Obstruction of the left ventricular outflow tract may be associated with hypoplasia of the left heart, which importantly influences the options for treatment. Although the influence of the size of the left heart on the outcome for critical aortic stenosis has been described, less is known about the spectrum of such hypoplasia seen with neonatal aortic coarctation, and how this influences outcome. To determine, first, the spectrum and influence of hypoplasia of the left heart in neonatal coarctation, second, if the previously described critical values for adequacy of the left heart in neonates with critical aortic stenosis are applicable to neonates with coarctation, and, third, if any of the variables or associated abnormalities are risk factors for recoarctation, we studied 63 neonates who underwent repair of coarctation. From the initial echocar diogram, we measured multiple structures in the left heart, and calculated a score for adequacy as has been done for critical aortic stenosis. The sizes were compared to previously reported minimal values. We then analyzed the influence of the variables and the associated anomalies on outcome. There were no deaths. There was a broad spectrum of sizes that did not correlate with the need for re-intervention. The calculated score for adequacy would have predicted survival in only 56% of the patients, and 73% of the neonates had at least one parameter measured in the left heart below the previously reported minimal values. There is, therefore, a broad spectrum of sizes for the left heart in neonates with aortic coarctation that is not predictive of outcome. Minimal sizes, and the score for adequacy used for critical aortic stenosis, are not applicable to neonates with coarctation.

The aim of the present study was to evaluate different aspects of a device designed to monitor physical activity. Measurements of different axes and placement of the monitor were tested using a treadmill with ranging increments in incline or speed. The monitor was also used to assess the level of physical activity among children and adolescents with congenital heart disease and in healthy controls at the same age. The results indicate that the monitor is a valid and reproducible instrument for measurements of physical activity. The study revealed that the level of activity was higher for healthy boys than healthy girls (p<0.0001). Boys with congenital heart disease also displayed higher values compared to girls with congenital heart disease, although the difference was not significant (p=0.067). Healthy boys revealed a significantly higher level of activity than did boys with congenital heart disease (p=0.003), but no such difference was found in girls (p=0.757). Nor were any differences found between younger and older indi viduals among patients with congenital heart disease. Young healthy controls, however, showed significantly higher levels of activity than their older counterparts. There were differences in activity monitored during the week, with lower activity in the weekends, but the activity on the same day in different weeks seemed stable. Neither were there any differences between measurements over whole weeks. The results indicate that the Computer Science & Application monitor is a valid instrument for assessing physical activity. The monitor may also be used, therefore, to validate the levels of physical activity level in children with congenital heart disease after medical and surgical treatment.

In order to test the effect of systematic supervised physical training, we divided a total of 129 children and adolescents with congenital heart disease into a group undergoing intervention and a control group. All patients underwent exercise tests, measurements of physical activity, and a survey of psychosocial factors. An improvement in uptake of peak level of oxygen was observed after intervention. There was also an improvement in physical activity in both groups measured by a monitor, although this was significant only in those with intervention. The psychosocial scales measured by the Child Behavior Checklist showed a decrease in internalizing scores for those subjected to intervention. This was decreased due to decreased withdrawal and somatic complaints. In conclusion, we recommend systematic supervised training, including testing of routine follow-ups, in patients with congenital heart disease.

Doppler derived systolic pressure gradients have become widely applied as noninvasively obtained estimates of the severity of aortic valvar stenosis. There is little correlation, however, between the Doppler derived peak instantaneous gradient and the peak-to-peak gradient obtained at catheterisation, the latter being the most applied variable to determine severity in children. The purpose of this study was to validate a mathematical model based on data from catheterisation which estimates the peak-to-peak gradient from variables which can be obtained by noninvasive means (Doppler derived mean gradient and pulse pressure), according to the formula: peak-to-peak systolic gradient=6.02+1.49*(mean gradient)−0.44*(pulse pressure). Simultaneous cardiac catheterization and Doppler studies were performed on 10 patients with congenital aortic valvar stenosis. Correlations between the gradients measured at catheter measured, and those derived by Doppler, were performed using linear regression analysis. The mean gradients correlated well (y=0.67 × + 11.11, r=0.87, SEE=6 mm Hg, p=0.001). The gradients predicted by the formula also correlated well with the peak-to-peak gradients measured at catheter (y=0.66 × + 14.44, r=0.84, SEE=9 mm Hg, p=O.002). The data support the application of the model, allowing noninvasive prediction of the peak-to-peak gradient across the aortic valvar stenosis.

We describe two children with isolated origin of the left subclavian artery from the left pulmonary artery detected by echocardiography during the assessment of their congenital cardiac malformations. Both patients demonstrated pre-operative evidence of subclavian steal. This entity results from persistence of the dorsal segment of the sixth left arch, with regression of the left fourth arch and interruption of the left dorsal arch distal to the origin of the seventh left intersegmental artery. The significance of this finding relates to the potential for pulmonary overcirculation, which could have significant post-operative ramifications if not detected prior to surgical repair of an associated cardiac malformation. This entity differs from cases with a right aortic arch and aberrant left subclavian artery which has the potential to form a vascular ring, unlike cases with isolated origin of the left subclavian artery from the pulmonary artery that do not cause compression of the airway.

We present two cases in which there was isomeric arrangement oi the left atrial appendages in association with multiple spleens and complete heart block. In both of these, the venous connections were normal. In one case the arrangement of the bronchial tree and the lungs was normal, the liver was left-sided, and the stomach was right-sided. In the other case, there was left isomeric broncho-pulmonary morphology, and both liver and stomach were right-sided. These unusual combinations show the need for full description of the morphology of other organs, and the venoatrial connections, in cases with isomeric arrangement of the atrial appendages. They also demonstrate that connection of the inferior caval vein cannot be taken as a reliable marker of an atrium having a morphologically right appendage.

A young female athlete is described with anomalous origin of the main stem of the left coronary artery from the non-facing sinus of Valsalva who sustained myocardial infarction and died suddenly after physical exertion. Autopsy findings illustrated the mechanistic importance of acute angle take-off of the left main coronary artery and a slit-like orifice, which was likely compressed and obstructed by acute expansion of the aortic wall. This rare type of coronary anomaly has been regarded as having little clinical significance, but it can lead to sudden cardiac death under physical exertion.

An atrial septal defect is commonly thought of as a benign cardiac lesion especially in infancy. The haemodynamic consequences for the lungs, nonetheless, can be comparable to that produced by patency of the arterial duct. In a preterm boy, this lesion led to the development of broncho-pulmonary dysplasia, and dependence on a respiratory ventilator. He could be extubated shortly after surgical closure of the septal defect.

An hydropic fetus seen at 28-weeks gestation had a saccular aortic aneurysm in the descending thoracic aorta. Histology disclosed marked fibrointimal hyperplasia, thrombus, and attenuation of the tunica media. The remainder of the descending thoracic aorta showed fibrointimal hyperplasia. We speculate that the narrowed lumen and rigid aortic wall resulting from this vasculopathy provided an increased afterload leading to cardiac failure.

Intermittent functional closure of a patent duct has been reported both clinically and echocardio-graphically. We describe the case of a 6-year-old with intermittent complete closure of a patent duct occurring during attempted transcatheter closure. Recurrence of clinical and echocardiographic signs prompted restudy. A patent duct of moderate size was demonstrated and successfully occluded by coil embolization. The interventional aspects of this unusual condition are discussed.

We discovered supraventricular tachycardia with advanced hydrops in the setting of normal cardiac anatomy at 26 weeks of gestation which resolved successfully following administration of digoxin and flecainide to the mother. A female baby was born after a premature rupture of the membranes at 30.6 weeks. The neonate was in sinus rhythm, showed a progressive regression of right ventricular insufficiency, but developed signs of acquired pulmonary valvar and subvalvar stenosis at 2 months of age.

We describe a rare instance of isolated pulmonary regurgitation caused by a dysplastic pulmonary valve which was detected prenatally. Fetal echocardiography demonstrated severe pulmonary regurgitation, and progressive cardiomegaly because of right ventricular volume overload. After birth, conservative therapy was successful in alleviating the pulmonary vascular resistance, and the pulmonary regurgitation gradually decreased.