galea, Cristina Chimenti
BACKGROUND: Mutations of α-actin gene (ACTC1) have been phenotypically related to various cardiac anomalies, including hypertrophic cardiomyopathy and dilated cardiomyopathy and left ventricular (LV) myocardial noncompaction. A novel ACTC mutation is reported as cosegregating for familial hypertrophic cardiomyopathy and LV myocardial noncompaction with transmural crypts. METHODS AND RESULTS: In an Italian family of 7 subjects, 4 aged 10 (II-1), 14 (II-2), 43 (I-4) and 46 years (I-5), presenting abnormal ECG changes, dyspnea and palpitation (II-2, I-4, and I-5), and recurrent cerebral ischemic attack (I-5), underwent 2-dimensional echo, cardiac magnetic resonance, Holter monitoring, and next-generation sequencing gene analysis...
February 10, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29431384/cardiomyopathy-an-overview
#12
Jay Brieler, Matthew A Breeden, Jane Tucker
The definition and classification of cardiomyopathy have evolved considerably in recent years. Cardiomyopathy can be separated into primary (genetic, mixed, or acquired) and secondary categories, which result in varied phenotypes including dilated, hypertrophic, and restrictive patterns. Hypertrophic cardiomyopathy is the most common primary cardiomyopathy and can cause exertional dyspnea, presyncope, atypical chest pain, heart failure, and sudden cardiac death. Dilated cardiomyopathy can be genetic or acquired and typically presents with classic symptoms of heart failure with reduced ejection fraction...
November 15, 2017: American Family Physician
https://www.readbyqxmd.com/read/29289512/long-term-follow-up-of-symptomatic-adult-patients-with-noncompaction-cardiomyopathy
#13
Joel Salazar-Mendiguchía, José González-Costello, Teresa Oliveras, Francisco Gual, Josep Lupón, Nicolás Manito
No abstract text is available yet for this article.
December 27, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/29221698/undulating-clinical-course-of-noncompaction-cardiomyopathy
#14
Paola Dolader, Ferran Gran, Gemma Giralt, Queralt Ferrer, Ferran Rosés-Noguer, Dimpna C Albert
No abstract text is available yet for this article.
December 5, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/29212898/genetic-testing-in-pediatric-left-ventricular-noncompaction
#15
Erin M Miller, Robert B Hinton, Richard Czosek, Angela Lorts, Ashley Parrott, Amy R Shikany, Richard F Ittenbach, Stephanie M Ware
BACKGROUND: Left ventricular noncompaction (LVNC) can occur in isolation or can co-occur with a cardiomyopathy phenotype or cardiovascular malformation. The yield of cardiomyopathy gene panel testing in infants, children, and adolescents with a diagnosis of LVNC is unknown. By characterizing a pediatric population with LVNC, we sought to determine the yield of cardiomyopathy gene panel testing, distinguish the yield of testing for LVNC with or without co-occurring cardiac findings, and define additional factors influencing genetic testing yield...
December 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/29176328/cardiomyopathy-associated-gene-1-sensitive-pkc-dependent-connexin-43-expression-and-phosphorylation-in-left-ventricular-noncompaction-cardiomyopathy
#16
Yuanyuan Xie, Shenghua Liu, Shengshou Hu, Yingjie Wei
BACKGROUND/AIMS: Cardiomyopathy-associated gene 1 (CMYA1) plays an important role in embryonic cardiac development, postnatal cardiac remodeling and myocardial injury repair. Abnormal CMYA1 expression may be involved in cardiac dysplasia and primary cardiomyopathy. Our study aims to establish the relationship between CMYA1 and Left ventricular noncompaction cardiomyopathy (LVNC) pathogenesis. METHODS: We explored the effects of CMYA1 on connexins (Cx), which contribute to gap junction intercellular communication (GJIC), and the underlying signaling pathway in human normal tissues, LVNC myocardial tissues and HL1 cells by means of western blotting, RT-qPCR, immunohistochemistry, immunofluorescence, co-immunoprecipitation and scrape loading-dye transfer...
2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29118297/phenotype-and-functional-analyses-in-a-transgenic-mouse-model-of-left-ventricular-noncompaction-caused-by-a-dtna-mutation
#17
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/29035186/left-ventricular-noncompaction-cardiomyopathy-and-recurrent-polymorphic-ventricular-tachycardia-a-case-report-and-literature-review
#18
Oluwaseun A Akinseye, Uzoma N Ibebuogu, Sunil K Jha
INTRODUCTION: Noncompaction cardiomyopathy is a rare phenotype of cardiomyopathy associated with severe cardiac arrhythmia and thromboembolic complications. CASE PRESENTATION: A 55-year-old woman presented with frank pulmonary edema and received a diagnosis of noncompaction cardiomyopathy. DISCUSSION: Left ventricular noncompaction cardiomyopathy is increasingly being diagnosed because of advances in imaging modalities. It is important to differentiate this new phenotype of cardiomyopathy from others because its diagnosis, management, and prognosis differ...
2017: Permanente Journal
https://www.readbyqxmd.com/read/29032583/third-generation-ventricular-assist-device-mid-term-outcomes-of-the-heartware-hvad-in-pediatric-patients
#19
Mustafa Pac, Sinan Sabit Kocabeyoglu, Umit Kervan, Dogan Emre Sert, Serhat Koca, Ibrahim Ece, Feyza Aysenur Pac
The HeartWare HVAD is a small, third generation continuous flow pump that is intracorporeally placed for support of a failing ventricle in adult patients. This device is small in size when compared to other left ventricular assist devices and can therefore be used in smaller sized pediatric patients. We present our initial experience using the HVAD as a bridge to heart transplantation in the pediatric population. We performed a retrospective, single center, nonrandomized review of 17 pediatric patients who underwent HVAD implantation between June 2013 and March 2016...
February 2018: Artificial Organs
https://www.readbyqxmd.com/read/28980384/noncompaction-cardiomyopathy-in-an-infant-with-walker-warburg-syndrome
#20
Sarah Abdullah, Cynthia Hawkins, Gregory Wilson, Grace Yoon, Luc Mertens, Melissa T Carter, Andrea Guerin
Walker-Warburg syndrome (WWS) is a rare autosomal recessive, congenital muscular dystrophy that is associated with brain and eye anomalies. Several genes encoding proteins involved in α-dystroglycan glycosylation have been implicated in the aetiology of WWS. We describe a patient with nonclassical features of WWS presenting with heart failure related to noncompaction cardiomyopathy resulting in death at 4 months of age. Muscle biopsy revealed absent α-dystroglycan on immunostaining and genetic testing confirmed the diagnosis with two previously described POMT2 mutations...
October 5, 2017: American Journal of Medical Genetics. Part A
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