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Hypertrophic cardiomyopathy.

Skip to Main Content. Maron MD, Lisa Salberg. First published: 20 January About this book Written by the authority on hypertrophic cardiomyopathy HCM and an HCM patient, and fully endorsed by the Hypertrophic Cardiomyopathy Association HCMA , the leading advocacy and support organization, the 3rd Edition of this best-selling guide offers unparalleled insight into all aspects of living with and treating HCM.

Accessible and practical, A Guide to Hypertrophic Cardiomyopathy For Patients, Their Families and Interested Physicians 3rd Edition: Answers the most pressing questions posed by patients, their families, and non-specialists health professionals involved in their care Covers everything from what is HCM and initial diagnosis of this increasingly prevalent condition to the latest treatment options Reflects the first-ever HCM guidelines published by the American Heart Association in Author Bios Barry J. Free Access. Summary PDF Request permissions. Tools Get online access For authors.

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Email or Customer ID. Forgot password? Notably, the low mortality rate of 0. The patients with HCM-related mortality averted by these treatment interventions outnumbered the patients with HCM-related deaths by a ratio of almost , most striking in the age group of 29 years of age or younger 3. Similarly, appropriate ICD interventions interrupting ventricular tachycardia or fibrillation and out-of-hospital defibrillation greatly exceeded sudden deaths by 3.

Indeed, disabling heart failure symptoms developing after ICD interventions are rare in patients with HCM without evidence that the mechanism of death shifts from sudden death to progressive heart failure, in contrast to coronary artery disease. Advanced end-stage heart failure in the absence of outflow obstruction, with or without systolic dysfunction, is emerging as the predominant cause of death for patients with HCM, underscoring the need for novel treatment initiatives to extend survival in this patient subgroup.

The substantial progress made in HCM management detailed here was directly attributable to systematically applied clinical science and research intertwined with patient care, and it was driven by the energies of the practicing cardiology community. Therefore, these clinical strategies for HCM are distinct from the initiatives in molecular biology and basic science mandated by the National Institutes of Health 25 years ago, 77 focusing instead on single disease-causing sarcomere mutations and cellular mechanisms, 78 , 79 and skeptical of clinical risk stratification strategies.

Recent comprehensive cohort data have underscored the range of therapies now available to patients with HCM Figure 5 and redefined the mortality risk and potential for survival implicit in this disease. This paradigm has altered the historical perception of HCM as relentless and uniformly progressive, and a frequent cause of premature death, and also underscores the understanding achieved recently with regard to the natural history and the effectiveness of therapeutic interventions for this inherited heart disease.

Indeed, it is time for patients and physicians to put aside old myths and misconceptions and recognize what is possible in HCM, thus permitting a measure of optimism to pervade this disease, which has too frequently been encumbered by negativity. Our data also underscore the advantage of evaluating and treating patients in emerging multidisciplinary centers of excellence dedicated to HCM. Highly populous countries such as India 87 and China may not yet possess all the necessary resources to take full advantage of the recent advances in management strategies, and many patients from diverse cultures may not be psychologically amenable to major treatment interventions, such as the ICD.

However, unmet needs remain in the management of patients with HCM, including a more specific risk stratification algorithm with reduction in excess device implants, targeted pharmacologic therapy to control symptoms, improved strategies for reversing or controlling advanced heart failure unrelated to obstruction, and, for genetics, refining the classification of pathogenic disease-causing sarcomere mutations and its relation to outcome. Nevertheless, the clinical initiatives and low mortality rates reported here represent what can now be achieved when contemporary management options and decision making are used in the care of patients with HCM.

Corresponding Author: Barry J. Published Online: March 2, Author Contributions: Drs B. Maron and M. Maron had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Critical revision of the manuscript for important intellectual content: All authors.

All Rights Reserved. Figure 1. View Large Download. Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine. J Am Coll Cardiol. PubMed Google Scholar Crossref. Clinical impact of contemporary cardiovascular magnetic resonance imaging in hypertrophic cardiomyopathy. The natural and unnatural history of hypertrophic obstructive cardiomyopathy.

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A Guide to Hypertrophic Cardiomyopathy For Patients, Their Families and Interested Physicians

Hypertrophic cardiomyopathy: an important cause of sudden death. Arch Dis Child. Evolution of hypertrophic cardiomyopathy to a contemporary treatable disease. Hypertrophic cardiomyopathy: from genetics to treatment. Eur J Clin Invest. Experimental therapies in hypertrophic cardiomyopathy. J Cardiovasc Transl Res.

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Silencing sudden death. Harvard Medical School News. October 3, Accessed March 6, Hypertrophic cardiomyopathy: can the horse be put back in the barn? Sudden death in hypertrophic cardiomyopathy. N Engl J Med. Risk assessment in hypertrophic cardiomyopathy: contemporary guidelines hampered by insufficient evidence. Asymmetrical hypertrophy of the heart in young adults.

Br Heart J. Idiopathic hypertrophic subaortic stenosis: I, a description of the disease based on the analysis of 64 patients.

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Idiopathic hypertrophic subaortic stenosis: II, operative treatment and the results of pre- and postoperative hemodynamic evaluations. Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy. Long-term survival in patients with resting obstructive hypertrophic cardiomyopathy comparison of conservative versus invasive treatment.

A contemporary European experience with surgical septal myectomy in hypertrophic cardiomyopathy. Eur Heart J. Contemporary surgical management of hypertrophic cardiomyopathy, the need for more myectomy surgeons and disease-specific centers, and the Tufts initiative. Am J Cardiol. The year history, controversy, and clinical implications of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy from idiopathic hypertrophic subaortic stenosis to hypertrophic cardiomyopathy: from idiopathic hypertrophic subaortic stenosis to hypertrophic cardiomyopathy.

Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy: II, effects on exercise capacity and symptomatic status. Multicenter study of the efficacy and safety of disopyramide in obstructive hypertrophic cardiomyopathy. Clinicopathologic features of hypertrophic cardiomyopathy managed by cardiac transplantation. Contemporary insights and strategies for risk stratification and prevention of sudden death in hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy in a large community-based population: clinical outcome and identification of risk factors for sudden cardiac death and clinical deterioration. Sudden deaths in young competitive athletes: analysis of deaths in the United States, Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 3: hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and other cardiomyopathies, and myocarditis: a scientific statement from the American Heart Association and American College of Cardiology.

Evidence that pharmacological strategies lack efficacy for the prevention of sudden death in hypertrophic cardiomyopathy. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. Termination of malignant ventricular arrhythmias with an implanted automatic defibrillator in human beings. Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. Prevention of sudden cardiac death with implantable cardioverter-defibrillators in children and adolescents with hypertrophic cardiomyopathy.

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Circadian rhythm of appropriate implantable cardioverter defibrillator discharges in patients with hypertrophic cardiomyopathy. Pacing Clin Electrophysiol. Determinants of implantable defibrillator discharges in high-risk patients with hypertrophic cardiomyopathy. Long-term follow-up of implantable cardioverter defibrillator therapy for hypertrophic cardiomyopathy. Implantable cardioverter-defibrillators in hypertrophic cardiomyopathy: patient outcomes, rate of appropriate and inappropriate interventions, and complications.

Am Heart J. Outcome and complications after implantable cardioverter defibrillator therapy in hypertrophic cardiomyopathy: systematic review and meta-analysis. Circ Heart Fail. Implantable cardioverter-defibrillator in patients with hypertrophic cardiomyopathy: efficacy and complications of the therapy in long-term follow-up.

J Cardiovasc Electrophysiol. Incidence of adequate ICD interventions in patients with hypertrophic cardiomyopathy supposed to be at high risk for sudden cardiac death. Acta Cardiol. Implantable cardioverter-defibrillators in patients with hypertrophic cardiomyopathy—dilemmas and difficulties. Kardiol Pol. The implantable cardioverter-defibrillator and hypertrophic cardiomyopathy: experience at three centers [in Spanish].

Rev Esp Cardiol. Long-term follow-up of hypertrophic cardiomyopathy patients with implantable cardioverter-defibrillators [in Spanish]. Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies.


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Hypertrophic cardiomyopathy in children, adolescents, and young adults associated with low cardiovascular mortality with contemporary management strategies. Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: systematic review of clinical risk markers. Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy.

Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy. Risk of sudden death and outcome in patients with hypertrophic cardiomyopathy with benign presentation and without risk factors. Evolving treatment for patients with hypertrophic obstructive cardiomyopathy.

Transaortic chordal cutting: mitral valve repair for obstructive hypertrophic cardiomyopathy with mild septal hypertrophy. Low operative mortality achieved with surgical septal myectomy: role of dedicated hypertrophic cardiomyopathy centers in the management of dynamic subaortic obstruction.

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Symptom assessment and exercise impairment in surgical decision making in hypertrophic obstructive cardiomyopathy: relationship to outcomes. J Thorac Cardiovasc Surg.

Clinical and echocardiographic determinants of long-term survival after surgical myectomy in obstructive hypertrophic cardiomyopathy. Short and medium term outcomes of surgery for patients with hypertrophic obstructive cardiomyopathy. Ann Thorac Surg. Surgical septal myectomy versus alcohol septal ablation: assessing the status of the controversy in Long-term outcomes after medical and invasive treatment in patients with hypertrophic cardiomyopathy.