Left ventricular diastolic functions in juvenile rheumatoid arthritis.

Cardiac involvement as pericarditis, myocarditis, and endocarditis is common in juvenile rheumatoid arthritis (JRA). Though there are many reports concerning systolic and diastolic functions of adults with rheumatoid arthritis, there are no studies on children with JRA. Thirty patients with JRA without any cardiac symptoms and 30 sex- and age-matched controls were included in the study. M-mode and pulsed-wave Doppler echocardiography were performed on each participant to assess the systolic and diastolic functions of the left ventricle. Left ventricular end-systolic diameter and volume were larger and ejection fraction and fractional shortening were decreased in the JRA group. Among the diastolic parameters, increased late flow velocity, decreased early flow velocity, and prolonged isovolumic relaxation time reflected an abnormal relaxation form of diastolic dysfunction. Mortality rate is increased in adults with rheumatoid arthritis, and ischemic heart disease is the leading cause of cardiovascular mortality. The abnormal relaxation form of diastolic dysfunction found in children with JRA is seen in ischemic heart disease. These children can therefore be candidates for ischemic heart disease in the future even though they are fully asymptomatic at present. In conclusion, children with JRA should be assessed for systolic and diastolic functions with serial echocardiography. In this way it may be possible to reduce the mortality and morbidity of the disease from cardiac causes.

Malformations Due to Warfarin: A Case Report.

We report a 5-month-old infant with severe growth retardation who was exposed to in utero warfarin for the first 4 months. She had a dysmorphic face with depressed nasal hypoplasia and low-set ears. Cranial computerized tomography revealed bifrontal atrophy, agenesis of corpus callosum, and dilation of lateral ventricles. In addition she had patent ductus arteriosus with pulmonary hypertension, which had to be ligated.

Prophylaxis of the occurrence of hyperbilirubinemia in relation to maternal oxytocin infusion with steroid treatment.

This study was carried out to investigate the steroid prevention on the occurrence and the severity of red blood cell destruction by the effect of oxytocin usage for labor induction. Venous cord blood was collected from the pregnancies who had oxytocin-induced or augmented labors (20), oxytocin-infused deliveries with steroid use (20), deliveries without oxytocin use (20) and cesarean sections (20). Evaluation of the data showed significant increase in serum bilirubin level, serum lactic dehydrogenase activity, erythrocyte fragility and reticulocyte count (p < 0.0083), and a significant decrease in hemoglobulin concentration, packed red cell volume fraction (p < 0.01) in groups with labor induction or augmentation with oxytocin in comparison to deliveries with oxytocin plus steroid use and the two other methods of delivery. Moreover, with regard to the above data, no significant difference was observed between the deliveries other than oxytocin-only use. Mean corpuscular volume in the oxytocin group was apparently (not significant) higher than the steroid group. The results of this study suggest that the use of 16 mg dexamethasone 21-phosphate at the beginning of the induction or augmentation of labor with oxytocin, followed by an additional 4-mg dose 4 h later intravenously, is advantageous for the prevention of erythrocyte destruction.

Hepatic artery aneurysm in a 10-year-old boy as a complication of infective endocarditis.

Mycotic aneurysms occur from septic emboli in patients with infective endocarditis and may involve any artery, but frequently they are not detected before autopsy. The most common sites are the brain, abdominal aorta, sinus of Valsalva, ligated ductus arteriosus, and superior mesenteric, splenic, coronary, and pulmonary arteries. The authors report on a 10-year-old boy who had a mycotic aneurysm of the common hepatic artery, which developed during the course of infective endocarditis of the mitral valve and was cured successively using a platinum coil embolization technique.

Heart disease in Friedreich's ataxia: a clinical and echocardiographic study.

Twelve patients with Friedreich's ataxia (FA) were evaluated clinically and echocardiographically for evidence of heart disease. Electrocardiographic and echocardiographic abnormal findings were discovered in eight (67%) and seven (58%) children, respectively. A high incidence of cardiac involvement is well known in FA cases. Although the patient number in the present study is small, the findings are consistent with those in the literature. The most common pathology was asymmetric septal hypertrophy (ASH), followed by concentric left ventricular hypertrophy (CLVH) and dilated cardiomyopathy (DC).

Spontaneous closure of interatrial septal openings in infants: an echocardiographic study.

Four-hundred-and-fifteen neonates were evaluated by echocardiographic means in order to detect interatrial septal openings and were followed for a maximum time of 18 months. In 68.67% of them, interatrial septal openings were present at the first week of life. The defects were larger than 3 mm in 50.18% of these infants. At the end of the 18th month, openings persisted in 3 cases. The statistical analyses showed significant difference about the percent of closing between the groups with initial opening sizes lesser and bigger than 3 mm. Also, there was a positive correlation between the initial size and the spontaneous closure time. Spontaneous closure incidences were not different in boys and girls. In a small group of infants (4.64%), interatrial openings close forming septal aneurysms. In 12.3% of the newborns without an opening, septal aneurysms were detected during the initial evaluation.

Echocardiographic findings in some metabolic storage diseases.

This study was planned to determine the presence and extent of cardiac involvement in metabolic storage diseases, including types I and II glycogenoses, Gaucher and Neimann-Pick diseases, galactosialidosis and mucopolysaccharidosis. M-mode, 2-dimensional and Doppler echocardiographic studies were used for the determination of left ventricular wall thickness, systolic function, anatomic derangement, valvular dysfunction and left ventricular diastolic function in the patient and control groups. In 19.35% of the cases valvular involvement, and in 59.26% thickening of the left ventricular wall due to metabolic storage, was found. Left ventricular systolic function was in the normal range. The diastolic mitral flow patterns of the cases showed obstructive type changes. It can be concluded that in this study, besides previously described cardiovascular changes, some new findings have been detected by means of echocardiography in patients with metabolic storage diseases.

Partial anomalous pulmonary venous return. Evaluation of 51 cases.

Partial anomalous pulmonary venous return (PAPVR) is a congenital anomaly in which one or more, but not all, of the pulmonary veins are connected to a systemic vein or to the right atrium directly. Its incidence is higher in autopsy series than in clinical series. We report 51 cases of PAPVR diagnosed by cardiac catheterization and evaluated from the aspects of age, sex, type and associated anomalies and diseases.