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A patient was found to have unexplained left ventricular and septal hypertrophy at age 11, but no pressure gradient across the left ventricular outflow tract. At age 13, there was a mean ejection gradient of 82 mm. Hg. The patient died suddenly 1 month later. This patient demonstrates the development of muscular subaortic stenosis in idiopathic myocardial hypertrophy with obstruction due to increasing muscular hypertrophy.A second patient with left ventricular hypertrophy secondary to a fixed left ventricular outflow tract obstruction produced by an anomalous insertion of a mitral valve leaflet developed additional obstruction due to septal hypertrophy.We believe that the cases presented establish that “muscular subaortic stenosis” may be the result of ventricular hypertrophy of different etiologies. A schema is presented to assist classification of muscular subaortic stenosis.

The Development of Outflow Tract Obstruction of the Left Ventricle in Idiopathic Myocardial Hypertrophy