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management of atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy

Srijita Sen-Chowdhry, Daniel Jacoby, James C Moon, William J McKenna
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiovascular disorder, affecting 1 in 500 individuals worldwide. Existing epidemiological studies might have underestimated the prevalence of HCM, however, owing to limited inclusion of individuals with early, incomplete phenotypic expression. Clinical manifestations of HCM include diastolic dysfunction, left ventricular outflow tract obstruction, ischaemia, atrial fibrillation, abnormal vascular responses and, in 5% of patients, progression to a 'burnt-out' phase characterized by systolic impairment...
November 2016: Nature Reviews. Cardiology
Milind Y Desai, Nicholas G Smedira, Aditya Bhonsale, Maran Thamilarasan, Bruce W Lytle, Harry M Lever
OBJECTIVES: We sought to assess the long-term outcomes in patients with hypertrophic cardiomyopathy and severe left ventricular outflow tract obstruction, in whom the decision regarding surgery (vs conservative management) was based on assessment of symptoms or exercise capacity. METHODS: This was an observational study of 1530 patients with hypertrophic cardiomyopathy (aged 50 ± 13 years, 63% were men) with severe left ventricular outflow tract obstruction (excluding those aged <18 years, with left ventricular ejection fraction <50%, and with left ventricular outflow tract gradient <30 mm Hg)...
October 2015: Journal of Thoracic and Cardiovascular Surgery
Jodie Ingles, Renee Johnson, Tanya Sarina, Laura Yeates, Charlotte Burns, Belinda Gray, Kylie Ball, Christopher Semsarian
INTRODUCTION: Social determinants of health play an important role in explaining poor health outcomes across many chronic disease states. The impact of the social gradient in the setting of an inherited heart disease, hypertrophic cardiomyopathy (HCM), has not been investigated. This study sought to profile the socioeconomic status of patients attending a specialized multidisciplinary clinic and to determine the impact on clinical factors, psychosocial wellbeing and adherence to medical advice...
April 1, 2015: International Journal of Cardiology
Barry J Maron, Steve R Ommen, Christopher Semsarian, Paolo Spirito, Iacopo Olivotto, Martin S Maron
Hypertrophic cardiomyopathy (HCM) is a common inherited heart disease with diverse phenotypic and genetic expression, clinical presentation, and natural history. HCM has been recognized for 55 years, but recently substantial advances in diagnosis and treatment options have evolved, as well as increased recognition of the disease in clinical practice. Nevertheless, most genetically and clinically affected individuals probably remain undiagnosed, largely free from disease-related complications, although HCM may progress along 1 or more of its major disease pathways (i...
July 8, 2014: Journal of the American College of Cardiology
Alan D Enriquez, Martin E Goldman
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is clinically defined as unexplained myocardial hypertrophy, and it is an autosomal dominant disease of the cardiac sarcomere. It is present in 1 in 500 in the general adult population, making it the most common genetic cardiovascular disease. The pathophysiology of HCM is complex, leading to significant variability in clinical presentation. This, combined with the lack of randomized trials, makes the management of these patients difficult...
January 2014: Annals of Global Health
Ming-yao Luo, Shui-yun Wang, Hong-tao Sun, Zhao-hua Yin, Xin Sun, Yun-hu Song, Sheng-shou Hu
OBJECTIVE: To analyze the clinical features, precaution and management of complete heart block (CHB) after transaortic extended septal myectomy operation (extended Morrow procedure) in patients with hypertrophic obstructive cardiomyopathy (HOCM). METHODS: From October 1996 to December 2011, 10[6 men; mean age (45.4 ± 15.8) years, range 13-60 years] out of 160 consecutive HOCM patients underwent extended Morrow procedure developed CHB postoperatively. Their clinical data were retrospectively analyzed...
July 2013: Zhonghua Xin Xue Guan Bing za Zhi
Flávia B Nerbass, Rodrigo P Pedrosa, Naury J Danzi-Soares, Luciano F Drager, Edmundo Arteaga-Fernández, Geraldo Lorenzi-Filho
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiac disease and is characterized by large and asymmetric septal and left ventricle hypertrophy. HCM is a cause of disability, including heart failure, atrial fibrillation, and sudden death, with an annual mortality varying from 1% to 6%. Obstructive sleep apnea (OSA) is extremely common among patients with established cardiovascular disease, including hypertension and atrial fibrillation and when present may contribute to worse cardiovascular outcome...
June 2013: Sleep Medicine Reviews
Barry J Maron, Martin S Maron
Hypertrophic cardiomyopathy is a common inherited cardiovascular disease present in one in 500 of the general population. It is caused by more than 1400 mutations in 11 or more genes encoding proteins of the cardiac sarcomere. Although hypertrophic cardiomyopathy is the most frequent cause of sudden death in young people (including trained athletes), and can lead to functional disability from heart failure and stroke, the majority of affected individuals probably remain undiagnosed and many do not experience greatly reduced life expectancy or substantial symptoms...
January 19, 2013: Lancet
Roberto Spoladore, Martin S Maron, Rossella D'Amato, Paolo G Camici, Iacopo Olivotto
Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease, affecting over one million individuals in Europe. Hypertrophic cardiomyopathy patients often require pharmacological intervention for control of symptoms, dynamic left ventricular outflow obstruction, supraventricular and ventricular arrhythmias, and microvascular ischaemia. Current treatment strategies in HCM are predicated on the empirical use of long-standing drugs, such as beta-adrenergic and calcium blockers, although with little evidence supporting their clinical benefit in this disease...
July 2012: European Heart Journal
A Robertson
Hypertrophic cardiomyopathy (HCM) is a genetic disorder defined by the presence of a hypertrophied nondilated left ventricle in the absence of other known causes. Anatomic variants exist, and dynamic features of this disease process may include left ventricular outflow tract obstruction during systole, systolic anterior motion of the mitral valve, and mitral regurgitation. Patients with HCM are at higher risk for sudden cardiac death, stroke, atrial fibrillation, atrial reentrant tachycardia, syncope, and congestive heart failure (CHF)...
June 2010: Transplantation Proceedings
Paola Melacini, Cristina Basso, Annalisa Angelini, Chiara Calore, Fabiana Bobbo, Barbara Tokajuk, Nicoletta Bellini, Gessica Smaniotto, Mauro Zucchetto, Sabino Iliceto, Gaetano Thiene, Barry J Maron
AIMS: Hypertrophic cardiomyopathy (HCM) is an important cause of heart failure-related disability over a wide range of ages. Profiles of severe progressive heart failure symptoms and death, or heart transplantation deserve more complete definition within large patient cohorts. METHODS AND RESULTS: Clinical and morphological features of heart failure were assessed in 293 consecutive HCM patients over a median follow-up of 6 (inter-quartile range 2-11) years. Gross and histopathological features were analysed in 12 patients for whom the heart was available for inspection...
September 2010: European Heart Journal
Albert A Hagège, Michel Desnos
The management of patients with hypertrophic cardiomyopathy (HCM) has evolved markedly over the past 20 years, particularly with the rising number of indications for implantable cardiac defibrillators (ICDs) and alcohol septal ablation (ASA). However, medical therapies targeted to improve quality of life are underused; when resting and/or exercise obstruction is present, an incremental and additive approach should be used based on a high dosage of beta-blockers, verapamil and/or disopyramide. Radiofrequency catheter ablation of atrial fibrillation or A-V node has been proposed in some instances...
May 2009: Archives of Cardiovascular Diseases
Gerald Serwer
With increasing awareness of genetic abnormalities and the aging of patients with structural cardiac disease, particularly those who have had cardiac surgery, primary ventricular tachycardia is being recognized more frequently in children and adolescents and is becoming a larger management issue. The distinction of primary versus secondary is based on whether ventricular tachycardia is the inciting arrhythmia or the end result of a prolonged ischemic event, such as prolonged bradycardia with resultant ventricular tachycardia...
September 2008: Current Treatment Options in Cardiovascular Medicine
Horst Kuhn, Thorsten Lawrenz, Frank Lieder, Christian Leuner, Claudia Strunk-Mueller, Ludger Obergassel, Markus Bartelsmeier, Christoph Stellbrink
BACKGROUND: Catheter-based treatment of patients with hypertrophic obstructive cardiomyopathy (HOCM) by alcohol ablation (transcoronary ablation of septal hypertrophy, TASH) leads to symptomatic and haemodynamic improvement. However, little is known regarding the survival and its evolution since the introduction of the method in 1995. Theoretically, the method may be harmful, because widening of the obstructed left ventricular outflow tract is achieved by a septal infarction and subsequently by a potentially arrhythmogenic scar...
April 2008: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
Franco Cecchi, Iacopo Olivotto, Sandro Betocchi, Claudio Rapezzi, Maria Rosa Conte, Gianfranco Sinagra, Elisabetta Zachara, Antonello Gavazzi, Roberto Rordorf, Gianfranco Carnemolla, Maurizio Porcu, Stefano Nistri, Paolo Gruppillo, Simona Giampaoli
BACKGROUND: National registries are advocated as instrumental to the solution of rarity-related problems for patients with hypertrophic cardiomyopathy (HCM), including limited access to advanced treatment options. Thus, an Italian Registry for HCM was created to assess the clinical profile and the level of care nationwide of patients with HCM. METHODS: Cardiology centers over the national territory were recruited to provide clinical data of all patients with HCM ever seen at each institution...
November 2005: American Heart Journal
Georges Feghali, Jamshid Alaeddini, Stephen Ramee, Hector O Ventura
Hypertrophic cardiomyopathy is a complex genetic condition with a heterogeneous clinical course. Some patients remain asymptomatic throughout life while others develop one or more of the adverse clinical consequences including symptoms of congestive heart failure with exertional dyspnea and functional disability (usually with preserved left ventricular systolic function), atrial fibrillation, or sudden cardiac death. Because of this heterogenicity in the clinical presentations, management of patients with hypertrophic cardiomyopathy includes a wide range of pharmacologic therapies as well as invasive approaches...
November 2003: Congestive Heart Failure
Hubert Seggewiss, Angelos Rigopoulos
Hypertrophic cardiomyopathy (HCM) is an inherited cardiac disease characterized by unexplained left ventricular hypertrophy, typically involving the interventricular septum. Hypertrophy may be present in infants, but commonly develops during childhood and adolescence. Management of children with HCM aims to provide symptomatic relief and prevention of sudden death, which is the primary cause of death. Unfortunately, no randomized comparative trials to date have assessed different treatment options in HCM. Medical treatment with negative inotropic agents (beta-adrenoceptor antagonists [beta-blockers], verapamil) is the first therapeutic choice in all symptomatic patients...
2003: Paediatric Drugs
B J Maron, S A Casey, L C Poliac, T E Gohman, A K Almquist, D M Aeppli
CONTEXT: Hypertrophic cardiomyopathy (HCM) has been regarded as a disease that causes substantial disability, with annual mortality rates of up to 6%, based largely on reports from tertiary referral centers. OBJECTIVE: To assess the clinical course of HCM in a patient cohort more closely resembling the true disease state. DESIGN: Retrospective cohort study. SETTING: A regional cohort from Minnesota and adjoining regions, free of referral center bias, studied at Minneapolis Heart Institute...
February 17, 1999: JAMA: the Journal of the American Medical Association
J Cuquerella, L Ferrer, P Rivera, J A Tuset, E Medina, S Pamós, V Ariete, A Tomé, V García
A 53-year-old male suffered splenic infarction etiologically related to atrial fibrillation and non-obstructive hypertrophic cardiomyopathy. The main clinical manifestations were a one-month history of epigastric and left upper quadrant pain, with tenderness to palpation in the later zone. Laboratory tests revealed a slight leucocytosis (14.700) with left shift and a marked increase in LDH concentration (945 IU). Abdominal CAT and arteriography established the diagnosis, Echography proved normal. Patient evolution was satisfactory with conservative medical treatment...
June 1996: Revista Española de Enfermedades Digestivas
J L Cox, J P Boineau, R B Schuessler, K M Kater, D G Lappas
Between September 25, 1987, and December 31, 1992, 75 patients (53 men, 22 women; average age, 52 years) underwent the maze procedure for the treatment of atrial fibrillation. Six patients had undergone a previous cardiac operation and 28% underwent concomitant cardiac procedures in addition to the maze procedure. One patient (1.3%) died 10 days after undergoing a combined maze procedure and Morrow procedure for the management of chronic atrial fibrillation and hypertrophic obstructive cardiomyopathy. Postoperative atrial pacemakers were required in 40%: 26% for preoperative sick sinus syndrome and 6% for iatrogenic injury of the sinus node, and 8% had pacemakers in place preoperatively...
October 1993: Annals of Thoracic Surgery
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