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hypertrophic obstructive cardiomyopathy

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https://www.readbyqxmd.com/read/28819957/four-dimensional-flow-magnetic-resonance-imaging-in-hypertrophic-obstructive-cardiomyopathy
#1
Mateusz Śpiewak, Agata Kubik, Mariusz Kłopotowski, Konrad Werys, Magdalena Marczak
No abstract text is available yet for this article.
2017: Kardiologia Polska
https://www.readbyqxmd.com/read/28794111/clinical-characteristics-and-long-term-outcome-of-hypertrophic-cardiomyopathy-in-individuals-with-a-mybpc3-myosin-binding-protein-c-founder-mutation
#2
Hannah G van Velzen, Arend F L Schinkel, Rogier A Oldenburg, Marjon A van Slegtenhorst, Ingrid M E Frohn-Mulder, Jolanda van der Velden, Michelle Michels
BACKGROUND: MYBPC3 (Myosin-binding protein C) founder mutations account for 35% of hypertrophic cardiomyopathy (HCM) cases in the Netherlands. We compared clinical characteristics and outcome of MYBPC3 founder mutation (FG+) HCM with nonfounder genotype-positive (G+) and genotype-negative (G-) HCM. METHODS AND RESULTS: The study included 680 subjects: 271 FG+ carriers, 132 G+ probands with HCM, and 277 G- probands with HCM. FG+ carriers included 134 FG+ probands with HCM, 54 FG+ relatives diagnosed with HCM after family screening, 74 FG+/phenotype-negative relatives, and 9 with noncompaction or dilated cardiomyopathy...
August 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28793143/intraoperative-diagnosis-of-anderson-fabry-disease-in-patients-with-obstructive-hypertrophic-cardiomyopathy-undergoing-surgical-myectomy
#3
Franco Cecchi, Maria Iascone, Niccolò Maurizi, Laura Pezzoli, Irene Binaco, Elena Biagini, Maria Laura Fibbi, Iacopo Olivotto, Federico Pieruzzi, Ana Fruntelata, Lucian Dorobantu, Claudio Rapezzi, Paolo Ferrazzi
Importance: Diagnostic screening for Anderson-Fabry cardiomyopathy (AFC) is performed in the presence of specific clinical red flags in patients with hypertrophic cardiomyopathy (HCM) older than 25 years. However, left ventricular outflow tract obstruction (LVOTO) has been traditionally considered an exclusion criteria for AFC. Objective: To examine a series of patients diagnosed with HCM and severe basal LVOTO undergoing myectomy in whom the diagnosis of AFC was suspected by the cardiac surgeon intraoperatively and confirmed by histological and genetic examinations...
August 9, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28768581/cardiac-defects-morbidity-and-mortality-in-patients-affected-by-rasopathies-carnet-study-results
#4
Giulio Calcagni, Giuseppe Limongelli, Angelo D'Ambrosio, Francesco Gesualdo, M Cristina Digilio, Anwar Baban, Sonia B Albanese, Paolo Versacci, Enrica De Luca, Giovanni B Ferrero, Giuseppina Baldassarre, Gabriella Agnoletti, Elena Banaudi, Jan Marek, Juan P Kaski, Giulia Tuo, M Giovanna Russo, Giuseppe Pacileo, Ornella Milanesi, Daniela Messina, Maurizio Marasini, Francesca Cairello, Roberto Formigari, Maurizio Brighenti, Bruno Dallapiccola, Marco Tartaglia, Bruno Marino
BACKGROUND: RASopathies are developmental disease caused by mutations in genes encoding for signal transducers of the RAS-MAPK cascade. The aim of the present study was to provide a comprehensive description of morbidity and mortality in patients with molecularly confirmed RASopathy. METHODS: A multicentric, observational, retrospective study was conducted in seven European cardiac centres participating to the CArdiac Rasopathy NETwork (CARNET). Clinical records of 371 patients with confirmed molecular diagnosis of RASopathy were reviewed...
July 21, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28766836/doppler-echocardiography-underestimates-the-prevalence-and-magnitude-of-mid-cavity-obstruction-in-patients-with-symptomatic-hypertrophic-cardiomyopathy
#5
James W Malcolmson, Stephen M Hamshere, Abhishek Joshi, Constantinos O'Mahony, Mehul Dhinoja, Steffen E Petersen, Neha Sekhri, Saidi A Mohiddin
OBJECTIVES: To evaluate utility of Doppler echocardiography in the assessment of left ventricular (LV) mid-cavity obstructive (LVMCO) hypertrophic cardiomyopathy (HCM). BACKGROUND: LVMCO is a relatively under-diagnosed complication of HCM and may occur alone or in combination with LV outflow tract obstruction (LVOTO). Identifying and quantifying LVMCO and differentiating it from LVOTO has important implications for patient management. We aimed to assess diagnostic performance of Doppler echocardiography in the assessment of suspected LV obstruction...
August 2, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28762919/-effectiveness-of-extended-myectomy-in-patients-with-hypertrophic-cardiomyopathy-with-midventricular-obstruction
#6
A V Bogachev-Prokophiev, S I Zheleznev, M S Fomenko, R M Sharifulin, A V Afanasyev, O Yu Malakhova, A M Karaskov
PURPOSE: to assess effectiveness and safety of extended myectomy performed in patients with hypertrophic obstructive cardiomyopathy (HOCM) with midventricular obstruction. MATERIAL AND METHODS: Between 2010 and 2013 185 HOCM patients were operated for left ventricular outflow tract (LVOT) obstruction. Among these patients 32 had midventricular obstruction. Their age was 22-74 (mean 51.9+/-14.2) years. Mean peak gradient across LVOT was 89.1+/-20.4 mm Hg, thickness of interventricular septum was 26...
May 2017: Kardiologiia
https://www.readbyqxmd.com/read/28747036/long-term-outcome-of-nonobstructive-versus-obstructive-hypertrophic-cardiomyopathy-a-systematic-review-and-meta-analysis
#7
Francesco Pelliccia, Vincenzo Pasceri, Giuseppe Limongelli, Camillo Autore, Cristina Basso, Domenico Corrado, Massimo Imazio, Claudio Rapezzi, Gianfranco Sinagra, Giuseppe Mercuro
BACKGROUND: Prognosis of hypertrophic cardiomyopathy (HCM) is particularly heterogeneous. Patients with nonobstructive HCM (NOCM) are thought to be at relatively low-risk as compared with obstructive HCM (HOCM) with no need of major treatment options. However, available evidence of NOCM comes mainly from tertiary centers where a referral bias is likely to occur. Aim of this study was to perform a systematic review and meta-analysis of the published literature on hypertrophic cardiomyopathy (HCM) in order to outline differences in presenting features and long-term outcome between NOCM and HOCM...
September 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28747034/impact-of-peak-provoked-left-ventricular-outflow-tract-gradients-on-clinical-outcomes-in-hypertrophic-cardiomyopathy
#8
Dai-Yin Lu, Bereketeab Hailesealassie, Ioannis Ventoulis, Hongyun Liu, Hsin-Yueh Liang, Alexandra Nowbar, Iraklis Pozios, Marco Canepa, Kenneth Cresswell, Hong-Chang Luo, M Roselle Abraham, Theodore P Abraham
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is traditionally classified based on a left ventricular outflow tract (LVOT) pressure gradient of 30mmHg at rest or with provocation. There are no data on whether 30mmHg is the most informative cut-off value and whether provoked gradients offer any information regarding outcomes. METHODS: Resting and provoked peak LVOT pressure gradients were measured by Doppler echocardiography in patients fulfilling guidelines criteria for HCM...
September 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28734889/subaortic-membrane-and-hypertrophic%C3%A2-obstructive-cardiomyopathy-in-the-cardiac-catheterization-lab
#9
Musa A Sharkawi, Talhat Azemi, Brett Duncan, Immad Sadiq
No abstract text is available yet for this article.
July 19, 2017: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/28733069/pulmonary-hypertension-and-clinical-correlates-in-hypertrophic-cardiomyopathy
#10
Maria Beatrice Musumeci, Vittoria Mastromarino, Matteo Casenghi, Giacomo Tini, Pietro Francia, Antonello Maruotti, Antonella Romaniello, Damiano Magrì, Rosa Lillo, Carmen Adduci, Massimo Volpe, Camillo Autore
BACKGROUND: Pulmonary hypertension (PH) in patients with hypertrophic cardiomyopathy (HCM) has been investigated in a small number of studies. Purpose of this study was to assess the prevalence and its association with outcome in a population of consecutive HCM outpatients. METHODS: We retrospectively analyzed data of 361 consecutive HCM outpatients in whom echocardiographic measurements of pulmonary artery systolic pressure (PASP) were available at initial and most recent evaluation...
July 14, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28728695/alcohol-septal-ablation-for-obstructive-hypertrophic-cardiomyopathy-a-word-of-balance
#11
REVIEW
Paul Sorajja
The management of drug-refractory symptoms of obstructive hypertrophic cardiomyopathy has long been debated and is primarily centered on the choice between surgical myectomy and alcohol septal ablation. Decision making in these patients requires consideration of procedural risk, expertise and efficacy, and the long-term impact on patients' survival. Although there have been numerous reports on these procedures that may help guide decision making, these data continue to be self-reported and voluntary. Greater insight into the real-world experience for these therapies and how they should be applied in practice would be gained from mandatory reporting, akin to other multidisciplinary, transcatheter-based therapies...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28728694/alcohol-septal-ablation-for-obstructive-hypertrophic-cardiomyopathy-a-word-of%C3%A2-endorsement
#12
REVIEW
Max Liebregts, Pieter A Vriesendorp, Jurrien M Ten Berg
Twenty years after the introduction of alcohol septal ablation (ASA) for the treatment of obstructive hypertrophic cardiomyopathy, the arrhythmogenicity of the ablation scar appears to be overemphasized. When systematically reviewing all studies comparing ASA with myectomy with long-term follow-up, (aborted) sudden cardiac death and mortality rates were found to be similarly low. The focus should instead shift toward lowering the rate of reinterventions and pacemaker implantations following ASA because, in this area, ASA still seems inferior to myectomy...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28688952/left-ventricular-mechanics-in-children-with-hypertrophic-cardiomyopathy-cmr-study
#13
Łukasz Mazurkiewicz, Lidia Ziółkowska, Joanna Petryka, Mateusz Śpiewak, Łukasz Małek, Agata Kubik, Magdalena Marczak, Jolanta Misko, Grażyna Brzezińska-Rajszys
OBJECTIVES: To assess the magnitude of myocardial displacement abnormalities and their alterations with the fibrosis, left-ventricular (LV) outflow tract obstruction (LVOTO) and hypertrophy in juveniles with hypertrophic cardiomyopathy (HCM). STUDY DESIGN: Fifty-five children [age 12,5±4.6years, 38 (69,1%) males, 19 (34,5%) with LVOTO] with HCM and 20 controls underwent cardiovascular magnetic resonance. The LV feature tracking (FT) derived strain and strain rates were quantified...
July 5, 2017: Magnetic Resonance Imaging
https://www.readbyqxmd.com/read/28688858/a-comprehensive-review-of-stress-testing-in-hypertrophic-cardiomyopathy-assessment-of-functional-capacity-identification-of-prognostic-indicators-and-detection-of-coronary-artery-disease
#14
REVIEW
Albree Tower-Rader, Jorge Betancor, Harry M Lever, Milind Y Desai
Hypertrophic cardiomyopathy is a heterogeneous condition that may present with functional limitation due to dyspnea on exertion, angina, or symptoms of heart failure. Although angina is a common symptom, it is thought to be multifactorial, including abnormal microvasculature and epicardial coronary artery disease. The role of stress testing in the detection of coronary artery disease and its limitations are discussed in this review. Stress testing yields additional information beyond the detection of ischemia, which is prognostic independent of the presence of coronary artery disease and can be beneficial in defining the presence of provocable left ventricular outflow tract obstruction, symptoms, response of heart rate and blood pressure to exercise, and functional capacity...
July 6, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28681495/underappreciated-occurrence-of-discrete-subaortic-membranes-producing-left-ventricular-outflow-obstruction-in-hypertrophic-cardiomyopathy
#15
Muhil Kannappan, Barry J Maron, Hassan Rastegar, Natesa G Pandian, Martin S Maron, Ethan J Rowin
Subaortic obstruction due to systolic anterior motion (SAM) of the mitral valve with ventricular septal contact is a major cause of progressive heart failure symptoms in patients with hypertrophic cardiomyopathy (HCM). However, we have recently observed a unique, but not uncommon subgroup of HCM patients with outflow tract obstruction due only to discrete subaortic membrane or who have a membrane in addition to SAM-septal contact. HCM patients with subaortic membranes may be at increased risk for developing progressive heart failure symptoms...
July 6, 2017: Echocardiography
https://www.readbyqxmd.com/read/28674779/analysis-of-three-dimensional-endocardial-and-epicardial-strains-from-cardiac-magnetic-resonance-in-healthy-subjects-and-patients-with-hypertrophic-cardiomyopathy
#16
Xiaodan Zhao, Ru San Tan, Hak Chiaw Tang, Shuang Leng, Jun-Mei Zhang, Liang Zhong
Hypertrophic cardiomyopathy (HCM) is a genetic disease that leads to left ventricle (LV) hypertrophy with or without the presence of LV outflow tract obstruction. The aim of this study was to find an easy and useful indicator based on cardiac magnetic resonance (CMR) images for control subjects and patients with and without obstruction. CMR scans were performed for 19 control subjects and 19 HCM patients. Endocardial strain was defined as [Formula: see text], with [Formula: see text] being the length of endocardium at end-diastole (end-systole); similarly for epicardial strain ([Formula: see text])...
July 3, 2017: Medical & Biological Engineering & Computing
https://www.readbyqxmd.com/read/28670188/an-uncommon-variant-of-an-uncommon-disease-a-caucasian-adolescent-with-apical-hypertrophic-cardiomyopathy-diagnosed-with-myocardial-perfusion-imaging
#17
Rami Kassem Zein, Zaid Al-Faham, Jason A Mouabbi, Edouard R Daher
Apical hypertrophic cardiomyopathy (ApHCM) is a subtype of HCM. This variant is more common in the Asian population when compared to North American patients. Patients may present with arrhythmias, heart failure, myocardial infarction, chest discomfort, fatigue, and presyncope or syncope. Initial evaluation requires electrocardiogram and two-dimensional echocardiogram. T-wave inversion in the precordial leads as well as hypertrophy of the left ventricle is hallmarks of the disease. Cardiac magnetic resonance (CMR) imaging is the most specific and sensitive imaging modality...
July 2017: World Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/28669440/electrical-storms-in-patients-with-apical-aneurysms-and-hypertrophic-cardiomyopathy-with-midventricular-obstruction-a-case-series
#18
Anita Nguyen, Hartzell V Schaff
No abstract text is available yet for this article.
June 8, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28667811/premature-ventricular-contractions-producing-brockenbrough-braunwald-sign-in-obstructive-cardiomyopathy
#19
Gaurav K Sharma, Angie Tripathi, Paul A Jones
Increased dynamic flow in hypertrophic obstructive cardiomyopathy depicts a classic sign on invasive pressure tracings of the aorta and left ventricle, simultaneously known as the Brockenbrough-Braunwald sign, which is demonstrated in the presented case.
July 2017: Journal of Invasive Cardiology
https://www.readbyqxmd.com/read/28658286/mybpc3-mutations-are-associated-with-a-reduced-super-relaxed-state-in-patients-with-hypertrophic-cardiomyopathy
#20
James W McNamara, Amy Li, Sean Lal, J Martijn Bos, Samantha P Harris, Jolanda van der Velden, Michael J Ackerman, Roger Cooke, Cristobal G Dos Remedios
The "super-relaxed state" (SRX) of myosin represents a 'reserve' of motors in the heart. Myosin heads in the SRX are bound to the thick filament and have a very low ATPase rate. Changes in the SRX are likely to modulate cardiac contractility. We previously demonstrated that the SRX is significantly reduced in mouse cardiomyocytes lacking cardiac myosin binding protein-C (cMyBP-C). Here, we report the effect of mutations in the cMyBP-C gene (MYBPC3) using samples from human patients with hypertrophic cardiomyopathy (HCM)...
2017: PloS One
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