Abstract
YPERTROPHIC CARDIOMOPATHY (HCM) is a complex condition with an extensive phenotypic expression and a broad clinical spectrum. Mutations in the sarcomeric proteins lead to abnormal hypertrophy, especially of the interventricular septum. 1 The prevalence of this condition in the adult population is approximately 0.2%, and it is considered the most common genetic cardiac disease. 2 The most common phenotype is characterized by dynamic left ventricular outflow tract (LVOT) obstruction resulting from basal septal hypertrophy and systolic anterior motion (SAM) of the anterior mitral valve leaflet. The rapid ejection of blood across a narrowed outflow tract results in a Venturi or drag effect, which, in turn, draws the mitral leaflet and chordae toward the interventricular septum. 3 Interestingly, similar degrees of basal hypertrophy can have different degrees of LVOT obstruction and symptoms. The clinical diagnosis is reliably confirmed with 2-dimensional echocardiography and Doppler measurement of outflow tract gradients. The authors present an unusual cause of LVOT obstruction in a patient who carried the diagnosis of HCM and who had undergone prior operations for the management of subaortic stenosis.
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