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left ventricular noncompaction

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https://www.readbyqxmd.com/read/29770913/diagnostic-value-of-myocardial-deformation-pattern-in-children-with-noncompaction-cardiomyopathy
#1
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
#2
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/29755943/holt-oram-syndrome-in-two-families-diagnosed-with-left-ventricular-noncompaction-and-conduction-disease
#3
Samantha Barratt Ross, Richard D Bagnall, Laura Yeates, Raymond W Sy, Christopher Semsarian
No abstract text is available yet for this article.
April 2018: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/29686556/usefulness-of-a-left-ventricular-assist-device-in-patients-with-left-ventricular-noncompaction
#4
Helen Hashemi, Fayez S Raza, David M Harmon, Tony Alias, Joost Felius, Melody J Sherwood
Left ventricular noncompaction (LVNC) is a multifactorial structural abnormality of the myocardial wall characterized by prominent trabeculae and deep trabecular recesses. LVNC may present as a congenital or acquired defect characterized by 2 distinct tissue layers: a spongy, noncompacted inner myocardium and a thin, compacted outer myocardium. Patients with LVNC are prone to thromboembolic events, either due to deep trabeculations in the noncompacted myocardium or due to arrhythmias accompanying the defect...
January 2018: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/29661525/a-preliminary-study-of-left-ventricular-rotational-mechanics-in-children-with-noncompaction-cardiomyopathy-do-they-influence-ventricular-function
#5
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
#6
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/29629024/a-rare-case-of-left-ventricular-noncompaction-in-leopard-syndrome
#7
Kyehwan Kim, Min Gyu Kang, Hyun Woong Park, Jin-Sin Koh, Jeong Rang Park, Seok-Jae Hwang, Jin-Yong Hwang
No abstract text is available yet for this article.
March 2018: Journal of Cardiovascular Ultrasound
https://www.readbyqxmd.com/read/29626229/left-ventricular-hypertrabeculation-noncompaction-cardiac-phenotype-and-neuromuscular-disorders
#8
C Stöllberger, C Wegner, J Finsterer
BACKGROUND: The prognosis of patients with left ventricular hypertrabeculation/noncompaction (LVHT) and its association with neuromuscular disorders (NMDs) is a controversial topic. The aim of this study was to assess whether the prognosis of LVHT patients is dependent on cardiac phenotype and the presence of NMDs. METHODS: Consecutive patients who were diagnosed with LVHT between 1995 and 2016 were included in the study. Cardiac phenotype was classified according to the recommendations of the European Society of Cardiology as: "dilated" if the left ventricular end-diastolic diameter (LVEDD) was >57 mm and left ventricular fractional shortening (FS) was ≤25%; "hypertrophic" if LVEDD was ≤57 mm, FS > 25%, and left ventricular posterior wall (LVPWT) and interventricular septal thickness (IVST) were both >13 mm; "intermediate" if LVEDD was >57 mm and FS > 25% or if LVEDD was ≤57 mm and FS ≤ 25%; and "normal" if LVEDD was ≤57 mm, FS > 25%, and IVST and LVPWT ≤ 13 mm...
April 6, 2018: Herz
https://www.readbyqxmd.com/read/29622970/sudden-cardiac-death-with-triple-pathologies-a-case-report
#9
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
#10
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
#11
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
#12
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
#13
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/29468808/prevalence-of-left-ventricular-hypertrabeculation-noncompaction-among-children-with-sickle-cell-disease
#14
M Louise Morrison, Corrina McMahon, Riona Tully, Noelle Enright, Ricardo Pignatelli, Jeffrey A Towbin, Colin J McMahon
OBJECTIVES: Incidence of sickle cell disease (SCD) in Ireland has dramatically increased. Disease survival has also steadily improved however cardiovascular manifestations remain important causes of morbidity. These include reports of left ventricular hypertrabeculation (LVHT)/noncompaction. We sought to investigate the prevalence of LVHT among a large cohort of children with SCD. METHODS: We retrospectively reviewed the records of all patients with a diagnosis of SCD who had undergone surveillance echocardiography at Our Lady's Children's Hospital Crumlin (OLCHC) from 1998 to 2015...
February 21, 2018: Congenital Heart Disease
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/29409092/-gallop-in-the-routineergometrie-only-chore-or-diagnostic-challenge
#20
Stephan Knöchner, K Kronberg, U Schumann
ANAMNESIS:  A 47-year-old recreational sportsman showed in a routine ergometry polymorphic ventricular extrasystoles with good physical performance. INVESTIGATIONS:  In resting ECG impressed ventricular extrasystoles (VES) predominantly right-hand-block-like with superior axis, a long-term ECG yielded up to 100 VES per hour. Echocardiographically imposing 4 - 5 trabeculae, feathered, reticular structures apically in the left and lower in the right ventricle...
February 2018: Deutsche Medizinische Wochenschrift
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