Gengi Satomi

Has fetal echocardiography improved the prognosis of congenital heart disease? Comparison of patients with hypoplastic left heart syndrome with and without prenatal diagnosis

Abstract Background: As prenatal diagnosis of congenital heart disease has gained in popularity, the questions of whether prenatal diagnosis of congenital heart disease is beneficial for the patient and whether fetal echocardiography has improved the prognosis of congenital heart disease are arising.

Modified Starnes operation for neonatal Ebstein’s anomaly

We report the case of a severely symptomatic neonate with Ebsteins anomaly. A modified Starnes operation was performed, but insufficient drainage of venous blood returning through thebesian veins caused overdistention of the right ventricle and severe left ventricular dysfunction. Urgent reestablishment of right ventricular-right atrial communication successfully resolved these problems.

Analysis of ventricular septal motion by doppler tissue imaging in atrial septal defect and normal heart.

The aortic root and the upper part of the ventricular septum moves anteriorly in early systole, while the lower part moves posteriorly. The hinge of the counterpart motion of the ventricular septum is called pivot point. Using Doppler tissue imaging (DTI), we attempted to clarify the location of the pivot point of the ventricular septum in children with normal heart and with atrial septal defect (ASD), and to investigate the relation between the degree of the downward shift of the pivot point and that of volume overload of the right ventricle in patients with ASD. Study subjects consisted of 20 healthy children and 36 patients with ASD, aged from 1 to 15 years (mean 5.7+/-3.4) in the normal group and 6 months to 12 years (mean 4.4+/-3.2) in the ASD group, respectively. The pivot point was designated as a border of the color signal of DTI of the ventricular septum in early systole. Measurements were then obtained on cross-sectional echocardiography and DTI: septal length in the parasternal long-axis view, distance from aortic valve to pivot point in early systole, diastolic left ventricular internal dimension, and diastolic right ventricular internal dimension. In the normal group, the ratio of distance from aortic valve to pivot point/septal length was 0.13+/-0.049, whereas it was 0.26+/-0.168 in the group with ASD (p <0.001). In the ASD group, the distance from aortic valve to pivot point normalized by body surface area (mm/m2) correlated with the ratio of diastolic right/left ventricular internal dimension and with the ratio of pulmonary to systemic flow (Qp/Qs) (r = 0.63 and 0.50, respectively). The ratio of the distance from aortic valve to pivot point/septal length correlated with the ratio of diastolic right/left ventricular internal dimension and Qp/Qs (r = 0.56 and 0.44, respectively). By DTI, the pivot point was located at the upper 13+/-5% of the total length of the ventricular septum in normal children, and was located at 26+/-17% in patients with ASD (p < 0.001). The degree of this displacement in ASD correlated with that of volume overload of the right ventricle. The paradoxic motion of the ventricular septum shown in the ASD could be explained by this downward shift of the pivot point.

Quantification of Right and Left Ventricular Volumes in Children with Congenital Heart Disease by Multidetector-Row Computed Tomography

Multidetector-row computed tomography (MDCT) of the heart is a new diagnostic approach for the quantitative evaluation of the coronary artery in adults. However, in children, the quantitative analysis of each cardiac chamber has not been established. We attempt to clarify the feasibility and validation of ECG-gated MDCT as a quantitative diagnostic tool to assess the right and left ventricular volume in children. The study consisted of 16 patients who had definite right and left ventricle. After obtaining multislice images by MDCT, we measured the end diastolic volume of both the right and the left ventricle by direct calculation of the region of interest and calculation from the projected image of three-dimensional reconstruction by traditional formulas. The correlation between the two calculations for both ventricles is excellent (r = 0.99 for LV and 0.94 for RV, respectively). The correlations for ventricular volume between calculation of catheterization and calculation of MDCT is also good (r = 0.99 for LV 0.99 for RV, respectively). Volume measurement by ECG-gated MDCT of the right and left ventricles is well correlated with that by catheterization in children and can reduce the necessity for cardiac catheterization.

Absent aortic valve : Successful palliation in the neonate

A successful palliation of a neonate with absent aortic valve in hypoplastic left heart syndrome is reported.

Normal values for cardiac and great arterial dimensions in premature infants by cross-sectional echocardiography

The number of neonates who undergo intracardiac repair for congenital heart disease has increased in recent years. In considering appropriate surgical options in such circumstances, normal values for various cardiovascular dimensions are needed. We measured 19 parameters for the heart and great arteries (four valves, pulmonary tree, aortic arch, ascending and descending aorta, ventricular septum, left ventricular internal and posterior wall dimensions) using cross-sectional echocardiography in 55 premature and full-term infants (ranging in gestational age from 23 to 41 weeks, in body weight from 543 to 3966 g). The increase in each dimension correlated closely with body weight (r=0.62 to 0.87) and was found to follow a linear regression. The diameter of the isthmus was found to be smaller, and the size of mitral valve annulus larger, than previously published values.

Prenatal Diagnosis of Total Anomalous Pulmonary Venous Connection with Asplenia

The prenatal diagnosis of 2 fetuses with total anomalous pulmonary venous connection was made by color Doppler echocardiography at 31 and 38 weeks of gestation, respectively. Both had asplenic hearts and their diagnoses were confirmed by postnatal echocardiography, magnetic resonance imaging, and angiography. The antenatal diagnosis of total anomalous pulmonary venous connection in right isomerism could provide an optimal postnatal medical and surgical management. Moreover, monitoring of the velocity profile of the pulmonary venous flow is beneficial for decreasing the risk of sudden collapse by pulmonary venous obstruction throughout the perinatal period.

Guidelines for fetal echocardiography.

Congenital heart defects have been reported to occur in 1 out of every 100 births but, because this statistic does not include miscarriages or stillbirths, it can be inferred that the actual number of fetuses with heart defects is almost fivefold greater than that reported. Approximately one-third of such patients have serious heart defects, which is the major cause of infant mortality. These congenital defects, however, are the most difficult to diagnose prenatally. Screening for heart defects is performed in only a low percentage of cases. In addition, 90% of heart defects occur in low-risk infants with no clear indication of a congenital anomaly on prenatal examination.

Successful transluminal angioplasty of renal arterial stenosis using the transcarotid approach

We successfully performed percutaneous transluminal angioplasty to treat severe renovascular hypertension with left ventricular failure in a 5-month-old infant. Using the transcarotid approach, we dilated the stenotic left renal artery without any difficulties, using progressively larger balloons designed for dilation of coronary arteries.

Fetal diagnosis of common arterial trunk with interrupted aortic arch using color power Doppler angiography.

We successfully visualized the brachiocephalic arteries and aortic arch in a fetus seen at 19 weeks of gestation with a common arterial trunk and interrupted aortic arch by means of color power Doppler angiography, a new diagnostic development of color Doppler echocardiography. Power Doppler imaging is more sensitive to the state of low flow in fetal vessels, thus providing better visualization of fetal vascular structures from an early gestational stage.