At our Egyptian tertiary care centre, hypertrophic cardiomyopathy has a relatively worse prognosis when compared with reports from Western and Asian series. Infants have a worse outcome than children presenting after the age of 1 year. A poorer prognosis in childhood hypertrophic cardiomyopathy is predicted by an extreme left ventricular hypertrophy, the presence of sinus tachycardia, and supraventricular tachycardia.

Our aim was (1) to detect the presence of fibrosis by Cardiac magnetic resonance imaging (CMR) in the pediatric age group. (2) Correlate CMR findings with demographic data, LV function, and other echocardiographic parameters. We studied 40 pediatric patients diagnosed as HCM by echocardiography. All patients were subjected to clinical examination (in which the NYHA classification was determined for each patient), echocardiography, and CMR. CMR was done on a 1.5T Philips Achieva scanner in SSFP with delayed myocardial enhancement (DE-MRI). All demographic and functional parameters as well as pressure gradient across left ventricular outflow tract (LVOT) were correlated with the percentage of myocardial enhancement. We studied 13 female and 27 male patients from 45 days up to 18 years. The mean percentage of DE-MRI was 9.7 ± 9%. We found significant correlation between the NYHA classification and the pressure gradient across the LVOT (P = < 0.001) as well as the percentage of DE-MRI (P = 0.004). The percentage of DE-MRI showed positive correlation with LV myocardial mass index (P = 0.042). It didn't correlate with any other demographic or LV functional cardiac parameters. A good positive correlation was detected between the percentage of DE-MRI and the severity of pressure gradient across LVOT measured by echocardiography (r = 0.69 and P = <0.001). We found a significant correlation between the percentage of DE-MRI in children with HCM and the pressure gradient across LVOT, NYHA classification, and LV myocardial mass. This may help in the further management of those patients, planning for follow-up, and prognosis of the disease.

Background/Aims: Congenital Adrenal Hyperplasia (CAH) patients are at higher risk of systemic hypertension secondary to high steroid dose therapy. Our aim was to look for early cardiovascular changes in young CAH patients, by the analysis of BP levels and echocardiographic parameters. We aimed also to investigate the possible risk factors that may contribute to such effects. Methods: We evaluated blood pressure measurements (using international percentiles) and echocardiography in 53 CAH pediatric patients. Thirty healthy age and sex matched children were included as controls. Results: It was found that 10(18.9%) patients had elevated BP and 23(43.4%) patients had hypertension. Out of those hypertensive patients, 7 (13.2%) patients had systolic hypertension, 3 (5.6%) patients had diastolic hypertension, and 13 (24.5%) patients had combined systolic and diastolic hypertension. BP levels of CAH cases were positively correlated with Hydrocortisone doses (r=0.14, P value=0.038) and negatively correlated with 17-OH-progesterone levels (r=-0.15, P value=0.023). Our patients had myocardial hypertrophy with increased left ventricular wall thickness, greater mean LVM and LVMI. Age and hypertension were predictors of LV hypertrophy. Conclusions: The present study showed that a considerable portion of CAH patients had hypertension. Their BP levels were influenced by Glucocorticoids (GCs) doses.

Introduction Duchenne muscular dystrophy (DMD) is known to impact the subepicardial layer of the myocardium through chronic inflammation. Recent animal studies have shown predominant subendocardial involvement in rats with DMD. The primary outcome parameter was to determine by cardiovascular MRI (CMR) if two differential patterns of myocardial involvements exist in DMD; the secondary outcome parameters were to correlate the observed pattern with metabolic markers such as insulin resistance measures. Methods Forty patients with DMD were screened using CMR to determine which of them had predominantly subendocardial dysfunction (SENDO group), or subepicardial/midmyocardial involvement (SEPMI group). Patients were subjected to body mass index measurement, serum creatinine kinase, serum lactate dehydrogenase enzyme, fasting glucose-insulin ratio (FGIR), full lipid profile, left ventricular ejection fraction (LVEF), left ventricle E/E´ ratio (the ratio of early mitral inflow velocity to average early diastolic velocities of the basal septum and mitral annulus) for left ventricle diastolic function, and myocardial layer strain discriminating echocardiography (MLSD-STE). Results: 26 patients displayed SENDO while 34 displayed SEPMI. SENDO group displayed overt insulin resistance; (FGIR (SENDO: 7 ± 1 vs. SEPMI: 5 ± 1, P < 0.001). FGIR was negatively correlated with Subendocardial Global Longitudinal Strain (ENDO-LS) with r = −0.75. Conclusion DMD does not seem to influence the heart uniformly; DMD cardiomyopathy probably has two separate phenotypes with different mechanisms. Insulin resistance might be implicated in its pathogenesis and its reversal may help to slow disease progression.

The relationship between myocardial iron load and eccentric myocardial remodeling remains an under-investigated area; it was thought that remodeling is rather linked to fibrosis. This study aims to determine whether or not measures of remodeling can be used as predictors of myocardial iron. For this purpose, 60 patients with thalassemia were studied with 3D echocardiography and myocardial relaxometry (T2*) by Cardiac MRI. 3D derived sphericity index was significantly higher in patients with myocardial iron load. It was correlated with T2* with a 100% sensitivity and specificity (cut-off value of 0.34) to discriminate between patients with and without myocardial iron overload.