Survival after first esophageal variceal hemorrhage in patients with biliary atresia.

OBJECTIVE: To determine the influence of the new onset of esophageal variceal hemorrhage (EVH) on transplant-free survival in children with biliary atresia and to examine variables that predicted survival after the onset of EVH. METHODS: Retrospective chart review of 134 patients with biliary atresia who underwent portoenterostomy between 1973 and 1992 at a single institution; 29% had EVH. RESULTS: The risk of death or need for liver transplantation was 50% at 6 years after the initial episode of EVH. Patients with a serum bilirubin concentration < or =4 mg/dL at the first episode of EVH had transplant-free survival of >80% for 4 years after this episode, those with bilirubin levels >4 to 10 mg/dL had 50% survival at 1 year, and those with bilirubin levels >10 mg/dL had 50% survival at 4 months. The risk of death or transplant for a child with EVH and total serum bilirubin levels >10 mg/dL was 12.0 (95% CI: 6.0, 24.1), 4 to 10 mg/dL was 7.2 (3.1, 16.7), and < or =4 mg/dL was 0.6 (0.1, 3.1) times the risk of a same-aged child who did not have EVH. CONCLUSIONS: Children with biliary atresia and first EVH episode have a variable prognosis related to total serum bilirubin concentration at the time of the episode.

Cardiopulmonary malformations in the inv/inv mouse.

The inv/inv mouse carries an insertional mutation in the inversin gene, (inv, for inversion of embryonic turning). Previously it had been reported that almost 100% of the homozygous offspring (inv/inv) were characterized by situs inversus totalis. In this report we identify the spectrum of cardiopulmonary anatomical abnormalities in inv/inv mice surviving to birth to determine whether the abnormalities seen are of the categories classically associated with human situs abnormalities. Stillborn mice, offspring that died unexpectedly (within 48 hr after birth), and neonates with phenotypic characteristics of situs inversus (right-sided stomachs, growth failure or jaundice) were processed for standard histological examination. Of 173 offspring, 34 (20%) neonates (11 stillborn, 9 unexpected deaths, and 14 mice with situs inversus phenotype) were examined, 27 of which were genotyped to be inv/inv. Interestingly, three inv/inv mice (11%) were found to have situs solitus. Twenty-four had situs inversus with normal, mirror-image cardiac anatomy (dextrocardia with atrioventricular concordance, ventriculoarterial concordance and a right aortic arch). The overall incidence of cardiovascular anomalies observed was 10 out of 27 (37%). The most frequent severe malformation, identified in 3 out of 27 animals, was a complex consisting of pulmonary infundibular stenosis/atresia with absence of pulmonary valve tissue and a ventricular septal defect. The pulmonary phenotype in inv/inv mice was situs inversus with occasional minor lobar abnormalities. We conclude that 1) cardiopulmonary malformations in inv/inv mice are not rare (37%), 2) the cardiopulmonary malformations observed in inv/inv specimens are not of the spectrum typically associated with human heterotaxia. In particular, inv/inv mice have a propensity for defects in the development of the right ventricular outflow tract and the interventricular septum, and 3) approximately one out of ten inv/inv mice is born with situs solitus and shows cardiac anomalies that correspond to those observed in inv/inv specimens with situs inversus. Our data therefore suggest that inversin, the product of the inv locus, may have specific roles in cardiac morphogenesis independent of its role in situs determination.

Transcatheter fenestration of hemi-Fontan baffles after completion of Fontan physiology using balloon dilatation and stent placement.

De novo transcatheter fenestration of hemi-Fontan baffles has not been previously described. The purpose of this report is to present our experience in such de novo transcatheter fenestration in two consecutive patients with absent fenestration in whom the hemi-Fontan baffle was the only direct access to the pulmonary venous atrium.

Incidence of femoral vein occlusion after catheter ablation in children: evaluation with magnetic resonance angiography.

Catheter ablation in children requires placement of multiple large femoral venous sheaths and catheters. Magnetic resonance angiography (MRA) was used to evaluate the effect of indwelling lines on femoral venous blood flow. Between October 1993 and February 1994 a total of 17 patients scheduled for catheter ablation underwent venous MRA. Two-dimensional time-of-flight MRA was performed 12-70 hours after catheterization on all patients. All patients received intravenous heparin during the procedure and had aspirin therapy instituted after ablation. Eighteen catheter ablations and MRA studies were performed on the 17 patients (one patient underwent repeat ablation). There were 7 females and 10 males, with a mean age of 14.8 +/- 4.2 years (range 8-21 years). Patients had three venous sheaths inserted in the left femoral vein (5F, 6F, and 7F with external diameters measuring 1.7, 2.0, and 2.3 mm, respectively) and one sheath in the right femoral vein (7F). Four patients (22%) had altered venous flow (two complete obstructions and two partial obstructions) following catheterization. None of these patients experienced symptoms or complications. It was concluded that there is an increased incidence (22%) of venous obstruction following catheter ablation, but there are no related complications. Venous MRA provides a rapid, noninvasive method for evaluating venous flow abnormalities and possibly detects patients at risk for complications.

Interatrial septal aneurysms and atrial arrhythmias in infants.

Studies have suggested that interatrial septal aneurysms (IASAs) may be the initiating mechanism of supraventricular tachycardia (SVT) in newborns and infants. A retrospective study was performed to determine the incidence of IASAs in 30 infants with atrial arrhythmias (SVT, atrial flutter, or frequent premature atrial contractions) and their possible relation to the mechanism of atrial arrhythmias. An IASA was defined as dilation of the septum > 5 mm beyond the plane of the atrial septum and associated with redundant tissue and abnormal mobility. The study patients were compared with age and sex-matched control subjects. Four (13%) of the 30 study patients and 2 (7%) of 30 control subjects had an IASA (difference not significant). In contrast to previous reports, this study demonstrates that there is not a significant relation between the presence of IASAs and the onset and recurrence of atrial arrhythmias.

High prevalence of repolarization abnormalities in children with simple ventricular ectopy.

BACKGROUND: Premature ventricular contractions (PVCs) may occur in 0.3 to 2.2% of routine resting electrocardiograms (ECGs) in children with structurally normal hearts. HYPOTHESIS: This study tests the hypothesis that repolarization abnormalities are present more frequently on the surface ECG in pediatric patients with benign ventricular ectopy. METHODS: A retrospective study was performed examining 32 children with benign ventricular ectopy and structurally normal hearts. The surface ECG was carefully reviewed, concentrating on repolarization abnormalities as evidenced by a prolonged QTc, a prolonged JTc, and/or an abnormal T-wave vector. QTc was calculated using Bazett's formula and considered prolonged if > 440 ms, and the JTc was calculated using a formula analogous to Bazett's formula and considered prolonged if > 340 ms. The T-wave vector was considered normal if between 0 to +90 degrees, concordant with the frontal QRS axis and within 60 degrees of the QRS axis. There were 13 females (41%) and 19 males (59%) with an average age of 9.1 years (range 1-16.1 years). These patients were then compared with age- and gender-matched controls. Fourteen of 32 study patients (44%) and 2 of 32 controls (6%) had evidence of repolarization abnormalities. RESULTS: Using chi 2 analysis, there was a significant difference between groups (p = 0.0005). No patient had an abnormal T-wave vector and there were no other significant ECG abnormalities. There was no significant relationship between repolarization abnormalities and patient gender or age. CONCLUSION: A significant percentage (44%; P = 0.0005) of children with benign ventricular ectopy have associated repolarization abnormalities as evidenced by a prolonged QTc and/or JTc. This finding suggests that children with "benign PVCs" may have repolarization changes indicative of underlying substrate abnormalities.

Hyperammonemia: the silent killer.

Nitrogen, derived from breakdown of dietary amino acids as ammonia, is normally converted to urea and excreted. Impairment in the conversion process (called the urea cycle) can occur, either as a consequence of primary genetic defects or through secondary suppression of enzyme activity. Either process results in hyperammonemia, producing a clinical picture virtually indistinguishable from many other diseases of infancy. Moreover, there is no way to detect hyperammonemia except to measure the blood ammonia level. Thus, the capability to do so is a minimum standard of care in any hospital setting. Use of commonly obtained laboratory studies is discussed in the context of rapid, presumptive diagnosis of the causes of hyperammonemia.