Relieved by the alcohol

Over 60 years ago, Donald Teare 1 described eight cases of young patients, seven of whom died suddenly, whose hearts presented asymmetric septal hypertrophy resembling a cardiac tumor, which he termed ''a muscular hamartoma of the heart''.Since that time much has changed in our knowledge of hypertrophic cardiomyopathy (HCM).With regard to phenotypes, unlike what was previously believed, the hallmark of the disease (septal hypertrophy) is only seen in about two-thirds of patients.2 Even fibrosis can now be quantified by cardiac magnetic resonance imaging.3 Information on hemodynamics in HCM has advanced from the first descriptions of abnormal systolic anterior motion (SAM) of the mitral valve and resulting mitral regurgitation on angiography in the mid-1960s 4 to the ability to quantify obstruction at rest by provocative maneuvers or exercise testing.5 The view of the importance of genomics in HCM has also changed, moving toward broader, more inclusive models that include both biological risk and acquired factors to explain the disease.Mutations in a single sarcomere gene are no longer considered an adequate driving force for the disease, since they do not properly explain regional left ventricular hypertrophy and myocardial fibrosis, as well as structurally abnormal elongated mitral valve leaflets and remodeled intramural coronary arterioles, which involve tissue types that do not express cardiomyocyte sarcomere proteins.