Hypertrophic Cardiomyopathy

Abstract

Hypertrophic cardiomyopathy has intrigued clinicians since the condition was first described in eight patients by Donald Teare almost 40 years ago.¹ The classic morphology of asymmetrical septal hypertrophy remains the most common form of the condition, but the increased availability of echocardiography, together with family and routine screening has demonstrated a diversity of cardiac phenotype.^2–5 Patients may be profoundly disabled or asymptomatic,6 and presentation may occur at any age.^7–9 Although the early studies focused on the classic form of the disease, the heterogeneity of the cardiac phenotype precludes a satisfactory definition, and diagnostic difficulty is relatively common. A working definition is to regard hypertrophic cardiomyopathy as an idiopathic condition characterised by a hypertrophied and non-dilated left and/or right ventricle in the absence of an identified cause such as hypertension or valvular heart disease.^10,11 A positive family history greatly strengthens the diagnosis in uncertain cases.^12,13