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 2.3 at start, 3.4 at study completion; P = 0.034), the rate of score increase rose with the number of telediagnoses (r = 0.72, P < 0.05), feedback from a specialist was very useful (4.4 and 4.9, respectively), and real-time image transmission was preferred over recorded images (score 3.7 vs 2.4, respectively; P = 0.042). The excellent educational effect of telemedicine is useful for staff members to improve their skills while nurturing their motivation, leading to the promotion of fetal cardiac screening in regional areas.

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 4.5, and in 96 patients (84% of all) telediagnosis was accurate (above 4), whereas in 18 patients it was inaccurate (level 2 or 3). In re-examination of 25 patients, telediagnosis was confirmed in patients in level 4 and 5, whereas heart disease was missed in patients in levels 2 or 3. The correct diagnosis matched the high confidence level of a specialist based on recognizable transmitted images.

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. The number of fetal telediagnosis for cardiac disease, and cases referred to a perinatal care center and emergent transportation of neonates with congenital heart disease from maternity hospitals, were calculated based on the hospital records. The percentage of patients found to have heart disease was compared between out-patient clinic and telediagnosis cases. Telediagnosis increased, allowing maternity hospital staff to obtain support easily from a specialist when making a diagnosis. Many severe cases were transferred to tertiary centers with the correct diagnosis; consequently, the number of emergent transportations of neonates with severe cardiac anomalies continued to below. Telediagnosis was also useful as an educational tool for maternity hospital staff, who improved their skills during conversations with a specialist. Unlike in the outpatient clinic, consultation by telediagnosis was requested even for cases of mild abnormalities, and the number of false-positives increased, while many cardiac anomalies were found in the early stage. Furthermore, telediagnosis was helpful for pregnant women requiring bed rest, and also had the advantage of allowing a doctor to be able to talk with parents. Establishing a fetal telediagnosis system is a useful strategy to improve neonatal care through a medical link between local maternity hospitals and a tertiary center.

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). In both cases the QT interval was over 0.55 s with episodes of torsades de pointes, and prominent ventricular trabeculations and deep intertrabecular recesses were detected by 2-dimensional echocardiography. Both cases had the KCNH2 mutation. To the best of our knowledge, this is the first report investigating INCVM with 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. The authors also discuss the optimal administration dosage and serum magnesium (SMg) concentration during MgSO4 therapy. METHODS: The authors studied seven consecutive LQTS children undergoing MgSO4 therapy for TdP. Of the seven children, five were congenital LQTS and two were acquired LQTS. A bolus injection of MgSO4 was given intravenously over 1-2 min followed by continuous infusion for the next 2-7 days. RESULTS: Of the seven patients, six responded completely to the initial bolus. The bolus dosage was 5.9 +/- 3.8 mg/kg (range, 2.3-12 mg/kg) in these six, and the other remaining one (neonate with congenital LQTS) required a total of 30 mg/kg until complete abolishment. The continuous infusion was given at rates of 0.3-1.0 mg/kg per h and patients did not show recurrence of TdP. The SMg concentration was 3.9 +/- 1.0 mg/dL (2.9-5.4 mg/dL) immediately after bolus injection. The mean corrected QT (QTc) interval before and after bolus injection did not show significant difference. CONCLUSION: Intravenous infusion of MgSO4 was effective for TdP in children with LQTS, and MgSO4 abolished TdP without shortening the QTc interval. The optimal bolus dosage, infusion rates and SMg concentration were 3-12 mg/kg, 0.5-1.0 mg/kg per h and 3-5 mg/dL, respectively.

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. A bolus injection of MgSO(4) was given intravenously over 1 to 2 minutes followed by continuous infusion for the next 2 to 7 days. RESULTS: Of the six patients, five responded completely to the initial bolus of 6.1 +/- 4.2 mg/kg (range, 2.3-12 mg/kg). One (a neonate with congenital LQTS) required a total of 30 mg/kg until complete TdP elimination. Continuous infusion was given at rates of 0.3 to 1.0 mg/kg/hr with no recurrence of TdP. SMg concentration was 3.9 +/- 1.0 mg/dL (2.9-5.4 mg/dL) immediately after bolus injection. CONCLUSION: Intravenous MgSO(4) infusion effectively treated TdP in children with LQTS. Optimal bolus dosage, infusion rates and SMg concentration were 3 to 12 mg/kg, 0.5 to 1.0 mg/kg/hr and 3 to 5 mg/dL, respectively.

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. Even in complex cases, fetal echocardiography was highly accurate; therefore, specific counseling can be given to parents.

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. The time taken for the Doppler signal to travel from the ascending to descending aorta was measured. AO peak flow was calculated from AO annulus cross-sectional area x peak aortic velocity (AoV); PWV = L/time taken for the Doppler signal to travel from the ascending to descending aorta; input impedance = (systolic - diastolic blood pressure)/(AO cross-sectional area x AoV); characteristic impedance = (PWV x rho)/Ao cross-sectional area (where rho = 1.06); and pressure strain elastic modulus = (systolic - diastolic blood pressure)/[(AO(S) - AO(D))/AO(D)]; beta index = ln (systolic/diastolic blood pressure)/[(AO(S) - AO(D))/AO(D)]. RESULTS: Results for PWV were ICTD = M > C (533, 496, 362 cm/s; P <.02); for input impedance were ICTD > M = C (245, 120, 116; P <.03); for characteristic impedance were ICTD < M = C (249, 107, 142; P <.05); for pressure strain elastic modulus were M > ICTD = C (262, 447, 187; P <.003); and for beta index were M > ICTD > C (2.91, 2.51, 2.13; P <.0006). CONCLUSIONS: This novel, relatively simple method of assessing the biophysical properties of the aorta shows abnormal function in patients with M and ICTD. This technique may be helpful in the long-term follow up of patients with arterial disease.

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. We should explore the cause of lobar emphysema thoroughly before lobectomy especially when it is extrinsic. The emphysema may regress by eliminating the extrinsic factor.

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. METHODS: Twenty-seven pediatric patients undergoing open-heart surgery with CPB were enrolled in this study. Controls were 23 pediatric patients undergoing palliative surgery without CPB. Serum Mg (SMg) concentrations and ionized Mg (iMg) concentrations were measured at four sample points: 24 h before the surgery, immediately after the surgery, 24 h after the surgery and 48 h after the surgery. RESULTS: Serum Mg and iMg concentrations were significantly decreased after open-heart surgery. Immediately after the surgery, the mean SMg concentration was 64.1% and the mean iMg concentration was 68.8% of the pre-surgical values. The concentration of iMg showed quicker recovery than that of SMg, and returned to normal range 48 h after surgery. However, SMg and iMg concentrations after palliative surgery did not show significant differences from the pre-surgical values. The incidence of dysrhythmia was almost the same between the two groups; dysrhythmia was observed in two patients in the open-heart surgery group and in three patients in the palliative surgery group. CONCLUSIONS: Mg concentrations showed significant decrease after CPB; however, they showed quick recovery and did not increase the incidence of dysrhythmia in pediatric patients.