In 1958, the British forensic pathologist, Donald Teare, reported a family in which eight young people had died suddenly from asymmetrical hypertrophy of the left ventricle. Five decades on, the prevention of premature death from ventricular tachyarrhythmia, heart failure and stroke remains a major aim of clinical management in what is now called hypertrophic cardiomyopathy. In this paper, we review the underlying mechanisms of death and discuss the strengths and weaknesses of current international guidelines for the identification and treatment of high-risk patients.