DESCRIPTION

Although 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 inver-sions. Echocardiography demonstrated pronounced asymmetric LV hypertrophy and thickened right ven-tricle (RV) muscular components (figure 1A–C; videos 1–3). Doppler revealed a peak LV mid-cavity gradient of 109 mmHg and subvalvar obstruction of the RV outflow tract with a peak gradient of 138 mmHg (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. Figure 1 (A) Parasternal long-axis view showing asymmetric septal hypertrophy. (B) Parasternal short-axis view showing biventricular hypertrophy. (C) Parasternal long-axis view showing hypertrophied muscle bundles in right ventricular (RV) outflow tract with colour flow turbulence (LV, left ventricle; RVOT, right ventricular outflow tract). Video 1 Parasternal long axis view showing biventricular hypertrophy. Hirapur I, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-206271