Insulin dependent diabetes mellitus accompanied by nephrocalcinosis and renal failure.

Renal failure was found in a five-year-old patient who had been treated with insulin since he was diagnosed as having insulin dependent diabetes mellitus (IDDM) at 3 years of age. Laboratory data showed that his renal failure was caused by a renal tubular dysfunction. The autopsy findings of his pancreas were compatible with those of IDDM. The kidneys were atrophied with an innumerable number of crystals in the proximal tubuli. Staining by Kossa indicated that the crystals contained calcium salt. The calcium content of his kidneys was significantly higher than that of control. The nephrocalcinosis seems to be caused by hypercalciuria associated with IDDM.

[Aortic input impedance in ventricular septal defect].

Aortic input impedance and hydraulic power were measured in patients with ventricular septal defect (VSD). Aortic input impedance consists of the characteristic impedance (index of aortic distensibility) and the terminal impedance (index of peripheral resistance) which represent the left ventricular hydraulic load. The external left ventricular hydraulic power consists of steady power which transmits blood to the peripheral vasculature and pulsatile power which is converted into heat energy. The ratio of pulsatile to total power indicates the efficiency of the arterial system. The present study was carried out on 6 patients with VSD whose Qp/Qs was greater than 2.0 (VSD group) and 10 age-matched controls who had a history of Kawasaki disease without coronary complications at the time of cardiac catheterization (control group). Descending aortic pressure and flow velocity were measured simultaneously at the level of the 10th thoracic vertebra using a multisensor-catheter. Impedance data were calculated from Fourier analysis of these findings. Characteristic impedance did not differ between the 2 groups. Terminal impedance was higher in the VSD group with the increasing peripheral resistance and vascular afterload. The pulsatile component of external hydraulic power was similar in both groups, however, the steady component was lower in the VSD group indicating a higher ratio of pulsatile to total power in the VSD group. In conclusion, vascular afterload was increased and the efficiency of the arterial system was reduced in patients with VSD having large shunts.

Cerebral and abdominal arterial hemodynamics in preterm infants with patent ductus arteriosus.

Using Doppler echocardiography we evaluated the effect of ductal shunt flow on the cerebral and abdominal arterial blood flow in 25 preterm infants. Eligible for inclusion in this study were healthy preterm newborn infants. They were divided into two groups based on their gestational age: group A, 33-36 weeks (15 infants) and group B, 28-32 weeks (10 infants). Two-dimensional Doppler echocardiograms were obtained in each infant during the first 8 hours of life and repeated every 6-12 hours until no ductal shunt flow could be detected. Flow in the ductus arteriosus, the basilar artery and the coeliac artery were examined. Closure of the ductus arteriosus occurred significantly later (p less than 0.05) in group B than in group A. Pulsatility indices of flow in the basilar and coeliac arteries were high when the ductus was patent, decreasing to a fixed level with closure. This study suggests that a shunt of the patent ductus arteriosus (PDA) adversely influences the cerebral and abdominal blood flow in preterm infants.

Criss-cross heart evaluated by colour Doppler echocardiography and magnetic resonance imaging.

Two-dimensional colour Doppler echocardiography was performed on a 1-month-old male infant with criss-cross heart, double outlet right ventricle, ventricular septum defect and pulmonary stenosis. Complex structural abnormalities were suspected after two-dimensional echocardiography (2-D echo) and confirmed by colour Doppler and magnetic resonance imaging (MRI). We stress that the blood streams in the ventricular inflow tracts revealed by colour Doppler and the spatial relationships of the cardiac segments disclosed by MRI are essential to make an accurate non-invasive diagnosis of this complex malformation.

Solitary interruption of the aortic arch with stenotic origin of the left subclavian artery in 45, XO/46, XY/47, XYY mosaicism.

A four-year-old boy whose karyotype was 45, XO/46, XY/47, XYY mosaicism was diagnosed as having interruption of the aortic arch without ventricular septal defect or patent ductus arteriosus, complicated by stenotic origin of the left subclavian artery, which resembled coarctation of the aorta hemodynamically. Solitary interruption of the aortic arch is a very rare anomaly.

[A case of isolated anomalous return of bilateral superior pulmonary veins].

A 14-year-old girl was admitted for evaluation of heart murmur. Intravenous digital subtraction angiography (DSA) showed the right superior pulmonary vein drained into the superior vena cava and the left superior pulmonary vein drained into the innominate vein. At the operation, an anomalous bilateral superior pulmonary venous return with an intact atrial septum was confirmed. An atrial septal defect was created and the right superior pulmonary vein was baffled into the left atrium with a pericardial patch. The left superior pulmonary vein was divided and anastomosed to the left inferior pulmonary vein with a expanded polytetra-fluoro-ethylene graft. Post-operative DSA showed satisfactory long-term result of the operation.

[Bidirectional ductal shunts in the early neonatal period: evaluation by Doppler color flow imaging].

Serial Doppler echocardiographic examinations were performed in 10 normal neonates (0.3-4.0 hrs after birth). The flow patterns through the ductus arteriosus were evaluated using Doppler color flow imaging, pulsed Doppler echocardiography and continuous-wave Doppler echocardiography. At the initial examination, flow through the ductus arteriosus was clearly visualized in all the neonates using Doppler color flow imaging. The ductal flow patterns were categorized as follows: 1. Systolic blue color (right-to-left shunt flow) and diastolic red color (left-to-right shunt flow) in four neonates (group 1). 2. Systolic blue color and diastolic mosaic colors in four neonates (group 2). 3. Continuous mosaic colors in two neonates (group 3). Using pulsed Doppler echocardiography, the systolic right-to-left ductal shunt flow in the groups 1 and 2 was triangular in shape beginning in early systole. The diastolic left-to-right shunt flow was box-like in shape beginning late in systole and lasting long in diastole in the group 1. In the group 2, the diastolic flow showed a wide spectrum (turbulent flow). In the group 3, the flow through the ductus arteriosus had a continuous wide spectrum (turbulent flow). Mosaic or turbulent ductal flow of a left-to-right ductal shunt had high velocities by continuous-wave Doppler echocardiography. Serial examinations revealed that the ductal flow pattern observed in the group 1 changed to the flow pattern observed in the group 2, and then to that of the group 3 with increasing diastolic ductal flow velocities. The estimated aorto-pulmonary pressure gradient according to the simplified Bernoulli equation (delta p = 4V2) using a maximum diastolic left-to-right ductal shunt velocity increased within 12 hrs after birth. It was concluded that bidirectional ductal shunts may be observed in most normal neonates (8/10). With increasing diastolic velocities the bidirectional ductal flows changed to the pattern of a continuous left-to-right shunt. The bidirectional ductal shunt is considered due to physiologic pulmonary hypertension of the newborn and due to less conduction time from the pulmonary valve to the pulmonary end of the ductus than from the aortic valve to the aortic end of the ductus. Analysis of the flow through the ductus provides informations about the neonatal circulatory adaptation, especially in the early neonatal period.

[Aortic flow patterns in normal neonates with patent ductus arteriosus: evaluation by Doppler color flow imaging].

Serial Doppler echocardiography was performed in 12 normal neonates (0.5-4.0 hrs after birth) to evaluate flow patterns through the ductus arteriosus, and in the aorta and brachiocephalic artery. At the initial examination, flow through the ductus arteriosus was bidirectional in eight of the 12 neonates and continuously left-to-right in the remaining four. The bidirectional ductal shunts became continuous left-to-right flows within 11-21 hrs after birth in seven of the eight neonates and resolved by 29-47 hrs after birth. In the remaining four neonates, the continuous left-to-right shunts disappeared 14-36 hrs after birth. Systolic ejection flow patterns in the aorta and brachiocephalic artery had a triangular shape with the peak velocity in early systole, followed by a minimal flow reversal in all sites examined. Diastolic flow patterns in each arterial site were as follows: 1. In the ascending aorta, there was slow and sustained diastolic forward flow, which did not change with increasing age. 2. In the brachiocephalic artery, there was a pan-diastolic flow reversal in the neonates with bidirectional ductal flow (7/8). This pattern changed to slow pan-diastolic forward flow when the ductal changed to continuous left-to-right flow or when the ductal closure was confirmed. Most (3/4) of the remaining four neonates with continuous left-to-right ductal flow exhibited pan-diastolic forward flow. Another showed a pan-diastolic flow reversal 2 hrs after birth, which changed to pan-diastolic forward flow in the second examination 6 hrs after birth. 3. In the distal aortic arch, there was a pan-diastolic forward flow in all the neonates, and the velocity decreased when a closure of the ductus was confirmed. 4. In the descending aorta, there was a pan-diastolic flow reversal in neonates with bidirectional ductal flow (7/8). This reversal changed to pan-diastolic forward flow, when the ductal flow changed to continuous left-to-right flow or when the ductal closure was confirmed. In the remaining four neonates with continuous left-to-right ductal flow, two showed a pan-diastolic flow reversal at the initial examinations 2 to 3 hrs after birth. This became a pan-diastolic forward flow at the second examinations 6 and 12 hrs after birth. In the other two, there was a pan-diastolic forward flow which did not change. This pan-diastolic flow reversal observed in the brachiocephalic artery and descending aorta was closely related to the bidirectional ductal flow.(ABSTRACT TRUNCATED AT 400 WORDS)

[Left and right ventricular cardiac outputs in early neonatal periods examined using Doppler echocardiography].

To evaluate early neonatal circulatory adaptation, left and right ventricular cardiac outputs were examined in 10 normal neonates using Doppler echocardiography. Serial examinations were performed until ductal closure was confirmed. Two-dimensional echocardiography and Doppler echocardiography were used to evaluate structures of the heart and great vessels and flow within them. Then, the diameters of the ascending aorta (dAo) and pulmonary artery (dPA) were measured using M-mode echocardiography. The flow velocity patterns of the ascending aorta and pulmonary artery were recorded, measuring mean aortic (VAo) and pulmonary artery velocities (VPA). Left (LVCO) and right ventricular cardiac outputs (RVCO) were calculated as follows: LVCO = (dAo)2/4 x pi x VAo x 60, RVCO = (dPA)2/4 x pi x VPA x 60. 1. Patency of the ductus arteriosus was confirmed by the shunt through it in all neonates initially examined. The initial velocity pattern of the ductal shunts was bidirectional (9/10) or continuous left-to-right (1/10). The flow velocity pattern changed to continuous left-to-right in most neonates, and spontaneous closure of the ductus was confirmed at the age of 13 to 64.5 (mean 31.4) hrs. 2. Left-to-right shunt through the stretched foramen ovale was noted in six neonates temporarily. 3. As for the arterial diameter, dAo did not change, but dPA decreased at the time of spontaneous closure of the ductus. 4. Concerning mean aortic velocity, VAo increased when a continuous ductal left-to-right shunt was initially suspected, while VPA increased when spontaneous closure of the ductus was confirmed. 5. For cardiac output, LVCO (ml/min) changed from 618.4----718.3----562.7, while RVCO showed no change (576.1----546.5----557.8) according to a ductal flow change from bidirectional to continuous left-to-right and finally to no shunt. The LVCO/RVCO ratio increased with increasing age and (in 7/9) the maximal rate was noted when the continuous left-to-right ductal shunt was confirmed. The increased LVCO contributed to the increased LVCO/RVCO ratio. Using this method, serial evaluations of two ventricular cardiac outputs could be made.

Cardiac tumour in a neonate with tuberous sclerosis: echocardiographic demonstration and magnetic resonance imaging.

In a neonate with tuberous sclerosis, cardiac tumours were diagnosed by two-dimensional echocardiography and evaluated by electrocardiogram-gated magnetic resonance imaging (MRI). The tumour size, shape and mobility in the ventricular cavities were more precisely determined by two-dimensional echocardiography than electrocardiogram-gated MRI, while the extent of tumour mass at the apex was more clearly delineated by MRI. As two-dimensional echocardiography provides real-time imaging of cardiac anatomy without sedation, it is useful for initial evaluation of cardiac masses in neonates with genetic predisposition to tuberous sclerosis.

[Left-to-right shunt via the foramen ovale in the early neonatal period: evaluation by Doppler color flow mapping].

Serial two-dimensional Doppler echocardiography was performed in 22 normal neonates (2-9 hours after birth). A left-to-right shunt through the foramen ovale and a shunt through the ductus arteriosus were evaluated. Interatrial shunts were observed as transseptal jets in 16 of 22 neonates on initial examination and resolved nine to 26 hours after birth in 13 neonates. In the remaining three neonates the shunts were observed more than six days and resolved by two months of age. Shunts through the ductus arteriosus were observed in all neonates examined as diastolic or continuous flows toward the transducer in the main pulmonary artery at the initial examination. The ductal flow resolved simultaneously with (3/13) or after (10/13) the disappearance of interatrial flow. With two-dimensional echocardiography, the enlarged left atrium and rightward deviation of the interatrial septum were observed in the neonates with interatrial shunts. This was confirmed by the immobile septum primum and the increased ratio of the left atrial to aortic diameters (LA/Ao ratio) on the M-mode echo. The interatrial septum became mobile and the LA/Ao ratio decreased after disappearance of the interatrial shunts. It was speculated that in neonates with large ductal shunts, large pulmonary venous flow enters into the less compliant left ventricle and raises the left ventricular end-diastolic pressure. Then, increased left atrial pressure distends the interatrial septum and causes left-to-right interatrial shunts via the stretched foramen ovale. This shunt is considered one of the physiological phenomena occurring in the early neonatal period.

Right and left ventricular ejection patterns in D-transposition of the great arteries assessed by pulsed Doppler echocardiography.

Pulsed Doppler echocardiography (PDE) was performed to evaluate the flow velocity patterns of the right (RVOT) and left ventricular outflow tracts (LVOT) in 10 patients with transposition of the great arteries (D-TGA). Twenty normal children served as controls. Systolic time intervals (STIs) and acceleration times (AcT) of the right and left ventricles were measured using PDE. The following results were obtained. In normal controls, the right ventricular pre-ejection period (RPEP) was shorter than the left ventricular pre-ejection period (LPEP), and the right ventricular ejection time (RVET) was longer than the left ventricular ejection time (LVET). The mean RPEP/RVET was 0.33 (range 0.25-0.42) and the mean LPEP/LVET was 0.38 (range 0.28-0.55). In normal controls, the flow velocity pattern of the RVOT had a dome-like contour with a peak velocity in mid-systole, and the mean AcT/RVET ratio was 0.50 (range 0.41-0.62); that of the LVOT had a triangular shape with a mean AcT/LVET ratio of 0.31 (range 0.24-0.38). In patients with D-TGA, the RPEP was longer than the LPEP and the RVET was shorter than the LVET. The mean RPEP/RVET was 0.53 (range 0.39-0.76) in patients with intact ventricular septum and 0.52 (range 0.54-0.60) in patients with ventricular septal defect (VSD). The RPEP/RVET was significantly greater in patients with D-TGA than in normal controls. The mean LPEP/LVET was 0.27 (range 0.16-0.42) in patients with intact ventricular septum, and 0.34 (range 0.23-0.40) in patients with VSD. The LPEP/LVET was significantly less in patients with intact ventricular septum than in normal controls. The flow velocity patterns of the RVOT and LVOT were the same as those for normal controls. The mean AcT/RVET ratio was 0.51 (range 0.49-0.55) in patients with intact ventricular septum and 0.49 (range 0.39-0.67) in patients with VSD. The mean AcT/LVET was 0.32 (range 0.27-0.38) in patients with intact ventricular septum and 0.28 (range 0.20-0.45) in patients with VSD. The flow patterns did not change after intraatrial baffle repair. Our studies indicate that the flow velocity patterns of the RVOT and LVOT in patients with D-TGA were not reversed, in spite of inverted afterloads.

Ultrasonographic evaluation of cavum septi pellucidi and cavum Vergae.

Cavum septi pellucidi (CSP) and cavum Vergae (CV) are frequently found in premature and mature neonates an autopsy. We examined 116 premature infants, 45 full-term neonates and 31 healthy 1-month-old infants to clarify the incidence of CSP and CV using cranial ultrasonography through anterior fontanelle. CSP was detected in 97% of the premature infants, 56% of the full-term neonates and 29% of the 1-month-old infants. The incidence of CV was 60% in premature infants and 7% in full-term neonates. None of the 1-month-old infants were found to have CV. The size of the largest CSP observed ultrasonographically was 10 mm wide. No complicated pathological cavum was found in any subject.