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cardiomyopathy guidelines

Mark N Belkin, Nir Uriel
PURPOSE OF REVIEW: Advancements in highly active antiretroviral therapy (HAART) for human immunodeficiency virus (HIV) has led to increased survival, approaching that of the general population. These patients are at an increased risk for heart disease, specifically HIV-associated cardiomyopathy. RECENT FINDINGS: Initially, HIV-associated cardiomyopathy was predominantly noted as systolic dysfunction, but diastolic dysfunction has become more common with increased use of HAART...
March 14, 2018: Current Opinion in Cardiology
Ping Hu, Fengchang Qiao, Yan Wang, Lulu Meng, Xiuqing Ji, Chunyu Luo, Tianhui Xu, Ran Zhou, Jingjing Zhang, Bin Yu, Leilei Wang, Ting Wang, Qiong Pan, Dingyuan Ma, Dong Liang, Zhengfeng Xu
OBJECTIVES: This study aimed to determine the diagnostic yield of targeted next-generation sequencing (NGS) in prenatal diagnosis of congenital heart defects (CHDs) and for investigating the possible genetic etiology of prenatal CHD cases. METHODS: Forty-four fetuses with CHDs and normal molecular karyotypes underwent targeted NGS in this study. Fetal genomic DNA was directly extracted from amniotic fluid cells in each prenatal case. A customized targeted NGS panel containing 77 CHD-associated genes was designed to detect variants in the coding regions and the splicing sites of these genes...
March 13, 2018: Ultrasound in Obstetrics & Gynecology
Daniele Masarone, Juan Pablo Kaski, Giuseppe Pacileo, Perry M Elliott, Eduardo Bossone, Sharlene M Day, Giuseppe Limongelli
Cardiomyopathies (CMPs) are an increasingly recognized cause of heart failure and sudden death, particularly in young patients. Since their original description, major advances were achieved in the phenotype knowledge, natural history, and nosography of CMPs leading to different classification systems and therapies. However, a deeper knowledge of different causes, genotype-phenotype link, and natural history in different disease stages (preclinical, overt disease, and end-stage disease) according to a recognized standard of care (ie, international guidelines) is needed...
April 2018: Heart Failure Clinics
Julie T Bidwell, Karen S Lyons, James O Mudd, Kathleen L Grady, Jill M Gelow, Shirin O Hiatt, Christopher V Chien, Christopher S Lee
BACKGROUND: Although current guidelines emphasize the importance of social support to the success of left ventricular assist device (LVAD) therapy, few studies examine the influence of the caregiver on patient outcomes or quantify the impact of LVAD caregiving on caregiver outcomes. The purpose of this analysis was to identify patient and caregiver determinants of patient quality of life (QOL) and caregiver strain in response to LVAD therapy. METHODS AND RESULTS: Data on patients receiving LVAD therapy and their caregivers (n=50 dyads) were prospectively collected pre-implantation and 1, 3, and 6 months post-implantation...
March 7, 2018: Journal of the American Heart Association
Neal A Chatterjee, E Kevin Heist
Cardiac resynchronization therapy (CRT) is an important therapeutic tool in the management of patients with heart failure and electrical dyssynchrony. In appropriately selected patients, landmark randomized controlled trials have demonstrated morbidity and mortality benefit beyond standard goal-directed medical therapy. Current guidelines emphasize the greatest clinical efficacy of CRT in patients with symptomatic heart failure, left bundle branch block, and wide QRS duration (> 150 ms). Other relevant considerations include the presence of atrial fibrillation, the presence of AV block, the etiology of cardiomyopathy, the presence of masked left-sided conduction delay, and the impact of comorbidities that might predict poor clinical response...
March 6, 2018: Current Treatment Options in Cardiovascular Medicine
Peter Manu, Yevgeniya Lapitskaya, Atef Shaikh, Jimmi Nielsen
BACKGROUND: Clozapine is widely prescribed for treatment-refractory schizophrenia, but its use is limited by many potentially life-threatening adverse effects. The risk of rechallenge after these complications has never been comprehensively assessed in controlled studies. Thus, clinical guidelines must rely on the published case reports. The number of such reports is likely to increase over time, and updated analyses of larger samples are needed, as they may lead to changes in clinical guidelines...
March 2018: American Journal of Therapeutics
Lazaro E Hernandez
BACKGROUND: Microvascular dysfunction in hypertrophic cardiomyopathy has been associated with poor clinical outcome. Several studies have demonstrated a reduced perfusion reserve proportional to the magnitude of the hypertrophy. We investigated the utility of stress perfusion cardiac MRI to detect microvascular dysfunction in children with hypertrophic cardiomyopathy. METHODS: From January 2016 to January 2017, 13 patients, with a mean age of 15.3 years, with hypertrophic cardiomyopathy underwent regadenoson stress perfusion cardiac MRI (1...
February 22, 2018: Cardiology in the Young
JoEllyn C Moore, Lena Trager, Lucille E Anzia, Walid Saliba, Mohamed Bassiouny, Mandeep Bhargava, Mina Chung, Milind Desai, Ross Garberich, Harry Lever, Bruce D Lindsay, Jay Sengupta, Patrick Tchou, Oussama Wazni, Bruce L Wilkoff
BACKGROUND: Limited medical options are available for rhythm control in patients with atrial fibrillation (AF) and hypertrophic cardiomyopathy (HCM). There are no published reports of dofetilide use in this population. METHODS: A retrospective chart review was conducted on1,404 patients loaded on dofetilide for AF suppression at the Cleveland Clinic from 2008-2012, 25 of whom were found to have HCM. RESULTS: The HCM cohort was 32% female, 76% with persistent AF, mean age of 59 ± 10 years and mean ejection fraction (EF) of 54 ± 9 %...
February 16, 2018: Pacing and Clinical Electrophysiology: PACE
Jort Merken, Mark Hazebroek, Pieter Van Paassen, Job Verdonschot, Vanessa Van Empel, Christian Knackstedt, Myrurgia Abdul Hamid, Michael Seiler, Julian Kolb, Philipp Hoermann, Christian Ensinger, Hans-Peter Brunner-La Rocca, Gerhard Poelzl, Stephane Heymans
BACKGROUND: Inflammatory cardiomyopathy (infl-CMP) is characterized by increased cardiac inflammation in the absence of viruses, ischemia, valvular disease, or other apparent causes. Studies addressing the efficacy of immunosuppressive therapy in patients with infl-CMP are sparse. This study retrospectively investigates whether immunosuppressive agents on top of heart failure therapy according to current guidelines improves cardiac function and long-term outcome in patients with infl-CMP...
February 2018: Circulation. Heart Failure
P Z Szymanski, M Badri, B M Mayosi
BACKGROUND: There is limited information on acute heart failure (AHF) and its treatment in sub-Saharan Africa. OBJECTIVE: To describe the clinical characteristics and causes of heart failure (HF), adherence to HF treatment guidelines, and mortality of patients with AHF presenting to Groote Schuur Hospital (GSH), Cape Town, South Africa. METHODS: This sub-study of The Sub-Saharan Africa Survey of Heart Failure (THESUS-HF) was a prospective and observational survey that focused on the enrolment and follow-up of additional patients with AHF presenting to GSH and entered into the existing registry after publication of the primary THESUS-HF article in 2012...
February 1, 2018: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
A Zorzi, A Pelliccia, D Corrado
Competitive sports activity is associated with an increased risk of sudden cardiovascular death in adolescents and young adults with inherited cardiomyopathies. Many young subjects aspire to continue competitive sport after a diagnosis of cardiomyopathy and the clinician is frequently confronted with the problem of eligibility and the request of designing specific exercise programs. Since inherited cardiomyopathies are the leading cause of sudden cardiovascular death during sports performance, a conservative approach implying disqualification of affected athletes from most competitive athletic disciplines is recommended by all the available international guidelines...
February 8, 2018: Netherlands Heart Journal
Alexander Fürnkranz, Hisaki Makimoto
Idiopathic ventricular tachycardia: Frequent monomorphic premature ventricular contractions or non-sustained ventricular tachycardia without underlying structural heart disease is not a rare condition and may cause significant symptoms. A subgroup of patients develops IVT-associated cardiomyopathy. Current ESC guidelines recommend primary catheter ablation in symptomatic patients with right ventricular outflow tract IVT. Catheter ablation is also recommended in symptomatic patients with a left ventricular outflow tract (or other rare) origin, if antiarrhythmic drugs are ineffective or not desired...
February 2018: Deutsche Medizinische Wochenschrift
Konstantinos A Gatzoulis, Stavros Georgopoulos, Christos-Konstantinos Antoniou, Aris Anastasakis, Polychronis Dilaveris, Petros Arsenos, Skevos Sideris, Dimitris Tsiachris, Stefanos Archontakis, Elias Sotiropoulos, Artemisia Theopistou, Ioannis Skiadas, Ioannis Kallikazaros, Christodoulos Stefanadis, Dimitrios Tousoulis
BACKGROUND: Sudden cardiac death (SCD) risk stratification in hypertrophic cardiomyopathy (HCM) in the context of primary prevention remains suboptimal. The purpose of this study was to examine the additional contribution of programmed ventricular stimulation (PVS) on established risk assessment. METHODS: Two-hundred-and-three consecutive patients with diagnosed HCM and ≥1 noninvasive risk factors were prospectively enrolled over 19years. Patients were risk stratified, submitted to PVS and received an implantable cardioverter-defibrillator (ICD) according to then-current American Heart Association (AHA) guidelines and inducibility...
March 1, 2018: International Journal of Cardiology
Philip Brainin, Daria Frestad, Eva Prescott
AIMS: Coronary vascular dysfunction is linked with poor cardiovascular prognosis in patients without obstructive coronary artery disease (CAD) but a critical appraisal of the literature is lacking. METHODS AND RESULTS: We performed a systematic review and meta-analysis to quantify the cardiovascular risk associated with endothelial dependent and non-endothelial dependent coronary vascular dysfunction in patients with normal or non-obstructive CAD (epicardial stenosis <50%)...
March 1, 2018: International Journal of Cardiology
Antonio Berruezo, Markus Linhart, Angelo Auricchio, José Luis Zamorano, Pilar Santamaria, Roger Borràs, Felip Burgos, Josep Brugada
PURPOSE: Dual-chamber (DDD) pacing has been used for treatment of patients with hypertrophic obstructive cardiomyopathy (HOCM). Due to inconclusive results in prior trials, current guidelines assign DDD pacing a class IIb indication in selected patients. Prior observations indicate that lack of clinical improvement may result from suboptimal effect of DDD pacing with non-physiological AV intervals due to fusion of intrinsic and paced QRS complex. METHODS: The Triple Chamber Pacing in Hypertrophic Obstructive Cardiomyopathy Patients (TRICHAMPION) trial is a prospective, randomized, single-blinded, multicenter study to investigate the benefit of atrial synchronous biventricular pacing (CRT-P) in highly symptomatic HOCM patients with severe left ventricular outflow tract (LVOT) obstruction who are not candidates for ablative therapies...
February 3, 2018: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
Philippe Charron, Perry M Elliott, Juan R Gimeno, Alida L P Caforio, Juan Pablo Kaski, Luigi Tavazzi, Michal Tendera, Carole Maupain, Cécile Laroche, Pawel Rubis, Ruxandra Jurcut, Leonardo Calò, Tiina M Heliö, Gianfranco Sinagra, Marija Zdravkovic, Aušra Kavoliuniene, Stephan B Felix, Jacek Grzybowski, Maria-Angela Losi, Folkert W Asselbergs, José Manuel García-Pinilla, Joel Salazar-Mendiguchia, Katarzyna Mizia-Stec, Aldo P Maggioni
Aims: The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry. Methods and results: A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]...
January 24, 2018: European Heart Journal
Nicola Whiffin, Roddy Walsh, Risha Govind, Matthew Edwards, Mian Ahmad, Xiaolei Zhang, Upasana Tayal, Rachel Buchan, William Midwinter, Alicja E Wilk, Hanna Najgebauer, Catherine Francis, Sam Wilkinson, Thomas Monk, Laura Brett, Declan P O'Regan, Sanjay K Prasad, Deborah J Morris-Rosendahl, Paul J R Barton, Elizabeth Edwards, James S Ware, Stuart A Cook
PurposeInternationally adopted variant interpretation guidelines from the American College of Medical Genetics and Genomics (ACMG) are generic and require disease-specific refinement. Here we developed CardioClassifier (, a semiautomated decision-support tool for inherited cardiac conditions (ICCs).MethodsCardioClassifier integrates data retrieved from multiple sources with user-input case-specific information, through an interactive interface, to support variant interpretation...
January 25, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
Edward Sze, Zainab Samad, Allison Dunning, Kristen Bova Campbell, Zak Loring, Brett D Atwater, Karen Chiswell, Joseph A Kisslo, Eric J Velazquez, James P Daubert
BACKGROUND: Patients with left bundle branch block (LBBB) often respond to cardiac resynchronization therapy (CRT) with left ventricular ejection fraction (LVEF) improvement. Guideline-directed medical therapy (GDMT), not CRT, is first-line therapy for patients with reduced LVEF with LBBB. However, there are little data on how patients with reduced LVEF and LBBB respond to GDMT. OBJECTIVES: This study examined patients with cardiomyopathy and sought to assess rates of LVEF improvement for patients with LBBB compared to other QRS morphologies...
January 23, 2018: Journal of the American College of Cardiology
Ares Pasipoularides
Genomics designates the coordinated investigation of a large number of genes in the context of a biological process or disease. It may be long before we attain comprehensive understanding of the genomics of common complex cardiovascular diseases (CVDs) such as inherited cardiomyopathies, valvular diseases, primary arrhythmogenic conditions, congenital heart syndromes, hypercholesterolemia and atherosclerotic heart disease, hypertensive syndromes, and heart failure with preserved/reduced ejection fraction. Nonetheless, as genomics is evolving rapidly, it is constructive to survey now pertinent concepts and breakthroughs...
January 16, 2018: Journal of Molecular and Cellular Cardiology
Julien Higny, Antoine Guédès, Jacques Jamart, Claude Hanet, Laurence Gabriel, Vincent Dangoisse, Christophe de Meester de Ravenstein, Erwin Schroeder
BACKGROUND: Key predictors of survival after OHCA have been described in the literature. Current guidelines recommend emergency angiography in patients without an obvious extra-cardiac cause of arrest. However, the value of this strategy is debated. Moreover, diagnosis of acute coronary ischaemia after OHCA remains challenging, especially in patients without ST-segment elevation. OBJECTIVES: The primary objective was to identify qualitative variables associated with an increased chance of 30-d survival after OHCA...
January 16, 2018: Acta Cardiologica
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