Hypertrophic Cardiomyopathy

Clinical History

The specimen was obtained from a fit 16 year old boy who died after a game of squash. Other presenting features of this condition include dyspnoea, angina, cardiac arrhythmias and syncopal episodes. The classic murmur is a late ejection systolic murmur which increases with the Valsalva manoeuvre.

Pathology

The most striking feature of the specimen is the marked hypertrophy of the left ventricle. Notice the increased thickness of the septum compared to the rest of the left ventricular wall. Left ventricular volume is greatly reduced and the heart is not enlarged. The aortic valve shows no evidence of stenosis. This is an example of Hypertrophic Cardiomyopathy which has been called many different names in the past including: H.O.C.M. - Hypertrophic Obstructive Cardiomyopathy; A.S.H. - Asymmetrical Septal Hypertrophy; I.H.S.S. - Idiopathic Hypertrophic Subaortic Stenosis These terms are often inappropriate as the condition is not always asymmetrical, nor does it always cause aortic obstruction. This condition should be differentiated from LVH secondary to Hypertension or Aortic Valve Stenosis.