Background: Hypertrophic cardiomyopathy (HCM) is an inherited autosomal dominant heart disease, characterized by increased left ventricular wall thickness and abnormal loading conditions. Imaging modalities are the first choice for diagnosis and risk stratification. Although heart dimensions have been characterized widely in HCM adults from cardiac imaging, there is limited information about children affected by HCM. The aim of this study is to evaluate left ventricular function and left heart dimensions in a small population of children diagnosed with HCM.
Methods: A total of 16 (seven male, nine female) pediatric patients with an average age of 14.0 ± 2.5 years diagnosed with HCM at Great Ormond Street Hospital for Children were included in this study. Cardiac magnetic resonance (CMR) images were used to measure left and right ventricular dimensions, and septal and left ventricular free wall thicknesses in Simpleware ScanIP. The gender groups were compared using student t-test or non-parametric Mann-Whitney U-test depending on the sample distribution.
Results: Differences in heart rate, left ventricular end-diastolic volume and end-diastolic volume index, left ventricular stroke volume and stroke volume index, left ventricular end-systolic long axis length, left ventricular end-systolic long axis length index, left ventricular end-diastolic mid-cavity diameter, left ventricular end-diastolic free wall thickness, left ventricular end-diastolic free wall thickness index, right ventricular end-diastolic long axis length were statistically significant in males and females.
Conclusion: Left ventricular wall and intraventricular septal thickness increase affecting left ventricle cavity dimensions and there may be differences in anatomical and physiological parameters in males and females affected by HCM.
Keywords: cardiac magnetic resonance imaging; hypertrophic cardiomyopathy; pediatric patients.
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