Abstract 14030: Hypertophic Cardiomyopathy in the Young: Patterns and Distribution of Hypertrophy

Background Patterns and distribution of hypertrophy in hypertrophic cardiomyopathy (HCM) has been well categorized in the adult population but not systematically described in children and teenagers. We used CMR to characterize the hypertrophy patterns of young patients with HCM and corresponding phenotypic-genotypic relationships. Methods We undertook a retrospective analysis of 42 children (33 male: 9 female) diagnosed with HCM who had undergone CMR between August 1999 to Feb 2011. Left ventricular volumes, mass and systolic function were measured. An assessment of the pattern of hypertrophy, presence of systolic anterior motion (SAM), degree of left ventricular outflow tract obstruction (LVOTO) and delayed enhancement (DE) was made from the images obtained and compared with echocardiograms performed at close chronological proximity. Results The mean age of the group was 12.7 years standard deviation (SD) +/- 3.3 years. Mean left ventricular mass index (LVMi) was 111.3+/-38.1g/m2. 31 of the 42 individual...