Background: Infantile hepatic hemangioma is a benign liver tumor with potentially fatal complications. Response of these lesions to steroid therapy has been well documented in infants. Successful antenatal treatment of a fetal hepatic hemangioma with maternal corticosteroids is reported. Case: A 36-year-old woman, gravida 2, para 1, had an ultrasound at 17 weeks which showed a fetal hepatic hemangioma. Because of increase of the tumor and enlarged fetal heart, maternal oral corticosteroid therapy was commenced at 28 weeks, which progressively decreased the mass. The infant was delivered at 36 weeks with no evidence of heart failure, and at 7 months the tumor was barely seen on ultrasound. Conclusion: When an enlarging fetal hepatic hemangioma is detected antenatally, maternal corticosteroid therapy should be considered.

The trend to perform early primary repair of tetralogy of Fallot prompted us to review our experience in the current era with the traditional protocol consisting of palliation during infancy, if required, and repair after infancy. During a 10-year period, 270 infants with tetralogy of Fallot presented aged less than 18 months. Thirteen infants (4.8%) had major noncardiac lesions that precluded definitive care for their congenital heart disease. Twenty infants (7.4%) had major associated cardiac lesions (atrioventricular septal defect or absent pulmonary valve syndrome, or both). Survival in this group was poor, with only 58% +/- 12% reaching the age of 10 years. Four of the seven deaths occurred before intracardiac repair was performed. The remaining 237 infants presented with isolated tetralogy of Fallot. Eight-nine percent +/- 2.3% survived to age 10 years. Sixty percent of these infants required palliation, and survival in these infants did not differ from that in those who never required palliation. However, 19 infants (8%) required palliation in the first month of life. In these children, survival to age 10 years was significantly lower (77%), secondary palliation was frequently required (n = 11), and a transannular patch or conduit at the time of repair (10 of 14 patients) was more likely needed than it was in children who had not undergone a palliative procedure during the neonatal period. The survival in infants with tetralogy of Fallot is unlikely to be different, regardless of whether primary repair or a staged repair is carried out. The quality of survival, including the exercise capability and absence of arrhythmias, must be assessed to determine which protocol is superior.

Twelve children with pulmonary atresia and intact ventricular septum underwent closure of the tricuspid valve as a part of a new surgical procedure. In two cases a concomitant Fontan operation was performed. In each patient the right ventricle was very small and right ventricular pressure was higher than systemic pressure. Ventricle-coronary connections provided flow of desaturated blood from the right ventricle into the coronary arteries in 11 of 12 cases. Five of the 12 children did not survive operation and postmortem examination of each revealed severe acute and chronic myocardial ischemic damage and high-grade obstruction or interruption of the proximal left anterior descending coronary artery. Preoperative angiography demonstrated occlusive changes in the coronary arteries, resulting in right ventricular dependent circulation, in all five children who died and in one child who survived operation. Seven children who survived operation are well 4 months to 3.5 years later. Two have undergone subsequent successful Fontan operation and two others are considered suitable candidates for this operation. Tricuspid valve closure is recommended for a carefully selected group of infants with pulmonary atresia and intact ventricular septum provided a right ventricular-dependent coronary circulation can be excluded on the basis of preoperative coronary cineangiography.

A percutaneous catheter technique for permanent closure of the isolated, persistently patent ductus arteriosus (PDA) was first described in 1967.1 The development of a spring-loaded double foam disc occluder on a miniaturized (8 or 11Fr) delivery system2 placed transvenously3,4 has allowed this management approach to become an effective alternative to surgery in infants and children. The recent description of an improved release technique and the application of topical thrombin onto the foam of the occluder may further reduce embolization risk and increase total occlusion rates.5 Little attention, however, has been given to the variation in anatomy and topology of the isolated PDA and its influence, if any, on the technique of catheter occlusion. This report describes the angiographic appearances of isolated PDA as seen at the time of catheter occlusion, presents a classification of the observed variations and comments on how these may affect the procedure.

The role of combined two-dimensional and pulsed Doppler echocardiography in the postoperative assessment of patients with total anomalous pulmonary venous connection was evaluated. Twenty-two cases with a median age of 9.5 weeks at the initial examination were evaluated. Serial ultrasound examinations were performed throughout the study period. The ultrasound results were compared with chest radiographs obtained during the same period. Of the 22 patients, 16 had normal pulmonary venous flow profiles characterized by low-velocity laminar flow. Of this group 12 had persistent radiographic postoperative pulmonary edema that cleared in all by 4 months. Six patients with pulmonary venous obstruction were identified, the diagnosis being confirmed at catheterization or autopsy. The venous flow pattern in this group was uniformly high velocity and turbulent. It was possible to localize the site of obstruction in each case. Although pulmonary edema was present in each patient, the chest radiograph did not provide reliable information as to the exact site of obstruction. Combined two-dimensional and Doppler echocardiography is a useful adjunct in the postoperative evaluation of patients with total anomalous pulmonary venous connection.

Twenty nine patients (19 male, mean (SD) age 6.25 (0.5) years (range 0.16-15 years] with typical pulmonary valve stenosis were treated by balloon dilatation of the pulmonary valve. They were studied by echocardiography before the procedure, immediately after it, and at follow up (mean (SD) 10.2 (5.6) months, n = 18). The morphology of the pulmonary valve, the right ventricular-pulmonary artery gradient, and ratio of the systolic to diastolic endocardial dimensions (infundibular ratio) were examined. No patient had pulmonary regurgitation before the study. The valve gradient was significantly reduced (47%) from a mean (SD) of 72 (31) to 37 (23) mm Hg with no short term change in cardiac index after dilatation with a balloon with a mean (SD) diameter that was 118 (10.8)% of the valve annulus. The infundibular ratio was unchanged by the procedure (0.49 (0.11) (n = 21) before dilatation and 0.47 (0.14) (n = 16) after dilatation). In twenty seven patients the commissure of the pulmonary valve was seen to be torn after dilatation. Two patients with bicuspid valves had flail leaflets. Doppler examination at follow up showed mild pulmonary insufficiency in all 29 patients; the mean (SD) valve gradient (31 (+/- 21) mm Hg) at follow up was no different from the gradient found immediately after the procedure and infundibular ratio (0.58 (0.15) was not abnormal. These data indicate that commissural tears are the primary mechanism of valve disruption and demonstrate that the dynamic right ventricular outflow tract obstruction relaxes and gradient reduction persists at follow up.

Sixty CT scans in 31 patients who underwent lumboperitoneal shunting for communicating hydrocephalus showed that the size of the ventricles did not represent a good indicator of shunt malfunction. Instead, we discovered that the size of the basal cisterns around the brain stem enabled us to predict blockage earlier and more reliably. In a well functioning shunt, the basal cisterns are usually not visualized. In children with clinical shunt malfunction the cisterns dilate and become visible again. This occurs earlier and more frequently than ventricular enlargement. We therefore conclude that visible cisterns in association with persistent symptoms of malfunction are more reliable predictors of a true blockage that requires shunt revision than serial studies of ventricular size.

Twenty consecutive patients with ventricular septal defect and aortic valve prolapse were evaluated by cross-sectional echocardiography. Angiographic confirmation was available in all and surgical confirmation was found in 17. In 19, the right coronary cusp was involved and appeared to plug the defect in the precordial long- and short-axis cut. The cusp was deformed and appeared to pivot from the crest of the interventricular septum. In all 19 angiography demonstrated prolapse of the right cusp. Noncoronary cusp prolapse was observed in two by cross-sectional echocardiography and in six by angiocardiography. The ventricular septal defect was perimembranous in 14 and doubly committed subarterial in six by echocardiography. Angiographically, the ventricular septal defect was felt to be perimembranous in 15 and doubly committed in five. Aortic regurgitation was detected by Doppler interrogation in seven, all of whom underwent plication of the right coronary cusp. Angiographic evidence of regurgitation was noted in 11, but four were mild and possibly related to catheter position. Five patients had associated muscular right ventricular outflow tract obstruction and three had a subaortic ridge. Combined cross-sectional and pulsed Doppler echocardiography provide a reliable assessment of right coronary cusp prolapse associated with a ventricular septal defect. Noncoronary cusp prolapse appears more difficult to detect. This technique should help optimize the management of patients by providing a means of early detection prior to the development of aortic regurgitation.