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Prevention of sudden cardiac death with implantable defibrillator in hypertrophic cardiomyopathy

Thomas Sené, Olivier Lidove, Joel Sebbah, Jean-Marc Darondel, Hervé Picard, Laurent Aaron, Olivier Fain, Thierry Zenone, Dominique Joly, Philippe Charron, Jean-Marc Ziza
The incidence and predictive factors of arrhythmias and/or conduction abnormalities (ACAs) requiring cardiac device (CD) implantation are poorly characterized in Fabry disease (FD). The aim of our retrospective study was to determine the prevalence, incidence, and factors associated with ACA requiring CD implantation in a monocentric cohort of patients with confirmed FD who were followed up in a department of internal medicine and reference center for FD.Forty-nine patients (20M, 29F) were included. Nine patients (4M, 5F; 18%) had at least one episode of ACA leading to device therapy...
October 2016: Medicine (Baltimore)
Raghav Murthy, Matthew R Williams, James C Perry, Suzanne Shepard, Daniel DiBardino
The primary and secondary prevention of sudden cardiac death resulting from malignant arrhythmia, channelopathy, and hypertrophic cardiomyopathy often requires the implantation of automatic internal cardiac defibrillators (AICDs) in the pediatric population. In young patients, the small size of the systemic veins, complex anatomy of congenital heart disease, and body habitus often preclude safe and durable transvenous placement of the AICD coil, requiring innovative methods to circumvent this problem. This report describes the technique used at Rady Children's Hospital San Diego/UCSD for the epicardial placement of an ICD system with a transvenous ICD coil placed between the aorta and pulmonary artery, thereby producing a stable location and excellent coil-to-can vector for successful defibrillation...
October 2016: Annals of Thoracic Surgery
Adaya Weissler-Snir, Arnon Adler, Lynne Williams, Christiane Gruner, Harry Rakowski
Sudden cardiac death (SCD) is the most devastating complication of hypertrophic cardiomyopathy (HCM). Although the annual rate of SCD in the general HCM population is <1% per year according to contemporary series, there is still a small subset of patients who are at increased risk of SCD. The greatest challenge in the management of HCM is identifying those at increased risk as an implantable cardioverter defibrillator is a potentially life-saving therapy. In this review, we sought to summarize the available data on SCD in HCM and provide a clinical perspective on the current differing and somewhat conflicting European and American recommendations on risk stratification, with balanced guidance with regards to rational clinical decision making...
July 1, 2016: European Heart Journal
Amalie C Thavikulwat, Todd T Tomson, Bradley P Knight, Robert O Bonow, Lubna Choudhury
INTRODUCTION: Implantable cardioverter-defibrillators (ICDs) are effective at terminating the ventricular arrhythmias that cause sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). However, identifying patients at risk for SCD remains an ongoing challenge. METHODS AND RESULTS: We retrospectively studied all adult patients with HCM treated with ICDs at our referral center from 2000 to 2013 to determine the risk factor profile, rates of appropriate ICD therapy, and complications associated with ICD implantation and discharge...
August 2016: Journal of Cardiovascular Electrophysiology
Brynn E Dechert, David J Bradley, Gerald A Serwer, Macdonald Dick Ii, Martin J Lapage
BACKGROUND: Implantable cardioverter defibrillators (ICDs) are intended to prevent sudden cardiac death yet also impose a risk of morbidity. This study describes the outcomes of ICDs in a pediatric and congenital heart disease (CHD) population from a single center. METHODS: Retrospective cohort study of all patients with an ICD followed at the University of Michigan Congenital Heart Center from 2005-2013. The primary outcome was ICD system revision for any reason excluding routine generator change for battery depletion...
July 2016: Pacing and Clinical Electrophysiology: PACE
Muhammad Umar Kamal, Irbaz Bin Riaz, Rajesh Janardhanan
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy with a prevalence of 1:500 (0.2%) in the general population. Sudden cardiac death (SCD) is the most feared presentation of HCM. Therefore, it is essential to identify individuals at high risk in order to prevent SCD. The absence of conventional risk factors does not nullify the risk of HCM related SCD. Although echocardiography is currently the most widely used imaging modality, cardiac magnetic resonance (CMR) allows detailed characterization of the HCM phenotype, which makes it possible to differentiate HCM from other causes of left ventricular hypertrophy...
2016: Cardiology Journal
Christopher C Cheung, Zachary W M Laksman, Gregory Mellor, Shubhayan Sanatani, Andrew D Krahn
Sudden cardiac death (SCD) in an apparently healthy individual is a tragedy that prompts a series of investigations to identify the cause of death and to prevent SCD in potentially at-risk family members. Several inherited channelopathies and cardiomyopathies, including long QT syndrome (LQTS), catecholaminergic polymorphic ventricular cardiomyopathy (CPVT), hypertrophic cardiomyopathy (HCM), and arrhythmogenic right ventricular cardiomyopathy (ARVC) are associated with exercise-related SCD. Exercise restriction has been a historical mainstay of therapy for these conditions...
April 2016: Canadian Journal of Cardiology
Matthew Hammond-Haley, Riyaz S Patel, Rui Providência, Pier D Lambiase
Inherited primary arrhythmia syndromes are a clinically heterogeneous group of relatively uncommon but important inherited cardiac conditions that are associated with an increased risk of sudden cardiac death (SCD) in the setting of a structurally normal heart. These include long-QT syndrome (LQTS), Short-QT syndrome (SQTS), Brugada syndrome (BrS) and Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT). The cardiomyopathies represent the other major group of inherited cardiac conditions associated with SCD, of which hypertrophic cardiomyopathy (HCM) is the most common...
April 15, 2016: International Journal of Cardiology
Dimitrios M Konstantinou, Georgios K Efthimiadis, Vassilios Vassilikos, Stylianos Paraskevaidis, Efstathios Pagourelias, Barry J Maron, Haralambos Karvounis
AIMS: Sudden cardiac death (SCD) may complicate hypertrophic cardiomyopathy (HCM) natural course. Patient selection for implantable cardioverter defibrillator (ICD) therapy in the primary prevention setting is still a challenge. METHODS: Thirty-seven HCM patients with a primary prevention ICD were included. All patients underwent preimplantation SCD risk assessment and semi-annual device interrogation during follow-up. Primary end point was the time to first appropriate ICD intervention including antitachycardia pacing or shock...
June 2016: Journal of Cardiovascular Medicine
Tomasz Pizoń, Marek Rajzer, Wiktoria Wojciechowska, Marek Jastrzebski, Agnieszka Olszanecka, Marta Rojek, Artur Jurczyszyn, Danuta Czarnecka
A 76-year old woman with a history of stage 3 arterial hypertension, paroxysmal atrial fibrillation, hypercholesterolemia and type 2 diabetes mellitus. Ventricular tachycardia was the first clinical manifestation of the disease. Echocardiography revealed hypertrophic cardiomyopathy with a high intraventricular gradient of 47 mmHg and midventricular obstruction at the level of the papillary muscles (the lumen of the left ventricle was 1-2 mm during systole). No ventricular aneurysm was found but the ventricle was elongated and dilated in the periapical part where systolic function was decreased but synchronized in time...
2015: Przegla̧d Lekarski
Barry J Maron, Martin S Maron
Hypertrophic cardiomyopathy (HCM) is regarded as the most common nontraumatic cause of sudden death (SD) in young people (including trained athletes). Introduction of implantable cardioverter-defibrillators (ICD) to HCM 15 years ago represented a new paradigm for clinical practice and probably the most significant advance in management of this disease. ICDs offer protection against SD by terminating potentially lethal ventricular tachyarrhythmias (11%/year secondary and 4%/year primary prevention), although implant decisions are weighed against the possibility of device-related complications (5%/year)...
May 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
Barry J Maron, Ethan J Rowin, Susan A Casey, Ross F Garberich, Martin S Maron
Hypertrophic cardiomyopathy (HC) has become a contemporary and treatable genetic heart disease, now with disease-related mortality reduced to as low as 0.5% per year, based largely on more effective risk stratification and the use of the implantable cardioverter-defibrillator for primary prevention of sudden death. This paradigm change in the natural history of HC has caused us to reconsider the overall mortality risk in this disease. We interrogated the databases of 2 HC referral centers, Minneapolis Heart Institute and Tufts Medical Center...
February 1, 2016: American Journal of Cardiology
Peter Magnusson, Fredrik Gadler, Per Liv, Stellan Mörner
BACKGROUND: Risk stratification of sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) is mainly based on evaluations from patients at highly specialized centers. AIM: To evaluate risk markers for appropriate implantable cardioverter defibrillator (ICD) therapy in an unselected, nationwide cohort of HCM. METHODS: Patients with an ICD due to HCM were identified from the Swedish ICD Registry since its start in 1995, merged with Patient Register data, and medical records were retrieved...
March 2016: Pacing and Clinical Electrophysiology: PACE
Vineet Goyal, Davinder S Jassal, Naranjan S Dhalla
Sudden cardiac death (SCD) is known to occur in individuals with diverse diseases. Each disease state has a specific etiology and pathophysiology, and is diagnosed and treated differently. Etiologies for SCD include cardiac arrhythmias, coronary artery disease, congenital coronary artery anomalies, hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia, dilated cardiomyopathy, and aortic valve stenosis. A potential unifying mechanism of SCD in these diseases involves a massive stimulation of the sympathetic nervous system's stress response and the subsequent elevation of circulating catecholamines...
March 2016: Canadian Journal of Physiology and Pharmacology
Barry J Maron, Ethan J Rowin, Susan A Casey, John R Lesser, Ross F Garberich, Deepa M McGriff, Martin S Maron
BACKGROUND: Youthful age has been considered the time of greatest risk for patients with hypertrophic cardiomyopathy (HCM), largely because of the possibility of sudden death. The last 2 decades have witnessed more reliable identification of at-risk patients and utilization of implantable cardioverter-defibrillators for prevention of sudden death, and other contemporary treatment options. Whether such management advances have significantly altered the considerable mortality rate for young HCM patients remains unresolved...
January 5, 2016: Circulation
Barry J Maron, Susan A Casey, Raymond H Chan, Ross F Garberich, Ethan J Rowin, Martin S Maron
Risk stratification for sudden death (SD) is an essential component of hypertrophic cardiomyopathy (HC) management, given the proven effectiveness of implantable cardioverter-defibrillators (ICD) for preventing SD. Although highly effective in identifying high-risk patients, current stratification algorithms remain incomplete and novel strategies are encouraged. In this regard, reliability of the statistical model to predict SD risk in HC, as recommended by the recent European Society of Cardiology (ESC) guidelines, was retrospectively tested in an independent cohort of 1,629 consecutive patients with HC aged ≥16 years...
September 1, 2015: American Journal of Cardiology
Peter Magnusson, Fredrik Gadler, Per Liv, Stellan Mörner
INTRODUCTION: The expanded use of implantable cardioverter-defibrillators (ICDs) to prevent sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) based on risk stratification in individuals without known previous ventricular arrhythmia is justified by an acceptable risk of device-related adverse events. Such complications, leading to surgical procedures or inappropriate electrical shocks, may impact mortality, morbidity, quality of life, and cost-effectiveness. METHODS AND RESULTS: From the Swedish ICD Registry, implants due to HCM since 1995 until November 2012 in patients aged ≥18 years were identified and medical records reviewed...
October 2015: Journal of Cardiovascular Electrophysiology
Barry J Maron
The implantable cardioverter-defibrillator (ICD) was not originally envisioned as a treatment to prevent sudden death (SD) in young people with genetic heart diseases. In the case of hypertrophic cardiomyopathy (HCM), initially it was not known whether the ICD would be effective in patients with a disease very different morphologically and functionally from coronary artery disease. Nevertheless, several observational clinical studies have shown that the ICD reliably terminates life-threatening ventricular tachyarrhythmias in HCM, and is largely responsible for reducing HCM mortality to 0...
June 2015: Cardiac Electrophysiology Clinics
Barry J Maron, Ethan J Rowin, Susan A Casey, Mark S Link, John R Lesser, Raymond H M Chan, Ross F Garberich, James E Udelson, Martin S Maron
BACKGROUND: Hypertrophic cardiomyopathy (HCM) has been prominently associated with adverse disease complications, including sudden death or heart failure death and a generally adverse prognosis, with annual mortality rates of up to 6%. OBJECTIVES: This study determined whether recent advances in management strategy, including implantable cardioverter-defibrillators (ICDs), heart transplantation, or other therapeutic measures have significantly improved survival and the clinical course of adult HCM patients...
May 12, 2015: Journal of the American College of Cardiology
Pieter A Vriesendorp, Arend F L Schinkel, Max Liebregts, Dominic A M J Theuns, Johan van Cleemput, Folkert J Ten Cate, Rik Willems, Michelle Michels
BACKGROUND: The recently released 2014 European Society of Cardiology guidelines of hypertrophic cardiomyopathy (HCM) use a new clinical risk prediction model for sudden cardiac death (SCD), based on the HCM Risk-SCD study. Our study is the first external and independent validation of this new risk prediction model. METHODS AND RESULTS: The study population consisted of a consecutive cohort of 706 patients with HCM without prior SCD event, from 2 tertiary referral centers...
August 2015: Circulation. Arrhythmia and Electrophysiology
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