Quantitative assessment of severity of ventricular septal defect by three-dimensional reconstruction of color Doppler-imaged vena contracta and flow convergence region.

BACKGROUND: The aim of the present study was to investigate the feasibility and potential value of the computer-controlled, 3D, echocardiographic reconstruction of the color Doppler-imaged vena contracta (CDVC) and the flow convergence (FC) region as a means of accurately and quantitatively estimating the severity of a ventricular septal defect (VSD). METHODS AND RESULTS: We performed a 3D reconstruction of the CDVC and the FC region in 19 patients with an isolated VSD using an ultrasound system interfaced with a Tomtec computer. The variable asymmetric geometry of the CDVC and the FC region could be 3D-visualized in all patients. The 3D-measured areas of CDVC correlated well with volumetric measurements of the severity of VSD (r=0.97, P:<0.001). Regression analysis between the shunt flow rate (calculated from the product of the area of CDVC and the continuous Doppler-derived velocity time integral) and the corresponding reference results (calculated by cardiac catheterization) demonstrated a close correlation (r=0.95, P:<0.001). There was also a good correlation between shunt flow rates calculated using the conventional 2D, 1-axis measurement of the FC isovelocity surface area with the hemispheric assumption (r=0.95, P:<0.001); shunt flow rates calculated using 3D, 3-axis measurements of the FC region (r=0.97, P:<0.01); and reference results by cardiac catheterization. However, the 2D method substantially underestimated the actual shunt flow rate. CONCLUSIONS: The 3D reconstruction of the CDVC and the FC region may aid in quantifying the severity of VSD.

Sequential evaluation of left ventricular myocardial performance in children after anthracycline therapy.

This study prospectively assessed subclinical cardiotoxicity in patients undergoing chemotherapy by using the Tei index combining systolic and diastolic time intervals. A significant difference in the Tei index was observed between patients who received a low dose and those who received a moderate to high dose of anthracycline antibiotic drugs. The Tei index is a sensitive, accurate, and easy approach for detecting subclinical anthracycline cardiotoxicity.

Quantitation of the global right ventricular function in children with normal heart and congenital heart disease: a right ventricular myocardial performance index.

Although the assessment of right ventricular (RV) function is important in the clinical management of children with congenital heart disease, available imaging techniques have been limited because of the complex geometry of the right ventricle. A new Doppler index combining systolic and diastolic time intervals (the Tei index) has been reported to be useful for the assessment of global RV function in adults. However, normal values in children, age-related changes, and the clinical utility of the Tei index with regard to congenital heart disease have not been demonstrated. The purpose of this study was to prospectively assess RV function in children with normal heart and congenital heart disease using the Tei index. The subjects included 150 healthy children and 43 patients with congenital heart disease (35 patients with atrial septal defects and 8 patients who had had a Senning operation). The index was defined as the sum of isovolumetric contraction time and isovolumetric relaxation time divided by ejection time and was measured from conventional RV outflow and inflow Doppler velocity profiles. The Tei index was not affected by age in healthy children (0.24 +/- 0.04). There was a significant difference in index rating between patients who had had a Senning operation (0.58 +/- 0.09) and healthy children (p < 0.01), but there was no significant difference between children with atrial septal defect (0.25 +/- 0.13) and healthy children. The Tei index is a feasible approach to use when assessing global RV function in children with congenital heart disease.

Three-dimensional helical computed tomographic angiography in neonates and infants with complex congenital heart disease.

BACKGROUND: For the clinical management of patients with complex congenital heart disease (CHD), accurate evaluation of their morphologic conditions is critical. Three-dimensional (3D) helical computed tomography (CT) angiography has been used to assess the vascular system in adult patients; the indication for complex CHD, especially in the neonatal period, has not yet been defined. Therefore the purposes of our study were to determine the quality and limitations of current 3D helical CT angiography for neonates and infants with complex CHD and to assess the clinical utility of this technique. METHODS AND RESULTS: 3D helical CT angiography was performed in 17 patients with various types of complex CHD. Their median age was 41 days (range 3 days to 9 months), and mean body weight was 3.6 kg (range 2.2 to 8.5 kg). All 3D images were produced with the 3D reconstruction algorithm of shaded-surface display. Oral sedation was required in only 4 infants during the procedure. 3D helical CT angiography clearly demonstrated the shape and spatial relation of great arteries, proximal branch pulmonary arteries, anomalous pulmonary venous connections, the patent ductus arteriosus, and a shunt. The 3D information of extracardiac morphologic characteristics and 3D anatomic relation of each extracardiac structure were easily recognized by this imaging process. However, intracardiac structure could not be visualized because of blurred and/or unclear edges of the ventricular wall caused by respiratory movement. CONCLUSIONS: 3D helical CT angiography represents an important additional diagnostic tool and may become an alternative method to angiography or other noninvasive techniques used in the evaluation of extracardiac anomalies in neonates and infants with complex CHD.

Assessment of global left ventricular function in normal children and in children with dilated cardiomyopathy.

A Doppler index combining systolic and diastolic time intervals (Tei index) has been reported to be useful for assessing global left ventricular (LV) function and predicting clinical outcome in adult patients with LV dysfunction. However, normal values in children and age-related changes in the index have not yet been clarified. The aim of this study was to prospectively determine normal values of the Tei index and the effect of aging on the index in children and to assess the global cardiac function in patients with dilated cardiomyopathy with this index. The subjects included 161 consecutive normal children aged 30 days to 18 years and 5 patients with dilated cardiomyopathy. The Tei index was defined as the sum of the isovolumetric contraction time and the isovolumetric relaxation time divided by the ejection time and was measured from conventional LV outflow and inflow Doppler velocity profiles. The Tei index correlated significantly with the logarithm of age (r = 0.51, P <. 001). The index decreased with aging until 3 years and then did not change after age 3 years. The Tei index in children under age 3 years (0.40 +/- 0.09, n = 80) was significantly higher than that in children ranging in age from 3 to 18 years old (0.33 +/- 0.02, n = 81). The index in patients with dilated cardiomyopathy (0.78 +/- 0. 28) was markedly increased compared with that in normal subjects. Age-related changes in the Tei index may reflect maturational or developmental alterations in the LV properties in infants. The data in this study give basic information for further quantitative assessment of global cardiac function in children with congenital or acquired heart disease.

Selective high dose gamma-globulin treatment in Kawasaki disease: assessment of clinical aspects and cost effectiveness.

BACKGROUND: High-dose intravenous gamma-globulin (IVGG) plus aspirin (ASA) treatment is effective in preventing coronary artery complications in acute Kawasaki disease (KD). However, gamma-globulin is very expensive, especially in Japan. Furthermore the indication for IVGG treatment and the optimal dose of gamma-globulin remain controversial. OBJECTIVES: To examine these two issues, we used Harada's scoring system to investigate whether a single 2 g/kg dose therapy has any advantage over the 5 day 400 mg/kg per day therapy. METHODS: We studied 203 patients with KD who had no coronary artery complications on admission. Of these, 145 patients scored 4 or more on Harada score within the first 9 days of illness and were treated with IVGG treatment. Using a random number table, 72 patients were selected to receive a single 2 g/kg dose (2 g group), while the remaining 73 patients were treated with 400 mg/kg per day for 5 consecutive days (400 mg group). Those who had a Harada score of three or less received no IVGG (non-IVGG group) treatment (58 patients). RESULTS: The incidence rate of coronary artery complications in the 2 g group was significantly lower than in the 400 mg group. The duration of high fever, positive duration of C-reactive protein and the number of hospital days in the 2 g group were each significantly shorter than in the 400 mg group. The total medical expense in the 2 g group was significantly lower than in the 400 mg group. There were no coronary artery complications in the non-IVGG group. CONCLUSIONS: It was found to be clinically more effective and more cost effective to select a patient by Harada's scoring system and, where a score of four or more was obtained, to administer a single 2 g/kg intravenous dose of gamma-globulin for acute KD.

Serial evaluation for myocardial performance in fetuses and neonates using a new Doppler index.

BACKGROUND: Fetal echocardiography has been used for non-invasive evaluation of human fetal cardiac anatomy, function and hemodynamics. The Tei index, a new Doppler index known to be independent of both ventricular geometry and heart rate, has recently been applied to the evaluation of myocardial performance. METHODS: In the present study, the Tei index was prospectively and longitudinally determined in 50 normal fetuses, 35 fetuses with intrauterine growth retardation (IUGR), 30 fetuses of diabetic mothers (DM) and 20 normal infants. The Tei index of both left and right ventricles was calculated from a Doppler ventricular inflow and outflow trace using the following formula: Tei index = (ICT + IRT)/ET, where ICT is isovolumetric contraction time; IRT, isovolumetric relaxation time; and ET, ejection time). RESULTS: The Tei index of the left ventricle decreased linearly with advancing gestational age during 18-33 weeks and decreased acceleratively with increasing gestational age after 34 weeks. The index of the right ventricle decreased slightly and linearly with advancing gestational age during 18-41 weeks. In neonates, the Tci index of the left and right ventricle increased immediately and transitorily after birth and decreased and stabilized after 24 h of life. From 18 to 26 weeks of gestation, the Tei indices in fetuses with IUGR and of DM were not significantly different from controls. However, from 27 to 40 weeks of gestation, the Tei indices in both fetuses with IUGR and of DM were significantly greater than controls. CONCLUSIONS: This gradual decrease in the Tei index during gestation may represent the maturational or developmental alternation of myocardial performance in utero. Fetuses with IUGR and of DM may have abnormal myocardial performance in later gestation.

Coronary endothelial dysfunction after Kawasaki disease: evaluation by intracoronary injection of acetylcholine.

OBJECTIVES: This study sought to assess the endothelial function of long-term coronary artery lesions in patients with Kawasaki disease (KD). BACKGROUND: The vascular function of the coronary arteries in children with long-term KD remains uncertain. We report our findings of the vascular response of the coronary arteries to intracoronary injection of acetylcholine (ACh) in patients with KD. METHODS: A total of 35 patients (25 patients with KD and 10 control subjects) were examined using coronary angiography. Individual arteries were divided into four groups according to the type of the coronary artery lesion: group 1 consisted of 25 sites with regressed aneurysms. These aneurysms had developed in the acute stage but had subsequently regressed and demonstrated normal findings on the follow-up coronary angiogram. Group 2 consisted of 24 sites with persistent aneurysms. Group 3 involved 60 angiographically normal sites in the same patients as those in group 1 or 2. Group 4 consisted of 30 sites in control subjects who had congenital heart disease with normal coronary arteries. During coronary angiography we infused 15 microg of ACh chloride into the coronary artery. The lumen diameters were measured using a cine videodensitometric analyzer to study the distensibility of the coronary artery wall. RESULTS: The mean (+/-SD) change in diameter was an increase of 11.71+/-12.34% in group 3 (coronary arteries without lesions in patients with KD) and 12.21+/-9.71% in the control group, demonstrating marked vasodilation in both groups. In contrast, the changes in the regressed aneurysms of group 1 and in the persistent aneurysms of group 2 were -2.65+/-12.12% and -0.08+/-6.51%, respectively, demonstrating no change or mild vasoconstriction. The change in groups 1 and 2 was significantly less than that in group 3 or in the control group. Group 3 showed no significant difference from the control group. CONCLUSIONS: These findings suggest that long-term coronary artery lesions, even after aneurysm regression, may have impaired endothelial function. A long-term follow-up study for those patients is essential.

Coil occlusion of patent ductus arteriosus with detachable coils.

Twenty-five patients (mean age 7.0 +/- 4.8 years) underwent transcatheter coil occlusion of patent ductus arteriosus with detachable coils. The minimum diameter of the ductus arteriosus ranged from 1.0 to 4.2 mm (mean 2.6 +/- 0.9 mm). A single-coil technique was used in 17 patients, double- (six patients) or triple-coil (two patients) techniques were used in eight patients. The coil was not detached until sufficient shape and position of implanted coils were confirmed. All patients had successful implantation of coils regardless of the morphologic characteristics of the ductus. Immediately after the occlusion, heart murmurs were abolished in all patients. Color-flow mapping showed complete closure in 16 (64%) patients immediately after and 20 (80%) patients 1 month after the procedure. No significant complications occurred. The advantages of this detachable coil system may reduce coil migration and allow safer and more reliable execution of this procedure.

Long-term consequences of Kawasaki disease. A 10- to 21-year follow-up study of 594 patients.

BACKGROUND: The long-term consequences of the cardiovascular sequelae in Kawasaki disease remain uncertain. METHODS AND RESULTS: We identified 594 consecutive children with acute Kawasaki disease between 1973 and 1983, and this cohort was followed up for 10 to 21 years (mean, 13.6 years). In all patients, we evaluated coronary lesions by coronary angiography just after the acute stage. One hundred and forty-six patients (24.6%) were diagnosed as having coronary aneurysms. A second angiogram was performed 1 to 2 years later in all 146 patients who previously had coronary aneurysms, which demonstrated that 72 (49.3%) of these 146 had regression in the coronary aneurysm. A third angiogram was performed for 62 patients, a fourth for 29, and a fifth for 17. By 10 to 21 years after the onset of the illness, stenosis in the coronary aneurysm had developed in 28 patients. Myocardial infarction occurred in 11 patients, 5 of whom died. In the 26 patients with giant coronary aneurysms, stenotic lesions developed in 12, and no regression occurred. The 448 patients with normal findings at the first angiogram subsequently never developed any abnormal cardiac findings. Systemic artery aneurysms developed in 13 patients (2.2%), and valvular heart disease appeared in 7 (1.2%). CONCLUSIONS: The incidence of coronary aneurysm in acute Kawasaki disease was 25%, 55% of which showed regression. During follow-up, ischemic heart disease developed in 4.7% and myocardial infarction in 1.9%. Death occurred in 0.8%.