Evolving mortality and clinical scenarios in HCM: Where are we?

Hypertrophic cardiomyopathy (HCM) is a quite common genetic heart disease with a prevalence of 1:500 in the general population. 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. 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.