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Noncompaction cardiomyopathy

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https://www.readbyqxmd.com/read/29881600/a-rare-cause-of-heart-failure
#1
Dominika Zoltowska, Jagadeesh Kumar Kalavakunta
Noncompaction cardiomyopathy (NCCM) is a rare but important cause of heart failure. The reported prevalence of left ventricle NCCM based on echocardiographic criteria varies between 0.014% and 1.3%, while the biventricular involvement is extremely rare with only few cases reported.
June 2018: Clinical Case Reports
https://www.readbyqxmd.com/read/29877973/efficacy-of-cardiac-resynchronization-therapy-in-patients-with-isolated-ventricular-noncompaction-with-dilated-cardiomyopathy-a-systematic-review-of-the-literature
#2
Matteo Bertini, Cristina Balla, Rita Pavasini, Giuseppe Boriani
: This is a systematic review of current evidence regarding the efficacy of cardiac resynchronization therapy (CRT) on patients with dilated cardiomyopathy and isolated left ventricular noncompaction (IVNC). This systematic review was performed following Preferred Reporting Items for Systematic Reviews and Meta-analysis guidelines. Records were searched in Pubmed, Cochrane Library, Google Scholar, Biomed Central. We included only studies focused on ventricular noncompaction patients treated with CRT. Of 46 records screened, we included 14 studies involving a total of 70 patients...
July 2018: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/29859913/improvement-of-the-diagnosis-of-left-ventricular-noncompaction-cardiomyopathy-after-analyzing-both-advantages-and-disadvantages-of-echocardiography-and-cmri
#3
EDITORIAL
Yifeng Xu, Xiaoxiao Liu, Hongli Li
No abstract text is available yet for this article.
May 31, 2018: Progress in Cardiovascular Diseases
https://www.readbyqxmd.com/read/29794382/cardiomyopathy-phenotypes-and-pregnancy-outcomes-with-left-ventricular-noncompaction-cardiomyopathy
#4
Yusuke Ueda, Chizuko A Kamiya, Atsushi Nakanishi, Chinami Horiuchi, Takekazu Miyoshi, Ryoichi Hazama, Mitsuhiro Tsuritani, Naoko Iwanaga, Reiko Neki, Jun Yoshimatsu
Little is known about pregnancies of left ventricular noncompaction cardiomyopathy (LVNC), much less cases in which LVNC was definitively diagnosed prepregnancy. We report the cases of three pregnant Japanese women definitively diagnosed with LVNC prepregnancy. Case 1 presented LVNC with restrictive phenotype. Her pregnancy was terminated due to exacerbated pulmonary hypertension and low output status at 30 weeks' gestation. Case 2 presented isolated LVNC with nonsustained ventricle tachycardia. A cesarean section was performed at 36 weeks' gestation because of placenta previa...
May 23, 2018: International Heart Journal
https://www.readbyqxmd.com/read/29770913/diagnostic-value-of-myocardial-deformation-pattern-in-children-with-noncompaction-cardiomyopathy
#5
Putri Yubbu, Hythem M Nawaytou, Renzo Calderon-Anyosa, Anirban Banerjee
The current echocardiographic diagnostic criteria for noncompaction cardiomyopathy (NCC) have variable sensitivity and low specificity. Moreover, there are limited data on the use of myocardial deformation imaging for early detection of myocardial dysfunction in children with NCC. We describe left ventricular (LV) deformation patterns in children with NCC, with the goal of identifying a potential diagnostic pattern. We prospectively enrolled 30 children with NCC (47% male; mean age 7.2 years) and 30 age- and gender-matched controls...
May 16, 2018: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/29766225/potential-common-pathogenic-pathways-for-the-left-ventricular-noncompaction-cardiomyopathy-lvnc
#6
Ying Liu, Hanying Chen, Weinian Shou
Ventricular trabeculation and compaction are two essential morphogenetic events for generating a functionally competent ventricular wall. A significant reduction in trabeculation is usually associated with hypoplastic wall and ventricular compact zone deficiencies, which commonly leads to embryonic heart failure and early embryonic lethality. In contrast, the arrest of ventricular wall compaction (noncompaction) is believed to be causative to the left ventricular noncompaction (LVNC), a genetically heterogeneous disorder and the third most common cardiomyopathy among pediatric patients...
May 15, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29706203/complications-of-radiofrequency-ablation-for-supraventricular-tachycardia-in-the-wolff-parkinson-white-syndrome-associated-with-noncompaction-cardiomyopathy
#7
William C Roberts, Paul A Grayburn, Shelley A Hall
Described herein is a 52-year-old man with Wolff-Parkinson-White syndrome and noncompaction cardiomyopathy who underwent 4 sternotomies to correct complications of 3 ablation procedures (2 open) for recurring supraventricular tachycardia, mitral valve repair for a damaged mitral valve during the third radiofrequency ablation procedure, and finally orthotopic heart transplantation.
March 23, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29661525/a-preliminary-study-of-left-ventricular-rotational-mechanics-in-children-with-noncompaction-cardiomyopathy-do-they-influence-ventricular-function
#8
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
#9
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
#10
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: The Egyptian heart journal: (EHJ): official bulletin of the Egyptian Society of Cardiology
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
#11
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
#12
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...
May 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29563375/two-patients-with-coincident-noncompacted-myocardium-and-hypertrophic-cardiomyopathy
#13
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 30, 2018: International Heart Journal
https://www.readbyqxmd.com/read/29558024/predictors-of-poor-outcome-in-patients-with-left-ventricular-noncompaction-review-of-the-literature
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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