Increased satisfaction of maternity hospital staff involved in fetal telediagnosis.

Questionnaires were sent to 14 maternity hospital staff members for qualitative assessment at the start of fetal telediagnosis and at the end of the study using a five-point Likert scale: 5, I strongly think so; 4, I think so; 3, I can't decide; 2, I don't think so; 1, I never think so. Ten questionnaires were returned to us (71%). The results showed that the staff reported a significant increase in confidence in performing fetal cardiac screening (score 2.

Accuracy of telediagnosis of fetal heart disease using ultrasound images transmitted via the internet.

We verified the feasibility of telediagnosis of fetal disease by (i) grading telediagnosis by a pediatric cardiologist into five confidence levels; and (ii) comparison of fetal telediagnosis with hands-on fetal diagnosis or postnatal diagnosis. In 114 patients suspected of having heart disease (real time, n = 15; recorded image transmission, n = 99), 79 patients were in level 5 (excellent), 17 in level 4 (good), eight in level 3 (fair), 10 in level 2 (poor), and no patients in level 1 (bad). The average was 4.

A medical link between local maternity hospitals and a tertiary center using telediagnosis with fetal cardiac ultrasound image transmission.

Information and communication technology has been widely applied to various fields, including clinical medicine. We report here a telediagnosis system using ultrasound image transmission. The effect of telediagnosis, using a medical link between local maternity hospitals and our children's medical center, was verified.

Absence of the coronary sinus with coronary venous drainage into the main pulmonary artery.

We describe the case of a 9-year-old girl demonstrating isolated absence of the coronary sinus with abnormal coronary venous drainage into the main pulmonary artery. Coronary angiography showed normal coronary arterial trees and contrast medium from both coronary arteries drained into the main pulmonary artery via an abnormal cardiac vein on the anterior wall of the right ventricle.

Isolated non-compaction of the ventricular myocardium associated with long QT syndrome: a report of 2 cases.

Isolated non-compaction of the ventricular myocardium (INCVM) is a relatively newly defined clinicopathologic entity. INCVM is clinically accompanied by depressed ventricular function, arrhythmias, and systemic embolization. We report two cases of INCVM with long QT syndrome (LQTS).

Successful uses of magnesium sulfate for torsades de pointes in children with long QT syndrome.

Background: Administration of magnesium sulfate (MgSO4) is an effective and safe treatment for torsades de pointes (TdP) associated with acquired long QT syndrome (LQTS) in adults. As for children, there are few reports focusing on it. The authors discuss the efficacy of MgSO4 for TdP in children with congenital and acquired LQTS.

Optimal administration dosage of magnesium sulfate for torsades de pointes in children with long QT syndrome.

Background: Intravenous administration of magnesium sulphate (MgSO(4)) is a very effective and safe treatment for torsades de pointes (TdP) associated with acquired long QT syndrome (LQTS) in adults. Discussed here is the efficacy of MgSO(4) for TdP in children with congenital and acquired LQTS.

Methods: The optimal MgSO(4) dosage and serum magnesium (SMg) was determined in six consecutive children with TdP; four had congenital LQTS and two had acquired LQTS.

Diagnosis and outcome of dextrocardia diagnosed in the fetus.

A retrospective review of 5,539 fetal echocardiograms over a 22-year period revealed 85 cases of dextrocardia. In primary dextrocardia (46 cases), the incidence of situs solitus, inversus, and ambiguous, was similar and associated with a high incidence of complex cardiac malformations in situs solitus and situs ambiguous. Secondary dextrocardia (39 cases) was due to intrathoracic displacement and, when caused by diaphragmatic hernia, was associated with cardiac malformations in 31% of cases.

A novel Doppler echocardiographic method of measuring the biophysical properties of the aorta in pediatric patients.

Background: This study was performed to: (1) assess the feasibility of measuring pulse wave velocity (PWV) from aortic (AO) Doppler flow and deriving AO input and characteristic impedances and the arterial pressure strain elastic modulus; and (2) compare these indices in pediatric patients with arterial disease to control (C) patients.

Methods: There were 14 healthy children in the C group, 9 with inflammatory connective tissue diseases (ICTD), and 6 with Marfan syndrome (M). Using standard echocardiographic equipment, the AO annulus, AO root diameter in systole (AO(S)) and diastole (AO(D)), and AO length (L) were measured.

Lobar emphysema due to ductus arteriosus compressing right upper bronchus in an infant with congenital heart disease.

A 1-month-old boy with tetralogy of Fallot, pulmonary atresia, right aortic arch, and right ductus arteriosus, exhibited progressive right upper lobar emphysema since his birth. The emphysema was caused by the right ductus arteriosus compressing the right upper bronchus. After division of the ductus arteriosus the emphysema completely regressed.

Influence of heart surgery on magnesium concentrations in pediatric patients.

Background: There have been only a few investigations into the effects of cardiopulmonary bypass (CPB) on the magnesium (Mg) concentrations in pediatric patients. The purpose of the present study was to compare the postoperative Mg concentrations and their recovery time to pre-surgical values in pediatric patients undergoing CPB for surgical repair of congenital heart disease. The incidence of dysrhythmia was also determined.

Perioperative course in two cases of tetralogy of fallot with portal venous atresia.

We report here two rare surgical cases of tetralogy of Fallot with portal venous atresia. Both patients, previously diagnosed with tetralogy of Fallot, developed portal hypertension and showed hematemesis and/or melena. One patient needed endoscopic sclerotherapy.

Studies of magnesium in congenital long QT syndrome.

We studied the role of magnesium (Mg) in congenital long QT syndrome (LQTS). Twenty-two congenital LQTS patients and 30 control subjects were included in this study. We measured serum Mg (SMg) level and Mg retention (MgR) level, and evaluated the role of Mg (a high MgR level reflects Mg deficiency in the body).

Characteristics of improved NOVA magnesium ion-selective electrode: changes of ionized magnesium values and reference interval in healthy children.

Following the report of interference between the thiocyanate ion (SCN-) and NOVA's previous ion-selective electrode (ISE) for ionized magnesium (iMg2+), NOVA has developed a new ISE which eliminates the effect of SCN-. Two hundred and sixty healthy children were divided into two groups; those who had presented when using NOVA's previous ISE (group A; n = 160) and those using NOVA's new ISE (group B; n = 100). The mean iMg2+ value and the mean iMg2+ percent fraction (iMg2+/serum Mg) were significantly higher in group B than in group A (0.

Malignant fibrosarcoma with features of myxoma.

Left atrial tumor, diagnosed as myxoma at first investigation, was later demonstrated to be a malignant fibrosarcoma with myxoid change. Myxoma should be diagnosed and managed carefully because of its wide range of histopathologic features.

Surgical ligation of anomalous hepatic vein in a case of heterotaxy syndrome with massive intrahepatic shunting after modified fontan operation.

In case of progressive cyanosis after modified Fontan procedure, intrahepatic shunting should be considered. Ligation of the hepatic vein is safe and effective in managing this condition, and it is important to ensure that the pressure difference between superior vena cava and the portal vein system remains minimal after clamping of the hepatic vein before ligation is completed to minimize the possibility of varix due to portal vein hypertension.

Hypoplastic left heart syndrome: duration of survival without surgical intervention.

Background: Mortality rate for heart transplantation for patients with hypoplastic left heart syndrome (HLHS) has improved, but there is a considerable wait until a suitable donor is available. Thus it is important to examine the duration of survival and risk factors for early death in patients with HLHS who did not undergo surgical intervention.

Methods And Results: Twenty-six consecutive patients were studied retrospectively.

Rapid enlargement of cardiac rhabdomyoma during corticotropin therapy for infantile spasms.

Objective: To investigate the influence of corticotropin therapy on cardiac rhabdomyoma.

Design: Analysis of data from echocardiography performed on in-patients.

Patients: Six patients with rhabdomyoma who were admitted to the authors' medical centre with either convulsion (five cases) or prematurity (one case) between 1985 and 1995.

[Superior vena cava-right atrial appendage direct anastomosis for repair of partial anomalous pulmonary venous connection to superior vena cava].

Successful repair was performed for a 7-year-old male with a diagnosis of partial anomalous pulmonary venous connection (PAPVC) to superior vena cava (SVC) and superior sinus venosus atrial septal defect (ASD). The SVC was divided above the orifice of the anomalous pulmonary vein and the cephalad end of the SVC was anastomosed directly to the right atrial appendage. A patch was used to divert pulmonary venous flow from the orifice of the SVC through superior sinus venosus ASD into the left atrium.