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https://www.readbyqxmd.com/read/28645472/bradycardia-syncope-and-left-ventricular-noncompaction-cardiomyopathy
#1
D Luke Glancy, Frederick R Helmcke, Allen P Hoang
A 55-year-old man with syncopal episodes was found to have sinus bradycardia at a rate of 37 beats/min, an episode of nonsustained ventricular tachycardia, and left ventricular noncompaction cardiomyopathy. After placement of a dual-chamber cardioverter defibrillator 4 years ago, he has had no further syncopal episodes, and there have been no defibrillator shocks.
May 30, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28643402/clinical-findings-in-right-ventricular-noncompaction-in-hypoplastic-left-heart-syndrome
#2
Monique M Gardner, Meryl S Cohen
BACKGROUND: Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) may occur when the left ventricle is affected but is rarely seen in isolation. RVNC may have clinical significance affecting surgical and long-term outcomes. We describe the diagnosis and clinical course in three patients at our institution. METHODS: We performed a retrospective review of patients diagnosed with RVNC over a 12-month period at our institution and reviewed their imaging and clinical course...
June 23, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28595227/-aortic-valve-replacement-and-mitral-valve-plasty-in-a-patient-with-left-ventricular-noncompaction-associated-with-aortic-regurgitation-and-mitral-regurgitation-report-of-a-case
#3
Bun Nakamura, Toshiya Tokui, Yasumi Maze, Koji Hirano, Taro Fujii, Masahiro Inagaki, Ryosai Inoue
Left ventricular noncompaction (LVNC) is believed to represent an arrest in the normal process of myocardial compaction, resulting in persistence of both hyper-trabeculation and intra-trabecular recess within the left ventricle. The clinical features of this disease are left ventricular dilatation and systolic dysfunction, which carry a high mortality. Most patients die in infancy, but some cases of LVNC in adults have been reported. To the best of our knowledge, only 4 cases of LVNC with valvular heart disease in adults have recently been operated...
June 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28544142/discordant-phenotypes-in-monozygotic-twins-with-16p11-2-microdeletions-including-the-sh2b1-gene
#4
Lin Li, Linhuan Huang, Shaobin Lin, Yanmin Luo, Qun Fang
A 200∼240 kb SH2B1-containing deletion region on 16p11.2 is associated with early-onset obesity and developmental delay. Here, we describe monozygotic twin brothers with discordant clinical presentations. Intrauterine fetal growth restriction was present in both twins. Additionally, twin A exhibited coarctation of aorta, left ventricular noncompaction, atrial septal defect, pericardial effusion, left hydronephrosis, and moderate developmental delay, whereas twin B exhibited single umbilical artery. Chromosome microarray analysis was performed on both twins and their parents...
May 24, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28522032/left-ventricular-noncompaction-associated-with-bicuspid-aortic-valve-and-aortic-coarctation
#5
Nestor A Parra-Ordoñez, Nydia Avila-Vanzzini, Nilda Espinola-Zavaleta
No abstract text is available yet for this article.
May 15, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/28521630/isolated-ventricular-noncompaction-cardiomyopathy-presenting-as-fetal-hydrops-at-24-weeks-gestation
#6
Jane E Armes, Lisa Squires, Rohan Lourie, Mark Williams, Renee Gallagher, Gareth Price, Andrew Stubbs, Sigrid Ma Swagemakers, Peter J van der Spek, James Harraway, Joseph Thomas, Deon J Venter
Ventricular noncompaction cardiomyopathy is a rare form of congenital cardiomyopathy with increasing evidence of genetic etiology, especially when presenting in childhood. Fetal presentation is rare. We describe a case of fetal hydrops, presenting at 24 weeks gestation and leading to intrapartum death at 26 weeks gestation. Autopsy examination revealed characteristic features of left ventricular noncompaction. A genetic analysis identified a constellation of variants of unknown significance in MYH6, TNNC1, and MYBPC3, genes known to be important in sarcomeric function...
June 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28520539/left-ventricular-noncompaction-with-muscular-ventricular-septal-defect-in-mother-and-son
#7
Adam W Powell, Michael D Taylor, John L Jefferies
No abstract text is available yet for this article.
May 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28488311/left-ventricular-noncompaction-cardiomyopathy-in-a-patient-with-trisomy-13-a-report-and-review-of-the-literature
#8
Anri Hayashi, Tomohiro Kumada, Fumihito Nozaki, Ikuko Hiejima, Minoru Shibata, Takashi Kusunoki, Tatsuya Fujii
Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by prominent trabecular meshwork, and it is thought to result from arrest of the normal compaction process during embryogenesis. Patients with LVNC may be asymptomatic or have symptoms ranging from heart failure to stroke, life-threatening arrhythmias, or sudden death. The frequency of LVNC in children has increased with longer clinical courses. About 80% of patients with trisomy 13 have a congenital cardiac abnormality, but a clinical description of LVNC with trisomy 13 is lacking because of its poor prognosis and lack of awareness about LVNC...
May 9, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28483032/advances-in-diagnosis-and-management-of-mitochondrial-cardiomyopathy
#9
Ruiqi Zhuge, Rong Zhou, Xinhai Ni
Mitochondrial cardiomyopathy (MCM) is a series of myocardial conditions characterized by abnormal heart-muscle structure,function,or both,secondary to genetic defects involving the mitochondrial respiratory chain,in the absence of concomitant coronary artery disease,hypertension,valvular disease,or congenital heart disease. MCM patients typically have hypertrophic or dilated cardiomyopathy. Arrhythmias and left ventricular myocardial noncompaction are less common,and heart failure may occur as the first symptom in some patients...
April 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28469993/left-ventricular-noncompaction-cardiomyopathy
#10
Amer Hawatmeh, Habib Habib, Fayez Shamoon
No abstract text is available yet for this article.
April 2017: Avicenna Journal of Medicine
https://www.readbyqxmd.com/read/28458700/echocardiographic-characteristics-of-isolated-left-ventricular-noncompaction
#11
Afsoon Fazlinezhad, Mohammad Vojdanparast, Shadi Sarafan, Pouya Nezafati
BACKGROUND: Although isolated left ventricular noncompaction (ILVNC) has been described almost two decades ago, our knowledge about its diagnosis, presentation, echocardiographic features and clinical outcome is sparse. We aimed to assess the echocardiographic and clinical characteristics of ILVNC in a group of patients referred to our center. METHODS: Patients who were referred to a tertiary referral center, affiliated with Mashhad University of Medical Sciences, with primary diagnosis of dilated cardiomyopathy underwent comprehensive echocardiographic evaluation...
September 2016: ARYA Atherosclerosis
https://www.readbyqxmd.com/read/28400861/revision-of-echocardiographic-indications-and-findings-in-neurologically-ill-patients
#12
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
#13
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
#14
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/28290856/-chronic-heart-failure-with-preserved-systolic-function-and-reversible-dilatation-of-cardiac-chambers
#15
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
#16
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
#17
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...
May 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
#18
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
#19
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
#20
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
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