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Hypertrophic cardiomyopathies

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https://www.readbyqxmd.com/read/29141175/sudden-cardiac-arrest-during-participation-in-competitive-sports
#1
Cameron H Landry, Katherine S Allan, Kim A Connelly, Kris Cunningham, Laurie J Morrison, Paul Dorian
BACKGROUND: The incidence of sudden cardiac arrest during participation in sports activities remains unknown. Preparticipation screening programs aimed at preventing sudden cardiac arrest during sports activities are thought to be able to identify at-risk athletes; however, the efficacy of these programs remains controversial. We sought to identify all sudden cardiac arrests that occurred during participation in sports activities within a specific region of Canada and to determine their causes...
November 16, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29141071/usefulness-of-total-12-lead-qrs-voltage-as-a-clue-to-diagnosis-of-patients-with-cardiac-sarcoidosis-severe-enough-to-warrant-orthotopic-heart-transplant
#2
William C Roberts, Tiffany M Becker, Shelley A Hall
Importance: Severe heart failure caused by cardiac sarcoidosis is difficult to diagnosis without biopsy. Objective: To assess whether total electrocardiographic 12-lead QRS voltage may be a clue to diagnosis. Design, Setting, Participants: Case-series study with cases collected at Baylor University Medical Center at Dallas, Dallas, Texas, from January 13, 2005, to January 24, 2017. The clinical records of 16 patients with severe heart failure caused by cardiac sarcoidosis were studied...
November 15, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/29136770/magnesium-valproate-ameliorates-type-1-diabetes-and-cardiomyopathy-in-diabetic-rats-through-estrogen-receptors
#3
Samir Rabadiya, Shradhha Bhadada, Ashvin Dudhrejiya, Devendra Vaishnav, Bhoomika Patel
Estrogen is known to exhibit cardioprotective and antihyperlipidemic action. Valproic acid has been shown to upregulate estrogen receptors (ERs) in breast and prostate cancer tissues. No pharmacological evaluations for magnesium valproate (MgV) so far have been done for diabetic cadio-lipidemic complications. Based on the above context, current study was undertaken to evaluate the therapeutic effectiveness of MgV in cardiac complications associated with type-1 diabetes mellitus in rats wherein diabetes was induced by single tail vein injection of streptozotocin (STZ, 45mg/kg, IV) in female Sprague Dawley rats and treatment of MgV (210mg/kg, PO) was given for eight weeks to diabetic animals, after which, various biochemical and cardiac biomarkers, hypertrophic, hemodynamic and histological parameters along with immunohistochemistry of ERs in the left ventricle (LV) were estimated...
November 7, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29133479/mother-with-diabetes-mellitus-and-infant-with-hypertrophic-obstructive-cardiomyopathy-milrinone-precluded-need-for-extracorporeal-membrane-oxygenation
#4
Soham Dasgupta, Amna Qasim, Ashraf M Aly, Sunil K Jain
No abstract text is available yet for this article.
November 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29133065/-functional-tricuspid-regurgitation-and-apical-hypertrophic-cardiomyopathy-diagnostic-and-therapeutic-strategy-regarding-a-clinical-case
#5
Jesús Piqueras-Flores, Alfonso Jurado-Román, Pedro Pérez-Díaz, Ramón Maseda-Uriza, Germán Hernández-Herra, M Ángeles Pérez-Martínez, María Thiscal López-Lluva, Juan Antonio Requena-Ibáñez, Raquel Frías-García, Ignacio Sánchez-Pérez, Fernando Lozano-Ruiz-Poveda
No abstract text is available yet for this article.
November 10, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/29131444/native-t1-reference-values-for-nonischemic-cardiomyopathies-and-populations-with-increased-cardiovascular-risk-a-systematic-review-and-meta-analysis
#6
REVIEW
Maaike van den Boomen, Riemer H J A Slart, Enzo V Hulleman, Rudi A J O Dierckx, Birgitta K Velthuis, Pim van der Harst, David E Sosnovik, Ronald J H Borra, Niek H J Prakken
BACKGROUND: Although cardiac MR and T1 mapping are increasingly used to diagnose diffuse fibrosis based cardiac diseases, studies reporting T1 values in healthy and diseased myocardium, particular in nonischemic cardiomyopathies (NICM) and populations with increased cardiovascular risk, seem contradictory. PURPOSE: To determine the range of native myocardial T1 value ranges in patients with NICM and populations with increased cardiovascular risk. STUDY TYPE: Systemic review and meta-analysis...
November 13, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29129561/alcohol-septal-ablation-for-hypertrophic-obstructive-cardiomyopathy-a-16-year-australian-single-centre-experience
#7
Jason Nogic, Youlin Koh, Marek Bak, Robert P Gooley, Ian T Meredith, Liam M McCormick
BACKGROUND: Alcohol septal ablation (ASA), is a well-established treatment for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). We report the acute, short and long-term clinical and echocardiographic outcomes of our experience in a single Australian centre over 16 years. METHODS: We retrospectively analysed consecutive patients presenting to our centre for ASA between March 2000 and July 2016. Local databases were interrogated along with direct patient or physician contact occurred where required...
October 13, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/29126582/effect-of-body-mass-index-on-exercise-capacity-in-patients-with-hypertrophic-cardiomyopathy
#8
Carolyn M Larsen, Caroline A Ball, Virginia B Hebl, Kevin C Ong, Konstantinos C Siontis, Thomas P Olson, Michael J Ackerman, Steve R Ommen, Thomas G Allison, Jeffrey B Geske
The objective of this study was to evaluate the relation between body mass index (BMI), exercise capacity, and symptoms in patients with hypertrophic cardiomyopathy (HC) and to utilize results of cardiopulmonary exercise tests (CPX) and transthoracic echocardiograms to understand the mechanism(s) of reduced exercise capacity across body mass index groups. Over a 6-year period, 510 consecutive patients with HC seen at a tertiary referral center underwent (CPX) and a transthoracic echocardiogram. Increasing BMI was associated with decreased exercise capacity as assessed by peak VO2 (ml/kg/min)...
October 12, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/29122139/role-of-exercise-testing-in-hypertrophic%C3%A2-cardiomyopathy
#9
REVIEW
Ethan J Rowin, Barry J Maron, Iacopo Olivotto, Martin S Maron
Over the last 25 years, patients with hypertrophic cardiomyopathy (HCM) have been studied with a variety of methods employing physiological exercise that have made major contributions to disease management and are performed without increased risk. Previously under-utilized in HCM, exercise (stress) echocardiography has become incorporated into the standard clinical assessment and diagnostic armamentarium of HCM using upright or supine symptom-limited treadmill or bicycle modalities. In patients without outflow gradients at rest, exercise echocardiography is the most appropriate method for provoking obstruction, with the capability of predicting future development of progressive heart failure symptoms, and differentiating patients with provocable obstruction from those without obstruction, with major implications for dictating treatment options, that is, surgical myectomy (alternatively, alcohol septal ablation) versus heart transplant...
November 2017: JACC. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29121754/the-role-of-feature-tracking-in-cardiovascular-magnetic-resonance-imaging-in-hypertrophic-cardiomyopathy
#10
EDITORIAL
Theodore Murphy, Deirdre Waterhouse
No abstract text is available yet for this article.
December 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29121751/prognostic-implications-of-global-myocardial-mechanics-in-hypertrophic-cardiomyopathy-by-cardiovascular-magnetic-resonance-feature-tracking-relations-to-left-ventricular-hypertrophy-and-fibrosis
#11
Rocio Hinojar, Covadonga Fernández-Golfín, Ariana González-Gómez, Luis Miguel Rincón, Maria Plaza-Martin, Eduardo Casas, Ana García-Martín, Mª Angeles Fernandez-Mendez, Amparo Esteban, Jose Julio Jimenez Nacher, Jose Luis Zamorano
BACKGROUND: Interstitial fibrosis, myocardial fiber disarray and non-uniform shortening are common histological features of hypertrophic cardiomyopathy (HCM). The degree of LV hypertrophy and fibrosis are postulated to contribute to the impairment of myocardial shortening. Cardiovascular magnetic resonance myocardial (CMR) feature tracking (CMR-FT) has emerged as a robust method that provides quantitative measurements of myocardial deformation. Our aim was first to evaluate LV strain parameters in HCM by CMR-FT and their dependence on both functional parameters and late gadolinium enhancement (LGE); and secondly we sought to determine their association with major cardiovascular outcomes...
December 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29121657/hypertrophic-cardiomyopathy-clinical-phenotype-is-independent-of-gene-mutation-and-mutation-dosage
#12
Shiv Kumar Viswanathan, Heather K Sanders, James W McNamara, Aravindakshan Jagadeesan, Arshad Jahangir, A Jamil Tajik, Sakthivel Sadayappan
Over 1,500 gene mutations are known to cause hypertrophic cardiomyopathy (HCM). Previous studies suggest that cardiac β-myosin heavy chain (MYH7) gene mutations are commonly associated with a more severe phenotype, compared to cardiac myosin binding protein-C (MYBPC3) gene mutations with milder phenotype, incomplete penetrance and later age of onset. Compound mutations can worsen the phenotype. This study aimed to validate these comparative differences in a large cohort of individuals and families with HCM...
2017: PloS One
https://www.readbyqxmd.com/read/29121221/short-atrioventricular-delay-pacing-therapy-in-young-and-old-patients-with-hypertrophic-obstructive-cardiomyopathy-good-long-term-results-and-a-low-need-for-reinterventions
#13
Davood Javidgonbadi, Nils-Johan Abdon, Bert Andersson, Maria Schaufelberger, Ingegerd Östman-Smith
Aims: Examination of long-term results following different treatments in hypertrophic obstructive cardiomyopathy (HOCM) in a complete geographical cohort. Methods and results: HOCM patients attending during 2002-13 in all 10 hospitals in the West Götaland Region, Sweden, were identified (n = 251), follow-up 14.4 (±8.9) years (mean ± SD), 121 managed medically, 42 treated with myectomy and 88 with short atrioventricular (AV) delay pacing as first interventional procedure...
November 7, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29120016/the-subcutaneous-implantable-cardioverter-defibrillator-in-daily-clinical-practice
#14
Tardu Özkartal, Alexander Breitenstein, Ardan M Saguner, Devdas T Inderbitzin, Markus Wilhelm, Stefano Benussi, Francesco Maisano, Thomas F Lüscher, Frank Ruschitzka, Jan Steffel
INTRODUCTION: In Switzerland, the first implantation of a subcutaneous implantable cardioverter-defibrillator (S-ICD) took place in November 2012. Up until the end of 2016, a total of 111 S-ICDs have been implanted. The aim of this study was to summarise the experience of a tertiary centre in Switzerland and to discuss the results in the context of international registries. METHODS: All patients in whom an S-ICD was implanted between November 2012 and the end of December 2016 at the University Heart Centre Zurich were included in this study...
November 9, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/29119456/heterogeneity-of-longitudinal-and-circumferential-contraction-in-relation-to-late-gadolinium-enhancement-in-hypertrophic-cardiomyopathy-patients-with-preserved-left-ventricular-ejection-fraction
#15
Keita Sakamoto, Noriko Oyama-Manabe, Osamu Manabe, Tadao Aikawa, Yasuka Kikuchi, Harue Sasai-Masuko, Masanao Naya, Kohsuke Kudo, Fumi Kato, Nagara Tamaki, Hiroki Shirato
PURPOSE: To evaluate heterogeneity of myocardial contraction in relation to extensive late gadolinium enhancement (LGE) in hypertrophic cardiomyopathy (HCM) patients with preserved left ventricular ejection fraction, using fast strain-encoded magnetic resonance imaging. MATERIALS AND METHODS: Twenty-two HCM patients and 24 age-matched control subjects were included in this retrospective study. The regional and global peak values of longitudinal and circumferential strain (LSregional, LSglobal, CSregional, CSglobal), and their regional heterogeneities were evaluated using coefficients of variation (LSCoV, CSCoV) in relation to LGE...
November 9, 2017: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/29118996/predictors-of-risk-for-sudden-death-in-childhood-hypertrophic-cardiomyopathy-the-importance-of-the-ecg-risk-score
#16
Ingegerd Östman-Smith, Gunnar Sjöberg, Annika Rydberg, Per Larsson, Eva Fernlund
Objective: To establish which risk factors are predictive for sudden death in hypertrophic cardiomyopathy (HCM) diagnosed in childhood. Methods: A Swedish national cohort of patients with HCM diagnosed <19 years of age was collected between 1972 and 2014, consisting of 155 patients with available ECGs, with average follow-up of 10.9±(SD 9.0) years, out of whom 32 had suffered sudden death or cardiac arrest (SD/CA group). Previously proposed risk factors and clinical features, ECG and ultrasound measures were compared between SD/CA group and patients surviving >2 years (n=100), and features significantly more common in SD/CA group were further analysed with univariate and multivariate Cox hazard regression in the total cohort...
2017: Open Heart
https://www.readbyqxmd.com/read/29118892/apical-hypertrophic-cardiomyopathy-a-case-report
#17
Ashraf Abugroun, Fatima Ahmed, Daniel Vilchez, Lalita Turaga
Apical hypertrophic cardiomyopathy (ApHCM) is a rare variant of hypertrophic cardiomyopathy, characterized by a spade-like left ventricular cavity. A 58-year-old African-American female with past medical history of hypertension presented for evaluation of recurrent exertional chest tightness, palpitations and headache. Prior workup including multiple stress tests and angiogram was non-conclusive. Electrocardiogram (EKG) showed characteristic marked T-waves inversions in inferior leads and left ventriculography revealed left ventricle apical hypertrophy with spade like left ventricular cavity that was typical of Yamaguchi syndrome...
October 2017: Cardiology Research
https://www.readbyqxmd.com/read/29118891/systolic-anterior-motion-of-mitral-valve-subchordal-apparatus-a-rare-echocardiographic-pattern-in-non-obstructive-hypertrophic-cardiomyopathy
#18
Jezreel L Taquiso, Stephanie Martha O Obillos, Joerelle V Mojica, Lauro L Abrahan, Elleen C Cunanan, Jaime Alfonso M Aherrera, Jose Donato A Magno
Systolic anterior motion (SAM) of the mitral valve or chordate is one characteristic seen in hypertrophic cardiomyopathy (HCM) either in obstructive or non-obstructive phenotypes. More often than not, the obstruction is caused by valvular rather than chordal SAM. We describe the role of echocardiography in identifying the actual anatomical location of the mitral valve apparatus involved in SAM and in assessing consequent left ventricular outflow tract (LVOT) obstruction in an otherwise asymptomatic patient...
October 2017: Cardiology Research
https://www.readbyqxmd.com/read/29116137/cardiovascular-consequences-of-myocardial-bridging-a-meta-analysis-and-meta-regression
#19
Sorin Hostiuc, Mugurel Constantin Rusu, Mihaela Hostiuc, Ruxandra Irina Negoi, Ionuț Negoi
Myocardial bridging, a congenital abnormality in which a coronary artery tunnels through the myocardial fibres was usually considered a benign condition. Many studies suggested a potential hemodynamic significance of myocardial bridging and some, usually case reports, implied a possible correlation between it and various cardiovascular pathologies like acute myocardial infarction, ventricular rupture, life-threatening arrhythmias, hypertrophic cardiomyopathy, apical ballooning syndrome or sudden death. The main objective of this article is to evaluate whether myocardial bridging may be associated with significant cardiac effects or if it is strictly a benign anatomical variation...
November 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29111210/interaction-of-adverse-disease-related-pathways-in-hypertrophic-cardiomyopathy
#20
Ethan J Rowin, Martin S Maron, Raymond H Chan, Anais Hausvater, Wendy Wang, Hassan Rastegar, Barry J Maron
Hypertrophic cardiomyopathy (HC) has been characterized as a generally progressive genetic heart disease, creating an ominous perspective for patients and managing cardiologists. We explored the HC disease burden and interaction of adverse clinical pathways to clarify patient expectations over long time periods in the contemporary therapeutic era. We studied 1,000 consecutive HC patients (52 ± 17 years) at Tufts Medical Center, followed 9.3 ± 8 years from diagnosis, employing a novel disease pathway model: 46% experienced a benign course free of adverse pathways, but 42% of patients progressed along 1 major pathway, most commonly refractory heart failure to New York Heart Association class III or IV requiring surgical myectomy (or alcohol ablation) or heart transplant; repetitive or permanent atrial fibrillation; and least commonly arrhythmic sudden death events...
September 20, 2017: American Journal of Cardiology
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