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https://www.readbyqxmd.com/read/29684120/atrial-fibrillation-in-patients-with-inherited-cardiomyopathies
#1
Cynthia Yeung, Andres Enriquez, Laiden Suarez-Fuster, Adrian Baranchuk
Atrial fibrillation (AF) often complicates the course of inherited cardiomyopathies and, in some cases, may be the presenting feature. Each inherited cardiomyopathy has its own peculiar pathogenetic characteristics that can contribute to the development and maintenance of AF. Atrial fibrillation may occur as a consequence of disease-specific defects, non-specific cardiac chamber changes secondary to the primary illness, or a combination thereof. The presence of AF can denote a turning point in the progression of the disease, promoting clinical deterioration and increasing morbidity and mortality...
April 19, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29682452/extending-the-phenotypic-spectrum-of-sengers-syndrome-congenital-lactic-acidosis-with-synthetic-liver-dysfunction
#2
David B Beck, Kristina Cusmano-Ozog, Nickie Andescavage, Eyby Leon
Sengers syndrome is a rare autosomal recessive mitochondrial disease characterized by lactic acidosis, hypertrophic cardiomyopathy and bilateral cataracts. We present here a case of neonatal demise, within the first day of life, who initially presented with severe lactic acidosis, with evidence of both chorioamnionitis and cardiogenic shock. Initial metabolic labs demonstrated a severe lactic acidosis prompting genetic testing which revealed a homozygous pathogenic variant for Sengers syndrome in AGK, c.979A >  T; p...
April 13, 2018: Translational Science of Rare Diseases
https://www.readbyqxmd.com/read/29681423/ultrasonic-cavitation-enabled-treatment-for-therapy-of-hypertrophic-cardiomyopathy-proof-of-principle
#3
Douglas L Miller, Xiaofang Lu, Chunyan Dou, Yiying I Zhu, Rachael Fuller, Kristina Fields, Mario L Fabiilli, Gabe E Owens, David Gordon, Oliver D Kripfgans
Ultrasound myocardial cavitation-enabled treatment was applied to the SS-16BN rat model of hypertrophic cardiomyopathy for proof of the principle underlying myocardial reduction therapy. A focused ultrasound transducer was targeted using 10-MHz imaging (10 S, GE Vivid 7) to the left ventricular wall of anesthetized rats in a warmed water bath. Pulse bursts of 4-MPa peak rarefactional pressure amplitude were intermittently triggered 1:8 heartbeats during a 10-min infusion of a microbubble suspension. Methylprednisolone was given to reduce initial inflammation, and Losartan was given to reduce fibrosis in the healing tissue...
April 19, 2018: Ultrasound in Medicine & Biology
https://www.readbyqxmd.com/read/29680808/large-q-and-s-waves-in-lead-iii-on-the-electrocardiogram-distinguish-patients-with-hypertrophic-cardiomyopathy-from-athletes
#4
Alvin S Chen, Rachel E Bent, Matthew Wheeler, Joshua W Knowles, Francois Haddad, Victor Froelicher, Euan Ashley, Marco V Perez
OBJECTIVE: To identify electrocardiographic findings, especially deep Q and S waves in lead III, that differentiate athletes from patients with hypertrophic cardiomyopathy (HCM). METHODS: Digital ECGs of athletes and patients with HCM followed at the Stanford Center for Inherited Cardiovascular Disease were studied retrospectively. All patients with HCM had an echocardiogram performed. A multivariable logistic regression model was used to calculate ORs for various demographic and ECG characteristics...
April 21, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29679534/determinants-of-reverse-remodeling-of-the-left-atrium-following-transaortic-myectomy
#5
Anita Nguyen, Hartzell V Schaff, Rick A Nishimura, Joseph A Dearani, Jeffrey B Geske, Brian D Lahr, Steve R Ommen
BACKGROUND: In patients with hypertrophic cardiomyopathy (HCM), enlargement of the left atrium (LA) is associated with increased morbidity and mortality due to risk of atrial fibrillation (AF), stroke, and heart failure. In this study, we investigated whether LA reverse remodeling occurs following septal myectomy. METHODS: Between March 2007 and July 2015, 656 patients underwent myectomy at our institution and had pre- and postoperative transthoracic echocardiographic (TTE) recording of LA volume index (LAVI)...
April 18, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29678624/autonomic-cardiovascular-control-and-cardiac-arrhythmia-in-two-pregnant-women-with-hypertrophic-cardiomyopathy-insights-from-icd-monitoring
#6
Pietro Francia, Carmen Adduci, Beatrice Musumeci, Lorenzo Semprini, Francesca Palano, Luigi Zezza, Massimo Volpe, Camillo Autore
In women with hypertrophic cardiomyopathy (HCM), pregnancy prompts major changes in hemodynamic and cardiac autonomic function that may precipitate heart failure (HF) or increase the risk of cardiac arrhythmia. We report the clinical follow-up of two patients with non-obstructive HCM implanted with a cardioverter defibrillator (ICD) allowing for continuous analysis of heart rate (HR), heart rate variability (HRV) and cardiac arrhythmia throughout the entire course of pregnancy. Both patients experienced increased HR and decreased HRV from the early stages of pregnancy, which persisted until delivery...
April 17, 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29678336/stress-myocardial-blood-flow-heterogeneity-is-a-positron-emission-tomography-biomarker-of-ventricular-arrhythmias-in-patients-with-hypertrophic-cardiomyopathy
#7
Dai-Yin Lu, Hulya Yalçin, Fatih Yalçin, Min Zhao, Sanjay Sivalokanathan, Ines Valenta, Abdel Tahari, Martin G Pomper, Theodore P Abraham, Thomas H Schindler, M Roselle Abraham
Patients with hypertrophic cardiomyopathy (HC) are at increased risk of sudden cardiac death. Abnormalities in myocardial blood flow (MBF) detected by positron emission tomography (PET) are common in HC, but a PET marker that identifies patients at risk of sudden cardiac death is lacking. We hypothesized that disparities in regional myocardial perfusion detected by PET would identify patients with HC at risk of ventricular arrhythmias. To test this hypothesis, we quantified global and regional MBFs by 13 NH3 -PET at rest and at stress, and developed a heterogeneity index to assess MBF heterogeneity in 133 symptomatic patients with HC...
February 6, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29676867/-image-of-the-month-left-ventricle-apical-aneurism-apical-outpouching-in-a-context-of-apical-hypertrophic-cardiomyopathy
#8
V Weerts, P J Bruyère, C Acasandrei, P Lancellotti, L Davin
No abstract text is available yet for this article.
April 2018: Revue Médicale de Liège
https://www.readbyqxmd.com/read/29671882/electrocardiography-scar-quantification-correlates-with-scar-size-of-hypertrophic-cardiomyopathy-seen-by-multidetector-computed-tomography
#9
Tiago Costa Bignoto, Dalmo Antônio Ribeiro Moreira, Ricardo Garbe Habib, Edileide de Barros Correia, Ricardo Carneiro Amarante, Tannas Jatene, Mario Barbosa Guedes Nunes, Tiago Senra, Luiz Eduardo Mastrocolla
BACKGROUND: Hypertrophic cardiomyopathy (HCM), a genetically transmitted disease, is the most common genetic cardiovascular disease. Current strategies to stratify risk are expensive and concentrated in wealthy centers. Twelve-lead electrocardiography (ECG) is inexpensive and universally available and can be readily used for Selvester QRS scoring, which estimates scar size. This study aimed to establish the relation between ECG scar quantification and myocardial fibrosis (extent of myocardial delayed enhancement) in multidetector computed tomography (MDCT)...
April 19, 2018: Clinical Cardiology
https://www.readbyqxmd.com/read/29669825/single-cardiac-ventricular-myosins-are-autonomous-motors
#10
Yihua Wang, Chen-Ching Yuan, Katarzyna Kazmierczak, Danuta Szczesna-Cordary, Thomas P Burghardt
Myosin transduces ATP free energy into mechanical work in muscle. Cardiac muscle has dynamically wide-ranging power demands on the motor as the muscle changes modes in a heartbeat from relaxation, via auxotonic shortening, to isometric contraction. The cardiac power output modulation mechanism is explored in vitro by assessing single cardiac myosin step-size selection versus load. Transgenic mice express human ventricular essential light chain (ELC) in wild- type (WT), or hypertrophic cardiomyopathy-linked mutant forms, A57G or E143K, in a background of mouse α-cardiac myosin heavy chain...
April 2018: Open Biology
https://www.readbyqxmd.com/read/29667903/posterior-mitral-leaflet-plication-for-hypertrophic-obstructive-cardiomyopathy
#11
Praveen Kerala Varma, Neethu Krishna, Hisham Ahamed, Sujatha Madassery
Anomalies of the mitral valve apparatus in hypertrophic cardiomyopathy are an important cause of systolic anterior motion. Patients with significant residual obstruction due to systolic anterior motion after myectomy and anterior mitral leaflet plication may end up having mitral valve replacement. We describe the case of a 52-year-old man who underwent posterior mitral leaflet plication to correct residual systolic anterior motion after anterior mitral leaflet plication.
January 1, 2018: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/29666183/dilated-cardiomyopathy-myosin-mutants-have-reduced-force-generating-capacity
#12
Zoltan Ujfalusi, Carlos D Vera, Srbolujub M Mijailovich, Marina Svicevic, Elizabeth Choe Yu, Masataka Kawana, Kathleen Ruppel, James A Spudich, Michael A Geeves, Leslie A Leinwand
Dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM) can cause arrhythmias, heart failure, and cardiac death. Here, we functionally characterized the motor domains of five DCM-causing mutations in human β-cardiac myosin. Kinetic analyses of the individual events in the ATPase cycle revealed that each mutation alters different steps in this cycle. For example, different mutations gave enhanced or reduced rate constants of ATP binding, ATP hydrolysis, or ADP release or exhibited altered ATP, ADP, or actin affinity...
April 17, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29664407/mechanical-circulatory-support-device-utilization-and-heart-transplant-waitlist-outcomes-in-patients-with-restrictive-and-hypertrophic-cardiomyopathy
#13
Lakshmi Sridharan, Brian Wayda, Lauren K Truby, Farhana Latif, Susan Restaino, Koji Takeda, Hiroo Takayama, Yoshifumi Naka, Paolo C Colombo, Mathew Maurer, Maryjane A Farr, Veli K Topkara
BACKGROUND: Patients with restrictive cardiomyopathy (RCM) and hypertrophic cardiomyopathy (HCM) generally are considered poor candidates for mechanical circulatory support devices (MCSDs) and often not able to be bridged mechanically to heart transplantation. This study characterized MCSD utilization and transplant waitlist outcomes in patients with RCM/HCM under the current allocation system and discusses changes in the era of the new donor allocation system. METHODS AND RESULTS: Patients waitlisted from 2006 to 2016 in the United Network for Organ Sharing registry were stratified by RCM/HCM versus other diagnoses...
March 2018: Circulation. Heart Failure
https://www.readbyqxmd.com/read/29664404/outcomes-in-patients-with-hypertrophic-cardiomyopathy-awaiting-heart-transplantation
#14
Julio Zuñiga Cisneros, Josef Stehlik, Craig H Selzman, Stavros G Drakos, Stephen H McKellar, Omar Wever-Pinzon
BACKGROUND: Current organ allocation policy and the rapid growth of mechanical support favor heart transplant (HT) candidates on left ventricular assist devices. HT candidates with hypertrophic cardiomyopathy (HCM) are usually not left ventricular assist device candidates and may have a disadvantage compared with dilated forms of cardiomyopathy. METHODS AND RESULTS: Adult HT candidates registered in the Scientific Registry of Transplant Recipients database between 1999 and 2016 were included...
March 2018: Circulation. Heart Failure
https://www.readbyqxmd.com/read/29663722/homozygous-missense-mybpc3-pro873his-mutation-associated-with-increased-risk-for-heart-failure-development-in-hypertrophic-cardiomyopathy
#15
Antheia Kissopoulou, Cecilia Trinks, Anna Green, Jan-Erik Karlsson, Jon Jonasson, Cecilia Gunnarsson
Hypertrophic cardiomyopathy (HCM) is a primary autosomal-dominant disorder of the myocardium with variable expressivity and penetrance. Occasionally, homozygous sarcomere genetic variants emerge while genotyping HCM patients. In these cases, a more severe HCM phenotype is generally seen. Here, we report a case of HCM that was diagnosed clinically at 39 years of age. Initial symptoms were shortness of breath during exertion. Successively, he developed a wide array of severe clinical manifestations, which progressed to an ominous end-stage heart failure that resulted in heart transplantation...
April 16, 2018: ESC Heart Failure
https://www.readbyqxmd.com/read/29663123/creation-of-a-restrictive-atrial-communication-in-heart-failure-with-preserved-and-mid-range-ejection-fraction-effective-palliation-of-left-atrial-hypertension-and-pulmonary-congestion
#16
Anna Bauer, Markus Khalil, Monika Lüdemann, Jürgen Bauer, Anoosh Esmaeili, Roberta De-Rosa, Norbert F Voelkel, Hakan Akintuerk, Dietmar Schranz
BACKGROUND: Left atrial decompression is considered in patients with symptomatic heart failure with preserved ejection fraction (HFpEF). We aimed to evaluate the feasibility and efficacy of transcatheter generation of a restrictive atrial septum communication to manage HFpEF from infancy to adulthood with cardiomyopathy and congenital heart defect. METHODS AND RESULTS: From June 2009 to December 2016, 24 patients (50% with an age less than 16 years) with HFpEF were palliated; NYHA-/Ross class IV (n = 10); median systemic ventricular ejection fraction 64 (range 35-78) %...
April 16, 2018: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
https://www.readbyqxmd.com/read/29661764/family-matters-outcomes-of-hypertrophic-cardiomyopathy-family-screening
#17
EDITORIAL
Jodie Ingles, Christopher Semsarian
No abstract text is available yet for this article.
April 2018: Circulation. Genomic and precision medicine
https://www.readbyqxmd.com/read/29661763/outcomes-of-contemporary-family-screening-in-hypertrophic-cardiomyopathy
#18
Hannah G van Velzen, Arend F L Schinkel, Sara J Baart, Rogier A Oldenburg, Ingrid M E Frohn-Mulder, Marjon A van Slegtenhorst, Michelle Michels
BACKGROUND: Contemporary hypertrophic cardiomyopathy (HCM) family screening includes clinical evaluation and genetic testing (GT). This screening strategy requires the identification of a pathogenic mutation in the proband. Our aim was to examine the results of this HCM screening strategy. METHODS: Between 1985 and 2016, 777 relatives of 209 probands were assessed in the context of HCM screening. Genotype-positive (G+) relatives and relatives without genetic testing (GT) underwent repeated clinical evaluations...
April 2018: Circulation. Genomic and precision medicine
https://www.readbyqxmd.com/read/29660848/international-collaborative-study-to-assess-cardiovascular-risk-and-evaluate-long-term-health-in-cats-with-preclinical-hypertrophic-cardiomyopathy-and-apparently-healthy-cats-the-reveal-study
#19
Philip R Fox, Bruce W Keene, Kenneth Lamb, Karsten A Schober, Valerie Chetboul, Virginia Luis Fuentes, Gerhard Wess, Jessie Rose Payne, Daniel F Hogan, Alison Motsinger-Reif, Jens Häggström, Emilie Trehiou-Sechi, Deborah M Fine-Ferreira, Reid K Nakamura, Pamela M Lee, Manreet K Singh, Wendy A Ware, Jonathan A Abbott, Geoffrey Culshaw, Sabine Riesen, Michele Borgarelli, Michael B Lesser, Nicole Van Israël, Etienne Côté, John E Rush, Barret Bulmer, Roberto A Santilli, Andrea C Vollmar, Maribeth J Bossbaly, Nadine Quick, Claudio Bussadori, Janice M Bright, Amara H Estrada, Dan G Ohad, Maria Josefa Fernández-Del Palacio, Jenifer Lunney Brayley, Denise S Schwartz, Christina M Bové, Sonya G Gordon, Seung Woo Jung, Paola Brambilla, N Sydney Moïse, Christopher D Stauthammer, Rebecca L Stepien, Cecilia Quintavalla, Christophe Amberger, Ferenc Manczur, Yong-Wei Hung, Remo Lobetti, Marie De Swarte, Alice Tamborini, Carmel T Mooney, Mark A Oyama, Andrey Komolov, Yoko Fujii, Romain Pariaut, Masami Uechi, Victoria Yukie Tachika Ohara
BACKGROUND: Hypertrophic cardiomyopathy is the most prevalent heart disorder in cats and principal cause of cardiovascular morbidity and mortality. Yet, the impact of preclinical disease is unresolved. HYPOTHESIS/OBJECTIVES: Observational study to characterize cardiovascular morbidity and survival in cats with preclinical nonobstructive (HCM) and obstructive (HOCM) hypertrophic cardiomyopathy and in apparently healthy cats (AH). ANIMALS: One thousand seven hundred and thirty client-owned cats (430 preclinical HCM; 578 preclinical HOCM; 722 AH)...
April 16, 2018: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/29660794/diagnostic-utility-of-cardiac-troponin-i-in-cats-with-hypertrophic-cardiomyopathy
#20
Yasutomo Hori, Masayuki Iguchi, Yasuhiro Heishima, Yohei Yamashita, Kensuke Nakamura, Atsushi Hirakawa, Akihito Kitade, Toshiki Ibaragi, Michio Katagi, Tamotsu Sawada, Masashi Yuki, Nobuyuki Kanno, Haruki Inaba, Noriko Isayama, Hideyuki Onodera, Naoki Iwasa, Mikio Kino, Mikihiro Narukawa, Syuhei Uchida
BACKGROUND: Cardiac troponin I (cTnI) is useful for assessing hypertrophic cardiomyopathy (HCM) in cats. OBJECTIVE: To measure plasma cTnI concentrations in healthy cats and evaluate the clinical utility of cTnI in determining the severity of HCM. ANIMALS: Clinically healthy cats (n = 88) and cats with HCM (n = 93). METHODS: Multicenter prospective study. Cats with HCM, including hypertrophic obstructive cardiomyopathy at various stages, were diagnosed using echocardiography...
April 16, 2018: Journal of Veterinary Internal Medicine
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