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https://www.readbyqxmd.com/read/28420666/prevalence-and-clinical-implication-of-double-mutations-in-hypertrophic-cardiomyopathy-revisiting-the-gene-dose-effect
#1
Dana Fourey, Melanie Care, Katherine A Siminovitch, Adaya Weissler-Snir, Waseem Hindieh, Raymond H Chan, Michael H Gollob, Harry Rakowski, Arnon Adler
BACKGROUND: Available data suggests that double mutations in patients with hypertrophic cardiomyopathy are not rare and are associated with a more severe phenotype. Most of this data, however, is based on noncontemporary variant classification. METHODS AND RESULTS: Clinical data of all hypertrophic cardiomyopathy patients with 2 rare genetic variants were retrospectively reviewed and compared with a group of patients with a single disease-causing variant. Furthermore, a literature search was performed for all studies with information on prevalence and outcome of patients with double mutations...
April 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28391991/relation-of-qrs-duration-to-response-to-cardiac-resynchronization-therapy-in-patients-with-left-bundle-branch-block
#2
Biagio Sassone, Matteo Bertini, Matteo Beltrami, Michele Malagù, Giovanni Pasanisi, Helene Afi Kuwornu, Nicola Avigni, Giuseppe Fucà, Federico Pacchioni, Monica Minarelli, Maria Letizia Bacchi Reggiani, Luigi Padeletti
Left ventricular (LV) dyssynchrony (LVdys) is a necessary condition for successful cardiac resynchronization therapy (CRT). Despite left bundle branch block (LBBB) representing a reliable surrogate of LVdys, not all LBBB patients will respond to CRT. Our aim was to investigate the relation between QRS duration and LVdys in patients with LBBB who underwent CRT. We retrospectively studied 165 patients with LBBB who underwent CRT implantation according to the current guidelines. A 6-month reduction of LV end-systolic volume ≥15% identified responders to CRT...
March 16, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28370885/mortality-effect-of-icd-in-primary-prevention-of-nonischemic-cardiomyopathy-a-meta-analysis-of-randomized-controlled-trials
#3
Faraz Khan Luni, Hemindermeet Singh, Abdur R Khan, Sonia A Malik, Owais Khawaja, Haris Riaz, Wade Lee, Ameer Kabour, Mark Richards, Johan Aasbo
INTRODUCTION: Implantation of an implantable cardioverter defibrillator (ICD) for primary prevention of sudden cardiac death (SCD) is controversial in view of the recent DANISH trial which suggested no benefit with ICD for primary prevention in patients with non-ischemic cardiomyopathy (NICMP). METHODS: We conducted a meta-analysis of randomized control trials studying the role of ICD in primary prevention of SCD in patients with NICMP. Only six studies were identified after the application of inclusion/exclusion criteria...
March 30, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28369760/clinical-genetic-testing-in-pediatric-cardiomyopathy-is-bigger-better
#4
Allison Chang Ouellette, Jacob Mathew, Ashok Kumar Manickaraj, George Manase, Laura Zahavich, Judith Wilson, Kristen George, Lee Benson, Sarah Bowdin, Seema Mital
For clinical genetic testing of cardiomyopathy (CMP), current guidelines do not address which gene panels to use: targeted panels specific to a CMP phenotype or expanded (panCMP) panels that include genes associated with multiple phenotypic subtypes. Our objective was to assess the clinical utility of targeted versus panCMP panel testing in pediatric CMPs. 151 pediatric patients with primary hypertrophic (n = 66), dilated (n = 64), restrictive (n = 8), or left-ventricular non-compaction (n = 13) CMP who underwent clinical genetic panel testing at a single centre were included...
March 30, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28355432/primary-prevention-implantable-cardioverter-defibillators-in-patients-with-nonischemic-cardiomyopathy-a-meta-analysis
#5
Sana M Al-Khatib, Gregg C Fonarow, Jose A Joglar, Lurdes Y T Inoue, Daniel B Mark, Kerry L Lee, Alan Kadish, Gust Bardy, Gillian D Sanders
Importance: Conflicting data have emerged on the efficacy of implantable cardioverter defibrillators (ICDs) for primary prevention of sudden cardiac death (primary prevention ICDs) in patients with nonischemic cardiomyopathy. Objective: To investigate the association of primary prevention ICDs with all-cause mortality in patients with nonischemic cardiomyopathy. Data Sources: PubMed was searched from January 1, 2000, through October 31, 2016, for the terms implantable defibrillator OR implantable cardioverter defibrillator AND non-ischemic cardiomyopathy...
March 29, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28351902/risk-stratification-for-sudden-cardiac-death-too-late-to-establish-a-role-for-cardiac-mri
#6
Timothy M Markman, Saman Nazarian
Automatic detection and transvenous or subcutaneous defibrillation of malignant ventricular arrhythmias by implantable cardioverter defibrillators are vital for management of patients at high risk for sudden cardiac death. Guidelines and appropriate use criteria based on data from several clinical trials support prophylactic defibrillator implantation in patients with symptoms of heart failure and left ventricular ejection fraction ≤35%. Yet, existing guidelines direct defibrillator implantation to a small subset of the overall population at risk for sudden death, and with an average annual appropriate shock rate of 5...
March 28, 2017: Circulation
https://www.readbyqxmd.com/read/28351901/association-between-mid-wall-late-gadolinium-enhancement-and-sudden-cardiac-death-in-patients-with-dilated-cardiomyopathy-and-mild-and-moderate-left-ventricular-systolic-dysfunction
#7
Brian Halliday, Ankur Gulati, Aamir Ali, Kaushik Guha, Simon J Newsome, Monika Arzanauskaite, Vassilios S Vassiliou, Amrit S Lota, Cemil Izgi, Upasana Tayal, Zohya Khalique, Colin Stirrat, Dominique Auger, Nilesh Pareek, Tevfik F Ismail, Stuart D Rosen, Ali Vazir, Francisco Alpendurada, John Gregson, Michael P Frenneaux, Martin R Cowie, John G F Cleland, Stuart A Cook, Dudley J Pennell, Sanjay K Prasad
Background -Current guidelines only recommend the use of an implantable cardioverter defibrillator (ICD) in patients with dilated cardiomyopathy (DCM) for the primary prevention of sudden cardiac death (SCD) in those with a left ventricular ejection fraction (LVEF)<35%. However, registries of out-of-hospital cardiac arrests demonstrate that 70-80% of such patients have a LVEF>35%. Patients with a LVEF>35% also have low competing risks of death from non-sudden causes. Therefore, those at high-risk of SCD may gain longevity from successful ICD therapy...
March 28, 2017: Circulation
https://www.readbyqxmd.com/read/28341542/qt-spatial-dispersion-and-sudden-cardiac-death-in-hypertrophic-cardiomyopathy-time-for-reappraisal
#8
Damiano Magrì, Caterina Santolamazza, Luca Limite, Vittoria Mastromarino, Matteo Casenghi, Paola Orlando, Erika Pagannone, Maria Beatrice Musumeci, Antonello Maruotti, Agnese Ricotta, Giada Oliviero, Gianfranco Piccirillo, Massimo Volpe, Camillo Autore
BACKGROUND: The 12-lead surface electrocardiographic (ECG) analysis is able to provide independent predictors of prognosis in several cardiovascular settings, including hypertrophic cardiomyopathy (HCM). The present single-center study investigated the possible ability of several ECG-derived variables in stratifying sudden cardiac death (SCD) risk and, possibly, in improving the accuracy of the 2014 European Society of Cardiology guidelines. METHODS: A total of 221 consecutive HCM outpatients were recruited and prospectively followed...
March 21, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/28340197/the-evolution-and-benefit-of-device-therapy-in-patients-listed-for-heart-transplant
#9
Bert Vandenberk, Mark Hinderks, Gabor Voros, Christophe Garweg, Johan Vanhaecke, Rik Willems
Aims: The latest 2015 ESC Guidelines on the prevention of sudden cardiac death make a Class IIa recommendation for ICD implantation in patients listed for heart transplantation. This recommendation was based on expert consensus in view of the sparsity of data. Methods and results: All patients listed for heart transplantation at the University Hospitals of Leuven from 2002 until 2014 were studied retrospectively. Exclusion criteria were age <16 years, cardiac disease other than ischaemic or dilated cardiomyopathy and re-transplantation...
March 9, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28285949/considerations-in-cardio-oncology-multiple-mobile-left-sided-cardiac-thrombi-in-chemotherapy-induced-cardiomyopathy
#10
Toshinori Minamishima, Kenichi Matsushita, Hiromu Morikubo, Aoi Isaka, Noriko Matsushita, Hidehito Endo, Hiroshi Kubota, Konomi Sakata, Toru Satoh, Hideaki Yoshino
With advances in cancer chemotherapy, the importance of the new clinical discipline of cardio-oncology, which is concerned with the cardiac effects of chemotherapy, is increasing. Herein we describe the case of a 48-year-old woman with a history of breast cancer who presented with symptoms of heart failure due to chemotherapy-induced cardiomyopathy. Treatment for the patient's breast cancer had included surgery and chemotherapy with anthracyclines and trastuzumab. Echocardiography revealed multiple mobile thrombi in the left ventricle and atrium...
March 10, 2017: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
https://www.readbyqxmd.com/read/28267963/usefulness-of-cardiac-magnetic-resonance-imaging-to-measure-left-ventricular-wall-thickness-for-determining-risk-scores-for-sudden-cardiac-death-in-patients-with-hypertrophic%C3%A2-cardiomyopathy
#11
Jessica Webb, Adriana Villa, Imane Bekri, Joy Shome, Thomas Teall, Simon Claridge, Tom Jackson, Bradley Porter, Tevfik F Ismail, Gabriella Di Giovine, Christopher A Rinaldi, Gerald Carr-White, Khaled Al-Fakih, Reza Razavi, Amedeo Chiribiri
Echocardiography-derived measurements of maximum left ventricular (LV) wall thickness are important for both the diagnosis and risk stratification of hypertrophic cardiomyopathy (HC). Cardiac magnetic resonance (CMR) imaging is increasingly being used in the assessment of HC; however, little is known about the relation between wall thickness measurements made by the 2 modalities. We sought to compare measurements made with echocardiography and CMR and to assess the impact of any differences on risk stratification using the current European Society of Cardiology guidelines...
May 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28252256/arrhythmic-risk-stratification-in-heart-failure-time-for-the-next-step
#12
REVIEW
Konstantinos A Gatzoulis, Antonios Sideris, Emmanuel Kanoupakis, Skevos Sideris, Nikolaos Nikolaou, Christos-Konstantinos Antoniou, Theofilos M Kolettis
BACKGROUND: Primary prevention of sudden cardiac death by means of implantable cardioverter-defibrillators constitutes the holy grail of arrhythmology. However, current risk stratification algorithms lead to suboptimal outcomes, by both allocating ICDs to patients not deriving any meaningful survival benefit and withholding them from those erroneously considered as low-risk for arrhythmic mortality. METHODS: In the present review article we will attempt to present shortcomings of contemporary guidelines regarding sudden death prevention in ischemic and dilated cardiomyopathy patients and present available data suggesting encouraging results following implementation of multifactorial approaches, by using multiple modalities, both noninvasive and invasive...
March 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28243594/an-overview-and-evaluation-of-recent-machine-learning-imputation-methods-using-cardiac-imaging-data
#13
Yuzhe Liu, Vanathi Gopalakrishnan
Many clinical research datasets have a large percentage of missing values that directly impacts their usefulness in yielding high accuracy classifiers when used for training in supervised machine learning. While missing value imputation methods have been shown to work well with smaller percentages of missing values, their ability to impute sparse clinical research data can be problem specific. We previously attempted to learn quantitative guidelines for ordering cardiac magnetic resonance imaging during the evaluation for pediatric cardiomyopathy, but missing data significantly reduced our usable sample size...
March 2017: Data
https://www.readbyqxmd.com/read/28220574/selective-use-of-the-electrocardiogram-in-pediatric-preparticipation-athletic-examinations-among-pediatric-primary-care-providers
#14
Bradley C Clark, Joshua M Hayman, Charles I Berul, Kristin M Burns, Jonathan R Kaltman
OBJECTIVES: Recent literature examining insurance administrative data suggests that a selective approach, with concurrent history and physical exam (H&P), for obtaining an electrocardiogram (ECG) as a part of a preparticipation examination (PPE) for pediatric athletes is commonly used in the primary care setting demonstrating a high rate of disease detection. We sought to understand practice patterns of providers with regard to usage of ECG as a part of PPE. METHODS: Utilizing an online survey, we queried primary care providers regarding their practice patterns, rationale, and concerns regarding use of ECGs as a part of the PPE...
February 21, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28219595/comparison-of-post-liver-transplantation-outcomes-in-portopulmonary-hypertension-and-pulmonary-venous-hypertension-a-single-center-experience
#15
P Rajaram, A Parekh, M Fisher, J Kempker, R Subramanian
BACKGROUND: In potential liver transplant candidates, pulmonary vascular diseases, including portopulmonary hypertension (PoPH) and pulmonary venous hypertension (PVH), can be associated with high morbidity and mortality. Although there are clear guidelines regarding management and transplant listing criteria for patients with PoPH, the listing criteria for PVH are not well defined. OBJECTIVE: The aim of this study was to describe and compare the perioperative and postoperative morbidity and mortality associated with PoPH and PVH in patients undergoing liver transplantation...
March 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28217309/qrs-duration-versus-morphology-and-survival-after-cardiac-resynchronization-therapy
#16
Mand J H Khidir, Victoria Delgado, Nina Ajmone Marsan, Martin J Schalij, Jeroen J Bax
AIMS: The prognostic implications of QRS duration and morphology in heart failure patients treated with cardiac resynchronization therapy (CRT) remains debated. The present evaluation investigated the association between QRS duration (<150 vs. ≥150 ms) and QRS morphology (left bundle brand block [LBBB] vs. non-LBBB) and long-term prognosis of a large cohort of unselected heart failure patients treated with CRT according to contemporary guidelines. METHODS AND RESULTS: Of 973 heart failure patients treated with CRT (mean age 66...
February 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28214152/clinical-and-genetic-diagnosis-of-familial-hypertrophic-cardiomyopathy-results-in-pediatric-cardiology
#17
Bárbara Cardoso, Inês Gomes, Petra Loureiro, Conceição Trigo, Fátima Ferreira Pinto
INTRODUCTION: Hypertrophic cardiomyopathy (HCM) is most often of autosomal dominant inheritance with incomplete penetrance and variable expression. The main purpose of family screening is to identify relatives with unrecognized HCM and to monitor those at risk for disease, in order to minimize complications and to assess risk of sudden cardiac death. The ESC and ACCF/AHA guidelines on the diagnosis and management of HCM recommend the screening of child relatives from the age of 10-12 years...
March 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/28213711/implantable-cardioverter-defibrillators-for-primary-prevention-in-dilated-cardiomyopathy-post-danish-an-updated-meta-analysis-and-systematic-review-of-randomized-controlled-trials
#18
Georg Wolff, Yingfeng Lin, Athanasios Karathanos, Maximilian Brockmeyer, Susanne Wolters, Bernd Nowak, Alexander Fürnkranz, Hisaki Makimoto, Malte Kelm, Volker Schulze
BACKGROUND: Sudden cardiac death (SCD) is frequent in patients with heart failure due to dilated cardiomyopathy (DCM). Implantable cardioverter/defibrillator (ICD) device therapy is currently used for primary prevention. However, publication of the DANISH trial has recently given reason for doubt, showing no significant improvement in all-cause mortality in comparison to contemporary medical therapy. METHODS: We performed a meta-analysis of all randomized controlled trials comparing ICD therapy to medical therapy (MT) for primary prevention in DCM...
February 17, 2017: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
https://www.readbyqxmd.com/read/28188473/outpatient-treatment-with-the-wearable-cardioverter-defibrillator-clinical-experience-in-two-dutch-centres
#19
A F B E Quast, V F van Dijk, A A M Wilde, R E Knops, L V A Boersma
INTRODUCTION: The latest European Society of Cardiology Guidelines recommend consideration of a wearable cardioverter-defibrillator (WCD) for patients with a poor left ventricular ejection fraction (LVEF) who are at risk of sudden arrhythmic death but are not eligible for an implantable defibrillator. For these patients a WCD can be an alternative to long-term hospitalisation. PURPOSE: To evaluate the use of WCD therapy in these patient groups in two Dutch centres...
February 10, 2017: Netherlands Heart Journal
https://www.readbyqxmd.com/read/28151760/titin-truncating-mutations-in-dilated-cardiomyopathy-the-long-and-short-of-it
#20
Diane Fatkin, Inken G Huttner
PURPOSE OF REVIEW: Truncating variants in the TTN gene (TTNtv) are frequently identified in patients with dilated cardiomyopathy (DCM) but are also present in apparently healthy people in the general population. Consequently, there is considerable uncertainty about what it means for any single individual if a TTNtv is found. The aim of this review is to summarize current evidence implicating TTNtv in DCM pathogenesis and to provide some interpretative guidelines for clinical management...
February 1, 2017: Current Opinion in Cardiology
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