DESCRIPTIONAlthough hypertrophic cardiomyopathy (HCM) is classically considered a disease of the left ventricle (LV), we present a rare case of biventricular HCM presenting in middle age.A 57-year-old man without a family history of HCM and sudden death presented with presyncope and New York Heart Association (NYHA) class II breathlessness. Clinical examination was normal except for ejection systolic murmur. ECG showed biventricular hypertrophy with deep T-wave inversions. Echocardiography demonstrated pronounced asymmetric LV hypertrophy and thickened right ventricle (RV) muscular components (figure 1A-C; videos 1-3). Doppler revealed a peak LV mid-cavity gradient of 109 mm Hg and subvalvar obstruction of the RV outflow tract with a peak gradient of 138 mm Hg ( figure 2A-C). The patient underwent successful surgical myectomy and has been stable during the course of follow-up. The histopathological specimen revealed myofibril disarray as is expected in a case of HCM (figure 3). Genetic testing for Noonan's syndrome and three of the most common mutations were performed which were negative.
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