Ventricular diastolic filling indices in pulmonary stenosis.

Children with valvar pulmonary stenosis have right ventricular diastolic filling abnormalities that may be due to either right ventricular hypertrophy or right ventricular outflow obstruction. In order to investigate the reason for this abnormality, 23 consecutive cases with pulmonary stenosis (mean age 7.94 +/- 3.33 years) undergoing transluminal pulmonary balloon valvuloplasty without significant tricuspid or pulmonary valvar regurgitation were studied prospectively. Right ventricular diastolic filling indices and pulmonary valvar systolic gradients were measured in these children one day before and after pulmonary balloon valvuloplasty and were re-examined six months later. Right ventricular diastolic indices based on rapid early diastolic filling peak velocity (peak E), peak velocity during atrial contraction (peak A), and ratio of E/A were determined by pulsed Doppler echocardiography. In conclusion, right ventricular diastolic filling indices in patients with pulmonary stenosis did not improve after pulmonary balloon valvuloplasty in the first day but when re-examined by the sixth month there was a significant improvement. These data suggest that diastolic filling abnormalities are more likely a result of right ventricular hypertrophy than of right ventricular outflow obstruction.

Quantitative morphometric analysis of progressive infundibular obstruction in tetralogy of Fallot. A prospective longitudinal echocardiographic study.

BACKGROUND: The morphological hallmark of tetralogy of Fallot is controversial, with much disagreement as to whether the subpulmonary infundibulum in this lesion is hypoplastic. In addition, few quantitative data are available regarding the morphometry of the subpulmonary infundibulum, what anatomic characteristics are acquired in the postnatal period, and at what rate they progress. We also sought to determine whether echocardiographic morphometric analysis of the infundibulum can predict clinical course in infants with tetralogy of Fallot. METHODS AND RESULTS: Twenty-one infants with tetralogy of Fallot (median age at initial study, 1.6 months) were prospectively followed with serial echocardiograms until the time of first surgical intervention (median age at surgery, 10 months). Selected video still frames were digitized off-line with a computerized system. Compared with age-matched normal control infants (n = 37), the following indexed infundibular dimensions in patients with tetralogy of Fallot were significantly smaller: length (1.86 +/- 0.54 versus 2.7 +/- 0.56 cm/BSA0.5, P < .0001), cross-sectional area (1.6 +/- 0.49 versus 4.7 +/- 1.3 cm2/BSA, P < .0001), and volume (1.24 +/- 0.62 versus 7.2 +/- 3 mL/BSA1.5, P < .0001). The following measurements were increased in tetralogy patients: infundibular septal thickness (0.83 +/- 0.21 versus 0.54 +/- 0.06 cm/BSA0.5, P = .0002) and infundibular free-wall thickness (0.62 +/- 0.13 versus 0.49 +/- 0.06 cm/BSA0.5, P = .006). The angle between infundibular septum and ventricular septum had a greater degree of anterosuperior deviation in tetralogy patients, resulting in a larger infundibuloventricular septal angle (77 +/- 8.2 degrees versus 31 +/- 6.5 degrees, P < .0001). During follow-up, infundibular volume in tetralogy patients decreased from 1.24 +/- 0.62 to 0.81 +/- 0.47 mL/BSA1.5 (P = .002), correlating with infundibular septal thickness (r = -.63, P < .003). The mean rate of decrease of indexed infundibular volume was 0.1 +/- 0.13 mL.BSA-15.mo-1. Correlation analysis revealed a nonlinear correlation between the degree of infundibular septal malalignment and indexed infundibular volume (r = .93, P < .0001). Tetralogy patients who required early surgical intervention (4.8 +/- 0.9 versus 10.7 +/- 1.7 months, P < .0001) had a smaller infundibulum at presentation (0.92 +/- 0.35 versus 1.41 +/- 0.67 mL/BSA1.5, P = .04) and an accelerated rate of infundibular narrowing (0.17 +/- 0.18 versus 0.06 +/- 0.08 mL.BSA-1.5.mo-1, P = .04). CONCLUSIONS: Compared with normal infants, the subpulmonary infundibulum in tetralogy of Fallot is characterized by a smaller volume, shorter and thicker infundibular septum, and anterosuperior deviation of the infundibular septum. Infundibular obstruction in tetralogy patients is progressive, with an average rate of decrease in indexed infundibular volume of 0.1 +/- 0.13 mL.BSA-1.5.mo-1. Infants who are likely to require early therapeutic intervention may be identified on their initial echocardiogram as having an infundibular volume of < 0.9 to 1.0 mL/BSA1.5.

Cardiac rhabdomyosarcoma diagnosed by echocardiography.

Primary, malignant, cardiac tumors are extremely rare in infants and children. Only a few cases of cardiac rhabdomyosarcoma have been reported in childhood. Echocardiography greatly increases the rate of correct diagnosis of cardiac tumors. There are some cases in the literature which were diagnosed by echocardiography and confirmed histopathologically. In this report, a 13-year-old girl with the clinical findings of cardiac tamponade and who was diagnosed as having a cardiac tumor by two-dimensional echocardiogram is presented An echodense, solid tumor with irregular borders extending especially toward the left ventricular cavity from the apex was demonstrated on echocardiography. The diagnosis was confirmed histopathologically on postmortem examination.

Plasma lipid profile in obese children and in children with hereditary predisposition to coronary heart disease.

This study examined risk factors for coronary disease and plasma triglycerides, total cholesterol, HDL, LDL cholesterol (C), Apo A-1, and Apo B lipoprotein levels in obese children (n = 107) aged 9-12 years old and in children (n = 64) hereditarily predisposed to coronary heart disease (CHD). In the latter group, children's fathers had a history of MI before 50 years of age. A control group of 30 children was also studied, and the plasma lipid profile was investigated in 35 fathers with premature myocardial infarction. Seventy-five percent of the obese children and 60% of the children hereditarily predisposed to CHD had three or more coronary artery disease risk factors. Plasma HDL-C (p < 0.001), LDL-C (p < 0.001-p < 0.05, respectively), Apo A-1 (p < 0.001), and Apo B (p < 0.001) levels were different in both groups compared with controls.

Atrial natriuretic peptide and aldosterone in sick premature neonates.

Plasma atrial natriuretic peptide (ANP) and aldosterone concentrations were investigated in 25 sick premature neonates. Group A consisted of 11 premature neonates with sepsis and group B of 14 neonates with hyperbilirubinemia. In both groups, ANP and aldosterone levels were found to be higher than in the controls. Group A concentrations of ANP and aldosterone were also higher than in group B.