Our findings warrant attention for IRDS and diaphragmatic hernia, close monitoring of the aortic root early in life, and extensive vascular imaging afterwards. EM on skin biopsies shows disease-specific abnormalities.

Our aim was to determine, using a computer program for measurement and analysis, the effects, if any, of age and gender on the electrocardiographic measurements in a large cohort of Turkish children. We analyzed standard simultaneous 12-lead electrocardiograms from 2241 healthy Turkish children, aged from 1 day to 16 years, to calculate limits of normality of a variety of electrocardiographic measurements for 12 age groups. Clinically significant differences were shown to exist, and the results compared with previously established normal limits. Differences were demonstrated for gender in measurements of both amplitude and duration, particularly in the adolescent period. We have provided tables and figures showing age and gender-dependent means and upper limits of normal electrocardiographic parameters for the important leads.

Objective:Tricuspid annular plane systolic excursion (TAPSE) is an echocardiographic measurement used for evaluating right ventricular systolic function. While established reference values of TAPSE exist for the adult population, only a limited number of studies have attempted to evaluate reference values for the pediatric population. The aim of the present study was to determine the reference values for TAPSE in healthy children in Turkey.Methods:A total of 765 healthy children aged between 0 and 18 years, all of whom were referred to our clinic with cardiac murmurs, were evaluated prospectively. Patients with no cardiac pathologies or other disorders were excluded from the study. The measurement of TAPSE was obtained using a 2D-guided M-mode technique with echocardiography, and the relationship between age and surface area with TAPSE was investigated. The statistical analysis was carried out using the SPSS 20.0 software package (SPSS Inc., Chicago, IL, USA, 2012).Results:The mean TAPSE value was found to be 19.56±5.54 mm, and no significant difference was identified between male and female children. TAPSE values showed a positive correlation with increasing age and surface area. The mean TAPSE value was 9.09±1.36 mm in newborns and 25.91±3.60 mm in the 13-18 years age group. A negative correlation was seen between TAPSE and heart rate.Conclusion:In the present study, the reference values for TAPSE in healthy Turkish children were presented in percentile tables and the corresponding z-scores were determined. These reference values may be useful in daily practice for the evaluation of right ventricular systolic function in children.

Subvalvular aortic stenosis accounts for 1-2 % of all congenital heart disease and for 8-20 % of cases of left-ventricular outflow tract (LVOT) obstruction in children. Recurrence of subaortic stenosis (SAS) is not uncommon after surgical management. This study was performed to investigate the clinical and surgical outcomes and to estimate the predictability of recurrences of SAS. Seventy-nine patients age 3-21 years with SAS between 1994 and 2010 were reviewed. Fifty-one patients had discrete SAS, whereas the remaining 15 patients had fibromuscular ridge-type SAS. Mean follow-up time without surgery was 22 months (range of 1-94). Forty-one patients with a diagnosis of SAS underwent surgery. Recurrence rates were 22.7 % (15 patients), and these patients developed SAS at a mean of 4.7 years follow-up. We performed second surgical membrane resection in only 1 patient. The risk of recurrence of SAS was only linked to higher preoperative LVOT gradient. Twenty-three patients had no aortic regurgitation (AR) at preoperative echocardiography. Of these, 39.1 % had trivial, 8.7 % had mild, and 8.7 % had moderate AR after surgery; there was no significant AR. We conclude that surgical intervention was required most of the time in patients with SAS, and surgical outcomes was excellent even if there were associated cardiac defects. The risk of recurrences was higher, especially in patients with higher initial LVOT gradients, although a second surgery was rarely necessary in these patients.

SUMMARYThe purpose of this study was to determine the differences in the left ventricular dimensions, mass index and function in school-aged swimmers by echocardiography. The study group consisted of 82 swimmers who participated in a systematic swimming training for at least six months, and the control group consisted of 41 sedentary children of similar age, sex and weight. In the study group, left ventricular dimensions and wall thicknesses, aortic root and left atrium diameters, and left ventricle mass index were significantly greater than the normal children (p<0.05). However, there was no difference in the left ventricular systolic function (ejection fraction, shortening fraction) or in the left ventricular filling characteristics (p>0.05).In conclusion, in childhood swimmers there was a significant increase in left ventricular dimensions, wall thicknesses and mass index, but no differences in the systolic function and filling characteristics of the left ventricle. Thus, information on endurance training participation is necessary to interpret quantitative echocardiographic data. (Jpn Heart J 35: 295-300, 1994) Key words: Left ventricular dimension Diastolic function Training Swimming Children SCENT studies of the physiologic effects of exercise training indicate important differences between endurance athletes and untrained normal subjects. One of the more noticeable differences in endurance athletes is an increase in heart size.1-4) However children, particularly of elementary school age , are rarely classified as athletes.2)Although there are many reports on echocardiographic changes in adult athletes,1,3-7) the number of studies on school-aged athletes is relatively few . The