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- W4319335498 abstract "Hypertrophic cardiomyopathy (HCM) is a quite common genetic heart disease with a prevalence of 1:500 in the general population [ [1] Semsarian C. Ingles J. Maron M.S. Maron B.J. New perspectives on the prevalence of hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 2015 Mar 31; 65: 1249-1254 Crossref PubMed Scopus (679) Google Scholar ]. It has been 65 years since the disease was first described, and tremendous progress has been made in understanding the natural history and clinical management of HCM, so that our perception of the disease has progressively changed. As opposed to what believed in the past, it is now evident that patients with HCM in many cases can lead normal lives, and only a minority has serious disease-related complications. In the past two decades, the introduction of septal reduction therapies, associated to heart transplantation (HTx) and ventricular assist devices (VADs) have changed the prognosis of even the most critically ill patients. Furthermore, advances in arrhythmic risk stratification have enabled an increasingly better selection of candidates to implantable defibrillators (ICDs), mostly in primary prevention. As a consequence, a significant reduction in sudden cardiac deaths (SCD), which often affect younger HCM-patients, has been obtained and the overall reduction in HCM-related mortality decreased from 1.5%/year in the 1990s to a current estimated mortality of 0.5%/year [ [2] Maron B.J. Rowin E.J. Casey S.A. Maron M.S. How hypertrophic cardiomyopathy became a contemporary treatable genetic disease with low mortality: Shaped by 50 years of clinical research and practice. JAMA Cardiol. 2016; 1 (American Medical Association): 98-105 Crossref PubMed Scopus (151) Google Scholar , [3] Rowin Ethan J. Maron Martin S. Casey Susan A. Link Mark S. Chan Raymond H. Appelbaum Evan et al. Abstract 13294: evidence for reduced mortality in an adult cohort with hypertrophic cardiomyopathy. Circulation [Internet]. 2013; ([cited 2023 Jan 10]. Available from:)https://doi.org/10.1161/circ.128.suppl_22.A13294#d11133121e1 Crossref Google Scholar ]. The above therapeutic strategies had not only affected the life expectancy, but probably also the main clinical scenarios encountered in clinical practice, particularly regarding the end of life of patients with severe forms of HCM. Clinical scenarios of hypertrophic cardiomyopathy-related mortality: Relevance of age and stage of disease at presentationInternational Journal of CardiologyVol. 374PreviewThe evolving epidemiology of hypertrophic cardiomyopathy (HCM) has progressively changed our perception of HCM-related mortality. However, recent studies detailing individual causes of death based on age and clinical setting are lacking. Thus, the present study aimed to describe the modes of death in a consecutive cohort of HCM patients based on presenting clinical features and stage of disease. Full-Text PDF" @default.
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- W4319335498 date "2023-04-01" @default.
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- W4319335498 title "Evolving mortality and clinical scenarios in HCM: Where are we?" @default.
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- W4319335498 doi "https://doi.org/10.1016/j.ijcard.2023.02.001" @default.
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