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• W1983202419 abstract "Management of patients with hypertrophic cardiomyopathy (HCM) is implicitly based on their estimated risk of sudden cardiac death due to ventricular arrhythmias. 1 Spirito P Seidman CE McKenna WJ Maron BJ The management of hypertrophic cardiomyopathy. N Engl J Med. 1997; 336: 775-785 Crossref PubMed Scopus (874) Google Scholar , 2 Maron BJ Shen WK Link MS et al. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med. 2000; 342: 365-373 Crossref PubMed Scopus (849) Google Scholar Currently, risk cannot be defined with absolute accuracy but several important generalizations can be made. First, the bulk of outcome data is derived from tertiary referral centres and is biased towards highrisk patients. 1 Spirito P Seidman CE McKenna WJ Maron BJ The management of hypertrophic cardiomyopathy. N Engl J Med. 1997; 336: 775-785 Crossref PubMed Scopus (874) Google Scholar By contrast, data derived from populationbased studies of unselected patients suggest that the rate of sudden cardiac death is much lower and, in some studies, approaches that of matched controls, which underlines the fact that most patients with HCM do well. 3 Shapiro LM Zezulka A Hypertrophic cardiomyopathy: a common disease with a good prognosis. Five year experience of a district general hospital. Br Heart J. 1983; 50: 530-533 Crossref PubMed Scopus (56) Google Scholar , 4 Cannan CR Reeder GS Bailey KR Melton 3rd, LJ Gersh BJ Natural history of hypertrophic cardiomyopathy: a population-based study, 1976 through 1990. Circulation. 1995; 92: 2488-2495 Crossref PubMed Scopus (187) Google Scholar , 5 Maron BJ Olivotto I Spirito P et al. Epidemiology of hypertrophic cardiomyopathy-related death: revisited in a large non-referral-based patient population. Circulation. 2000; 102: 858-864 Crossref PubMed Scopus (643) Google Scholar Second, patients who present with sustained ventricular tachycardia or ventricular fibrillation, and also some who have a strong family history of sudden cardiac death, have already declared themselves at high risk and should be given an implantable defibrillator (ICD). 2 Maron BJ Shen WK Link MS et al. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med. 2000; 342: 365-373 Crossref PubMed Scopus (849) Google Scholar , 6 Elliott PM Sharma S Varnava A Poloniecki J Rowland E McKenna WJ Survival after cardiac arrest or sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 1999; 33: 1596-1601 Crossref PubMed Scopus (189) Google Scholar This view is supported by recent studies showing a likelihood of up to 11% of appropriate ICD discharges per year in these patients. 2 Maron BJ Shen WK Link MS et al. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med. 2000; 342: 365-373 Crossref PubMed Scopus (849) Google Scholar Third, for most patients uncertainties in the assessment of risk may be coming to an end with recent studies indicating that a low-risk group can be defined. 7 Elliott PM Poloniecki J Dickie S et al. Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol. 2000; 36: 2212-2218 Crossref PubMed Scopus (764) Google Scholar , 8 Spirito P Bellone P Harris KM Bernabo P Bruzzi P Maron BJ Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy. N Engl J Med. 2000; 342: 1778-1785 Crossref PubMed Scopus (853) Google Scholar There remain, however, considerable difficulties in identifying which patients warrant prophylactic implantation of a defibrillator. Relation between severity of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathyThe risk of sudden death associated with a wall thickness of 30 mm or more in patients without other risk factors is insufficient to justify aggressive prophylactic therapy. Most sudden deaths occurred in patients with wall thickness less than 30 mm, so the presence of mild hypertrophy cannot be used to reassure patients that they are at low risk. Full-Text PDF" @default.
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• W1983202419 date "2001-02-01" @default.
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• W1983202419 title "Risk management in hypertrophic cardiomyopathy" @default.
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• W1983202419 doi "https://doi.org/10.1016/s0140-6736(00)04022-8" @default.
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