UCL Medical School, University College London, London, UK.
Institute of Cardiovascular Science, University College London, London, UK.
Echocardiography. 2022 Sep;39(9):1233-1239. doi: 10.1111/echo.15437. Epub 2022 Aug 17.
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.
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.
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.
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.
肥厚型心肌病（HCM）是一种常染色体显性遗传性心脏病，其特征是左心室壁增厚和异常负荷状态。影像学检查是诊断和风险分层的首选方法。尽管从心脏成像方面广泛描述了 HCM 成人的心脏大小，但关于受 HCM 影响的儿童的信息有限。本研究旨在评估诊断为 HCM 的一小部分儿童的左心室功能和左心尺寸。
本研究共纳入了 16 名（男 7 名，女 9 名）平均年龄为 14.0±2.5 岁的在大奥蒙德街儿童医院诊断为 HCM 的儿科患者。使用心脏磁共振（CMR）图像在 Simpleware ScanIP 中测量左、右心室的大小以及室间隔和左心室游离壁的厚度。根据样本分布，使用学生 t 检验或非参数曼-惠特尼 U 检验比较性别组之间的差异。
男性和女性之间的心率、左心室舒张末期容积和舒张末期容积指数、左心室每搏量和每搏量指数、左心室收缩末期长轴长度、左心室收缩末期长轴长度指数、左心室舒张末期中腔直径、左心室舒张末期游离壁厚度、左心室舒张末期游离壁厚度指数、右心室舒张末期长轴长度存在统计学差异。
左心室壁和室间隔厚度增加会影响左心室腔尺寸，并且 HCM 患者的男性和女性之间可能存在解剖和生理参数的差异。
