Objective-To obtain normal M mode (one dimensional) echocardiographic values in a substantial sample of normal infants and children. Design-Data were obtained over three years from a single centre in central Europe. Patients-2036 healthy infants and children aged one day to 18 years. Methods-In line with recommendations for standardising measurements from M mode echocardiograms, and using digital echocardiographic equipment, measurements were obtained of the following: right ventricular anterior wall thickness at end diastole, right ventricular end diastolic dimension, thickness of interventricular septum at end diastole and end systole, thickness of posterior wall of the left ventricle at end diastole and end systole, left ventricular dimension at end diastole and end systole, pulmonary and aortic valve diameter, and left atrial dimension. Results-Measurements are presented graphically on centile charts with respect to body surface area, and as tables with mean and 2 SD values for newborns in relation to body weight, and for infants and children in relation to body surface area. Best fitting regression equations are given for each measured variable, using the 50th centile values. Conclusion-In comparison with previously published normal values, the presented charts and tables make it possible to judge echocardiographic measurements of a particular patient as normal or abnormal. (Heart 2000;83:667-672)
Aims: Fabry disease results from deficient activity of the lysosomal enzyme α‐galactosidase A. Progressive accumulation of the major substrates leads, in both men and women, to progressive hypertrophic cardiomyopathy. We aimed to evaluate the utility of different electrocardiographic (ECG) parameters for assessing the degree and severity of hypertrophic cardiomyopathy in patients with Fabry disease. Methods: A total of 166 ECGs of 94 hemi‐ and heterozygous patients with Fabry disease were analysed and compared with echocardiographic‐estimated left ventricular mass (LVM). Results: There was a significant (p > 0.0001) correlation between QRS duration (R2= 0.59), 12‐lead amplitude/duration product (R2= 0.61), Sokolow‐Lyon voltage/duration product (R2= 0.52) and LVM. Analysis of receiver operating characteristics revealed that the 12‐lead amplitude/duration product had the highest sensitivity‐specificity relationship (p > 0.01 compared with the Cornell index).
Conclusion: In general, ECG signs of left ventricular hypertrophy correlated well with LVM as revealed by echocardiography. Of the parameters studied, the 12‐lead amplitude/duration product was the most successful at describing the severity of cardiac involvement in Fabry disease. These data suggest that ECG parameters have potential for use as a simple and cost‐effective means of screening for hypertrophic cardiomyopathy in patients with Fabry disease.
Dissolution occurred in 57.2% of TCs within a mean of 25 months, and within 39.3 months all TCs showed decrease or loss of radio-opacity. Recanalisation of closed APCs occurred in 58.3%.
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