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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. [ABSTRACT FROM AUTHOR]

Background: Pediatric patients are becoming increasingly referred for cardiovascular magnetic resonance (CMR). Measurement of ventricular wall thickness is typically part of the assessment and can be of diagnostic importance, e.g. in arterial hypertension. However, normal values for left ventricular (LV) and right ventricular (RV) wall thickness in pediatric patients are lacking. The aim of this study was to establish pediatric centile charts for segmental LV and RV myocardial thickness in a retrospective multicenter CMR study. Methods: CMR was performed in 161 healthy children and adolescents with an age range between 6 and 18 years from two centers in the UK and Germany as well as from a previously published CMR project of the German Competence Network for Congenital Heart Defects. LV myocardial thickness of 16 segments was measured on the short axis stack using the American Heart Association segmentation model. In addition, the thickness of the RV inferior and anterior free wall as well as biventricular mass was measured. Results: The mean age (standard deviation) of the subjects was 13.6 (2.9) years, 64 (39.7%) were female. Myocardial thickness of the basal septum (basal antero- and inferoseptal wall) was 5.2 (1.1) mm, and the basal lateral wall (basal antero- and inferolateral) measured 5.1 (1.2) mm. Mid-ventricular septum (antero- and inferoseptal wall) measured 5.5 (1.2) mm, and mid-ventricular lateral wall (antero- and inferolateral wall) was 4.7 (1.2) mm. Separate centile charts for boys and girls for all myocardial segments and myocardial mass were created because gender was significantly correlated with LV myocardial thickness (p < 0.001 at basal level, p = 0.001 at midventricular level and p = 0.005 at the apex) and biventricular mass (LV, p < 0.001; RV, p < 0.001). Conclusion: We established CMR normal values of segmental myocardial thickness and biventricular mass in children and adolescents. Our data are of use for the detection of abnormal myocardial properties and can serve as a reference in future studies and clinical practice. [ABSTRACT FROM AUTHOR]

Objective To describe the sensitivity of fetal anomaly scanning at detecting transposition of the great arteries (TGA) and to investigate whether prenatal detection improves survival. Design Retrospective review of survival by comparing those who had an antenatal diagnosis with those who did not. Setting Population-based study in Wessex region over 13 years. Population Babies with isolated TGA and an intact ventricular septum. Methods Review of outcomes by comparing those who had an antenatal diagnosis with those who did not. Main outcome measures Mortality rates in each group. Results TGA occurred more commonly in boys than in girls. Using the existing national screening policy, the antenatal detection rate of TGA was only 6.9% over the study period, improving to 25% in the last 4 years. This contrasts with a 40% detection rate when TGA was associated with a ventricular septal defect (VSD). All the babies who had TGA diagnosed antenatally survived through surgery. Of those who were not diagnosed antenatally, two were stillborn, five died before the diagnosis was made and four died after surgery. Although the difference in survival rates between those who were antenatally diagnosed and those who were not is not statistically significant (χ2= 3.9; P = 0.11), some of these deaths could have been prevented if a prenatal diagnosis had been made. Conclusions Improved antenatal diagnosis could lead to a significant reduction in the mortality associated with TGA. The current low detection rate of TGA in the UK could be improved by the inclusion of outflow tract views in routine fetal anomaly scans, and we believe that the extra workload is justified. [ABSTRACT FROM AUTHOR]

Background: The associations between cardiovascular disease (CVD) risk factors and the biventricular geometry of the right ventricle (RV) and left ventricle (LV) have been difficult to assess, due to subtle and complex shape changes. We sought to quantify reference RV morphology as well as biventricular variations associated with common cardiovascular risk factors. Methods: A biventricular shape atlas was automatically constructed using contours and landmarks from 4329 UK Biobank cardiovascular magnetic resonance (CMR) studies. A subdivision surface geometric mesh was customized to the contours using a diffeomorphic registration algorithm, with automatic correction of slice shifts due to differences in breath-hold position. A reference sub-cohort was identified consisting of 630 participants with no CVD risk factors. Morphometric scores were computed using linear regression to quantify shape variations associated with four risk factors (high cholesterol, high blood pressure, obesity and smoking) and three disease factors (diabetes, previous myocardial infarction and angina). Results: The atlas construction led to an accurate representation of 3D shapes at end-diastole and end-systole, with acceptable fitting errors between surfaces and contours (average error less than 1.5 mm). Atlas shape features had stronger associations than traditional mass and volume measures for all factors (p < 0.005 for each). High blood pressure was associated with outward displacement of the LV free walls, but inward displacement of the RV free wall and thickening of the septum. Smoking was associated with a rounder RV with inward displacement of the RV free wall and increased relative wall thickness. Conclusion: Morphometric relationships between biventricular shape and cardiovascular risk factors in a large cohort show complex interactions between RV and LV morphology. These can be quantified by z-scores, which can be used to study the morphological correlates of disease. [ABSTRACT FROM AUTHOR]