Wakako Himeno

Quantitation of the Global Right Ventricular Function in Children with Normal Heart and Congenital Heart Disease: A Right Ventricular Myocardial Performance Index

Abstract. 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 congential heart disease.

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.

Increased angiogenic growth factor in cyanotic congenital heart disease.

To examine the relationship between the plasma levels of angiogenic growth factors and the severity of cyanosis, 80 patients with cyanotic heart disease (CHD) and 81 healthy controls were studied. Median age and mean arterial blood oxygen saturation respectively were 4.2 years and 81% in CHD subjects and 4.8 years and 98% in controls. Vascular endothelial growth factor (VEGF) and hepatocyte growth factor (HGF) were measured in plasma using enzyme-linked immunoassay. Plasma VEGF levels in controls depended negatively on age (p < 0.0001) until 3 months, when VEGF was no longer elevated. No such age dependence was found for HGF. Although VEGF levels did not differ between CHD and control subjects up to the age of 3 months, VEGF was significantly elevated in CHD patients older than 3 months compared to controls of similar age (149 ± 106 vs 65 ± 23 pg/ml, p < 0.0001). Moreover, the VEGF levels were negatively correlated with oxygen saturation (p = 0.03) and positively correlated with hemoglobin (p = 0.004) in CHD patients aged between 3 months and 10 years. Although the physiologic elevation of VEGF in the neonatal period decreases rapidly if oxygen saturation is normal, VEGF elevations persist if systemic hypoxia is present.

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.

Soluble forms of the selectin family in children with Kawasaki disease: prediction for coronary artery lesions

Aim: To investigate the relationship between the plasma levels of soluble forms of the selectin family and the incidence of coronary artery lesions (CALs) in patients with Kawasaki disease (KD). Methods: Thirty‐three patients with KD, including group A patients (n= 22) who had no CALs and group B patients (n= 11) who had CALs, as well as age‐matched febrile (n= 10) and afebrile controls (n= 11), were studied. Results: Peak plasma E‐selectin levels (172.0 ± 58.6 ng ml‐1) occurred during the acute phase of KD, while peak plasma P‐selectin levels (260.3 ± 43.2 ng ml‐1) occurred during the subacute phase of the illness (p± 0.05). Plasma L‐selectin levels (1757.3 ± 244.3 ng ml‐1) during the convalescent phase tended to be higher than in either the acute or the subacute phase (not significant). Before intravenous immunoglobulin treatment, the plasma levels of E‐ (225.1 ± 46.8 ng ml‐1) and P‐selectin (259.4 ± 76.2 ng ml‐1) of patients with CALs (n= 11) were significantly higher than those of patients (n= 22) with no CALs (E‐selectin, 131.6 ± 36.9 ng ml‐1; P‐selectin, 184.9 ± 84.6 ng ml‐1; p± 0.05). When a plasma E‐selectin value before immunoglobulin treatment of ± 184.7 ng ml‐1 was used as the cut‐off point, the sensitivity and specificity for the incidence of CALs were 81.8% and 90.9%, respectively. These findings demonstrate the relationship between plasma levels of selectins and disease severity of Kawasaki vasculitis.

Assessment of the Ability of Myocardial Contrast Echocardiography with Harmonic Power Doppler Imaging to Identify Perfusion Abnormalities in Patients with Kawasaki Disease at Rest and During Dipyridamole Stress

The aim of our study was to assess the ability of myocardial contrast echocardiography (MCE) with harmonic power Doppler imaging (HPDI) to identify perfusion abnormalities in patients with Kawasaki disease at rest and during pharmacological stress imaging with dipyridamole. Results were compared with those of 99mTc-tetrofosmin single-photon emission computed tomography (SPECT) imaging as the clinical reference standard. MCE with HPDI was performed on 20 patients with a history of Kawasaki disease. Images were obtained at baseline and during dipyridamole infusion (0.56 mg kg?1) in the apical two- and four-chamber views. Myocardial opacification suitable for the analysis was obtained in all patients. Nine patients with stenotic lesions had a reversible defect after dipyridamole infusion detected by both MCE with HPDI and SPECT, and 3 patients with a history of myocardial infarction had a partially or completely irreversible defect detected by both methods. Three patients with coronary aneurysm without stenotic lesion, 4 patients with regressed coronary aneurysm, and 2 patients with normal coronary artery in acute phase also had normal perfusion at rest and after pharmacological stress by both methods. A 96% concordance (? = 0.87) was obtained when comparing the respective segmental perfusion scores using the two methods at baseline, and an 86% concordance (? = 0.81) was obtained at postdipyridamole infusion. After combining baseline and postdipyridamole images, each segment was labeled as having normal perfusion, irreversible defects, or reversible defects. Using these classifications, concordance for the two methods was 92% (? = 0.87). MCE with HPDI is a safe and feasible method by which to detect asymptomatic ischemia due to severe stenotic lesion, and it may be an important addition to the modalities used to identify patients at risk for myocardial infarction as a complication of Kawasaki disease.

Prenatal diagnosis of sustained bradycardia with 1 : 1 atrioventricular conduction

The aims of this study were to elucidate the clinical course of fetal bradycardia with 1 : 1 atrioventricular conduction, and to discuss the optimal management of affected fetuses in the second and third trimesters of pregnancy.

Progression of congenital heart disease in the prenatal period

Abstract Background: Prenatal echocardiography has shown evidence of prenatal development of congenital heart disease. Prenatal cardiac anatomy, chamber size and function change during gestation, so that the appearance of cardiac structure in abnormal hearts may be different from that which is usually seen postnatally.

Enlarged bronchial arteries in a fetus with transposition of the great arteries and intact ventricular septum

Enlarged bronchial arteries are associated in some patients with transposition of the great arteries and intact ventricular septum. The etiologyof these enlarged bronchial arteries is not yet known. In this report, we describe a case of TGA/IVS in which enlarged bronchial arteries were demonstrated from the prenatal period. The arteriogram at one year after arterial switch repair demonstrated enlarged bronchial arteries. This prenatal information may be useful for deciding on a strategy for postnatal treatment and counseling the family members.

Transthoracic echocardiography-assisted valve perforation in pulmonary atresia.

Recent progress in interventional cardiology in the pediatric field contains various kinds of procedures, such as coil embolization of patent ductus arteriosus, 1,2 implantation of stents for vessel stenosis 3 etc. Infants with pulmonary atresia and intact ventricular septum (PA/IVS) complicated with severe cyanosis immediately after birth, and without surgery, will not survive beyond the neonatal period. 1 Infants with PA/IVS who have a relatively well-developed right ventricular chamber size and right ventricular outflow tract (RVOT) can be considered as good candidates for biventricular repair, including catheter intervention. 4–6 Recently, several types of catheter interventional procedures have been introduced for the purpose of perforating the atretic pulmonary valve and subsequently performing balloon dilatation. Recent studies have demonstrated high success rates and good clinical outcome, thus, these procedures can be considered as an alternative to surgery. 7,8 However, serious complications, including perforation of the RVOT, ventricular arrhythmia, and death directly caused by the interventional procedure, are still reported in some instances. 7,8 In this regard, perforation of the RVOT is the most frequently experienced complication at the time of the perforation of atretic pulmonary valve. 7