Objectives The aim of this study was to determine the efficacy of implantable cardioverter-defibrillators (ICDs) in children and adolescents with hypertrophic cardiomyopathy (HCM). Background HCM is ...the most common cause of sudden death in the young. The availability of ICDs over the past decade for HCM has demonstrated the potential for sudden death prevention, predominantly in adult patients. Methods A multicenter international registry of ICDs implanted (1987 to 2011) in 224 unrelated children and adolescents with HCM judged at high risk for sudden death was assembled. Patients received ICDs for primary (n = 188) or secondary (n = 36) prevention after undergoing evaluation at 22 referral and nonreferral institutions in the United States, Canada, Europe, and Australia. Results Defibrillators were activated appropriately to terminate ventricular tachycardia or ventricular fibrillation in 43 of 224 patients (19%) over a mean of 4.3 ± 3.3 years. ICD intervention rates were 4.5% per year overall, 14.0% per year for secondary prevention after cardiac arrest, and 3.1% per year for primary prevention on the basis of risk factors (5-year cumulative probability 17%). The mean time from implantation to first appropriate discharge was 2.9 ± 2.7 years (range to 8.6 years). The primary prevention discharge rate terminating ventricular tachycardia or ventricular fibrillation was the same in patients who underwent implantation for 1, 2, or ≥3 risk factors (12 of 88 14%, 10 of 71 14%, and 4 of 29 14%, respectively, p = 1.00). Extreme left ventricular hypertrophy was the most common risk factor present (alone or in combination with other markers) in patients experiencing primary prevention interventions (17 of 26 65%). ICD-related complications, particularly inappropriate shocks and lead malfunction, occurred in 91 patients (41%) at 17 ± 5 years of age. Conclusions In a high-risk pediatric HCM cohort, ICD interventions terminating life-threatening ventricular tachyarrhythmias were frequent. Extreme left ventricular hypertrophy was most frequently associated with appropriate interventions. The rate of device complications adds a measure of complexity to ICD decisions in this age group.
Abstract 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. Methods We addressed long-term outcomes in 1,000 consecutive adult HCM patients presenting at 30 to 59 years of age (mean 45 ± 8 years) over 7.2 ± 5.2 years of follow-up. Results Of 1,000 patients, 918 (92%) survived to 53 ± 9.2 years of age (range 32 to 80 years) with 91% experiencing no or only mild symptoms at last evaluation. HCM-related death occurred in 40 patients (4% 0.53%/year) at 50 ± 10 years from the following events: progressive heart failure (n = 17); arrhythmic sudden death (SD) (n = 17); and embolic stroke (n = 2). In contrast, 56 other high-risk patients (5.6%) survived life-threatening events, most commonly with ICD interventions for ventricular tachyarrhythmias (n = 33) or heart transplantation for advanced heart failure (n = 18 0.79%/year). SD occurred in patients who declined ICD recommendations, had evaluations before application of prophylactic ICDs to HCM, or were without conventional risk factors. The 5- and 10-year survival rates (confined to HCM deaths) were 98% and 94%, respectively, not different from the expected all-cause mortality in the general U.S. population (p = 0.25). Multivariate independent predictors of adverse outcome were younger age at diagnosis, female sex, and increased left atrial dimension. Conclusions In a large longitudinally assessed adult HCM cohort, we have demonstrated that contemporary management strategies and treatment interventions, including ICDs for SD prevention, have significantly altered the clinical course, now resulting in a low disease-related mortality rate of 0.5%/year and an opportunity for extended longevity.
Abstract Background Left ventricular outflow tract gradients are absent in an important proportion of patients with hypertrophic cardiomyopathy (HCM). However, the natural course of this important ...patient subgroup remains largely unresolved. Objectives The authors systematically employed exercise (stress) echocardiography to define those patients without obstruction to left ventricular outflow at rest and/or under physiological exercise and to examine their natural history and clinical course to create a more robust understanding of this complex disease. Methods We prospectively studied 573 consecutive HCM patients in 3 centers (44 ± 17 years; 66% male) with New York Heart Association functional class I/II symptoms at study entry, including 249 in whom left ventricular outflow tract obstruction was absent both at rest and following physiological exercise (<30 mm Hg; nonobstructive HCM) and retrospectively assembled clinical follow-up data. Results Over a median follow-up of 6.5 years, 225 of 249 nonobstructive patients (90%) remained in classes I/II, whereas 24 (10%) developed progressive heart failure to New York Heart Association functional classes III/IV. Nonobstructive HCM patients were less likely to experience advanced limiting class III/IV symptoms than the 324 patients with outflow obstruction (1.6%/year vs. 7.4%/year rest obstruction vs. 3.2%/year provocable obstruction; p < 0.001). However, 7 nonobstructive patients (2.8%) did require heart transplantation for progression to end stage versus none of the obstructive patients. HCM-related mortality among nonobstructive patients was low (n = 8; 0.5%/year), with 5- and 10-year survival rates of 99% and 97%, respectively, which is not different from expected all-cause mortality in an age- and sex-matched U.S. population (p = 0.15). Conclusions HCM patients with nonobstructive disease appear to experience a relatively benign clinical course, associated with a low risk for advanced heart failure symptoms, other disease complications, and HCM-related mortality, and largely without the requirement for major treatment interventions. A small minority of nonobstructive HCM patients progress to heart transplant.
Abstract Overt symptomatic atrial fibrillation (AF) occurs in over 20% of hypertrophic cardiomyopathy (HC) patients leading to impaired quality of life, loss of productivity, and the risk for embolic ...stroke. However, the overall burden presented by AF in the HC population is unresolved due to the unknown frequency of silent asymptomatic episodes that do not necessarily achieve clinical recognition, but nevertheless may have important disease-related implications. Therefore, stored electrograms were analyzed retrospectively for AF in 75 consecutive HC patients (without prior AF history) implanted with dual chamber cardioverter-defibrillators (ICD). Patients were followed for 5.0 ± 4.1 years at the Tufts Medical Center HCM Institute; ages were 50 ± 15 years and 55% were male. ICD interrogation in the 75 patients showed AF to be absent in 54 (72%), 18 (24%) had clinically silent AF episodes, and the remaining 3 (4%) without prior asymptomatic episodes developed symptomatic and clinically overt paroxysmal AF. Of the 18 patients initially with clinically silent AF, 8 developed symptomatic AF, 4.1 ± 1.5 years later. Non-fatal embolic stroke occurred in one patient associated with clinically silent AF and without other risk factors. In conclusion, clinically silent AF appears to be common in HC, occurring in almost 25% of patients. Such asymptomatic episodes of AF have important future implications, including potential thromboembolic risk, and development of symptomatic and clinically overt AF requiring prophylactic anticoagulation.
Preparticipation screening of athletes with 12-lead electrocardiography has been promoted for the detection of asymptomatic cardiovascular disease, particularly hypertrophic cardiomyopathy (HC). ...Although false-positive electrocardiographic (ECG) results for HC are well recognized in athlete screening, expected false-negative rates are unknown. The aim of this study was to characterize the rate of false-negative ECG findings in a cohort of young asymptomatic patients with phenotypically expressed HC, defined by cardiovascular magnetic resonance, using the 2010 European Society of Cardiology recommended ECG criteria for the identification of suspected heart disease in trained athletes. Cardiac magnetic resonance studies and 12-lead electrocardiography were performed in 114 consecutive asymptomatic patients with HC aged ≤35 years (mean age 22 ± 8 years; 77% male patients). Electrocardiograms were analyzed to distinguish pathologic ECG patterns from alterations considered nonpathologic and physiologic consequences of athletic training. Among the 114 patients with HC, 103 (90%) demonstrated ≥1 pathologic ECG abnormality, while the remaining 11 patients (10%) had normal or nonpathologic ECG patterns and therefore defined a subgroup in whom ECG screening would not be expected to raise suspicion of heart disease (i.e., false-negative results). In this false-negative ECG results group, maximal left ventricular wall thickness was 17 ± 2 mm (range 15 to 21), compared to patients with pathologic ECG patterns, in whom maximal left ventricular wall thickness was 22 ± 5 mm (p = 0.003). In conclusion, a substantial minority of young asymptomatic patients with HC with phenotypically expressed left ventricular hypertrophy have nonpathologic ECG findings on the basis of the 2010 European Society of Cardiology guidelines. In principle, this high false-negative rate of 10% represents an important limitation in applying 12-lead electrocardiography to large, apparently healthy athletic populations for the detection of HC.
Protecting the Heart of the American Athlete Lawless, Christine E., MD, FACC, FACSM; Asplund, Chad, MD, FACSM; Asif, Irfan M., MD ...
Journal of the American College of Cardiology,
11/2014, Letnik:
64, Številka:
20
Journal Article
Recenzirano
Odprti dostop
The American College of Cardiology (ACC) Sports and Exercise Cardiology Section convened the "Think Tank to Protect the Heart of the American Athlete and Exercising Individual" on October 18, 2012, ...in Washington, DC.\n Asif University of Tennessee Family and Sports Medicine--Assistant Professor; Fellowship Director, Sports Medicine None None None None None None Ron Courson University of Georgia--Senior Associate Athletic Director None None None None None None Michael S. Emery Carolina Cardiology Consultants None None None None None None Anthon R, Fuisz Medstar Washington Hospital Center-Director, Cardiac MRI None None None None None None Richard J. Kovacs Krannert Institute of Cardiology-Professor of Clinical Medicine Biomedical Systems Insight Pharmaceuticals Theravancedagger Xenoport None None Biotie (DSMB) Eli Lilly (DSMB)dagger Cook Incorporated Med Institutedagger None Silvana M. Lawrence Baylor College of Medicine--Associate Professor, Department of Pediatrics, Section of Cardiology None None None None None None Benjamin D. Levine Institute for Exercise and Environmental Medicine None None None None None Defendant, 2013, Postural Orthostatic Tachycardia Syndrome Mark S. Link Tufts Medical Center None None None None None None Matthew W. Martinez Lehigh Valley Health Network None None None None None None G. Paul Matherne University of Virginia Health Sciences Center-Division Chief of Pediatric Cardiology None None None None None None Brian Olshansky University of Iowa Hospitals-Professor of Medicine Arrhythmia Grand Roundslow * BioControl Boehringer Ingleheim Boston Scientific (guidant) Combined Medicare Medicaid Services Daiichi Sankyo Gerson Lehman Medtronicdagger Sanofi Aventis None None Amarin (DSMB) Boston Scientific (DSMB) Sanofi Aventis (DSMB) Boston Scientific Executive Health Resourcesdagger Thompson Reuterslow * Defendant, 2013 Event Monitors Third Party, 2012, Cardiac Arrest William O. Roberts University of Minnesota Medical School-Professor, Department of Family Medicine and Community Health None None None None None None Lisa Salberg Hypertrophic Cardiomyopathy Association-Chief Executive Officer None None None None None None Victoria L. Vetter Children's Hospital of Philadelphia Division of Cardiology-Professor of Pediatrics None None None None None None Robert A. Vogel University of Colorado-Professor of Medicine Pritikin Longevity Center National Football Leaguedagger None None Sanofidagger None None Jim Whitehead American College of Sports Medicine-Executive Vice President/Chief Executive Officer * This table represents all relationships of committee members with industry and other entities that were reported by authors, including those not deemed to be relevant to this document, at the time this document was under development.
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