No abstract

Newborns exposed in utero to SSRIs, have a twofold higher risk of mild nonsyndromic heart defects than unexposed infants. The data suggest that women who require SSRI treatment during pregnancy can be reassured that the fetal risk is low and possible cardiac malformations will probably be mild. Late-targeted ultrasound and fetal echocardiography at 22 to 23 weeks' gestation are recommended in this patient group.

Velocardiofacial syndrome (VCFS) is a relatively common developmental neuropsychiatric syndrome caused by a 22q11 microdeletion. There is an extensive variability in the phenotypic expression of this disease. The most common psychiatric disorder in VCFS is attention-deficit/hyperactivity disorder (ADHD), affecting 35-55% of patients. This study investigated the association of familial, developmental, and physical factors with the occurrence of ADHD in 51 patients with nonfamilial VCFS. Twenty-one patients (41.2%) were diagnosed with ADHD. There was a significantly greater prevalence of ADHD in the first-degree relatives of the patients with ADHD than in those without (OR = 5.9, 95% CI = 1.6-22.1, P = 0.006). No differences were noted between the ADHD and non-ADHD groups in mean Obstetric Complication Scale Score, gestational age, birth weight, age at first words, walking, and achieving bowel control. The two groups also had similar IQ scores (total, verbal, and performance) and had a similar average degree of severity of facial dysmorphism and cardiac and cleft anomalies. These findings indicate that ADHD in VCFS has a genetic contribution and the patients' VCFS-related developmental factors and physical illnesses play a lesser role.

These results with the longest follow-up period in the literature suggest that i.v. high-dose DFO for 8-10 h daily may be as effective as continuous 24-h infusion for the reversal of established cardiac disease in TM.

The acute complications of therapeutic cardiac catheterization for congenital heart disease as performed currently in a small unit were reviewed. In recent years, there has been a significant increase in the number of lesions thought amenable to catheter therapy. Only a few reports, however, have addressed the overall incidence of acute complications of therapeutic cardiac catheterization, all representing the experience of centres performing moderate-to-large numbers of procedures. A retrospective review was performed of 425 therapeutic catheter procedures performed at our institution between May 1993 and November 1997. Acute complications were retrieved from the database. This included all adverse events that were clinically recognized at the time of or within 2 weeks after the procedure and which, to the best of the authors' clinical judgement, were related to the catheterization and not part of the natural history of the child's illness. All patients were observed overnight following the procedure, and stayed in hospital if a complication developed. There were 49 acute complications (11.5%), of which 43 (10.1%) were deemed minor and 6 (1.4%) were considered major. The rate was low in patients with valvar pulmonary stenosis, including three neonates (3/45, 6.7%), for those undergoing angioplasty of native co-arctation (1/15, 6.7%) and pulmonary arteries (2/27, 7.4%); and for coil embolization of systemic to pulmonary collateral arteries (1/16, 6.3%). The rate was high in patients with valvar aortic stenosis, including 12 neonates (9/37, 24.3%), and for angioplasty of re-coarctation (4/23, 21.7%). There were more overall complications in neonates (25.6%) than in older patients (10.1%) (p < 0.01). Two patients died (0.5%), but no patient required emergency surgical intervention. In spite of the introduction of many new therapeutic modalities with greater intrinsic risk, and the fact that patients with more complex lesions and who are more acutely ill are being treated, the overall rate of complications remains relatively low. This probably reflects improvements in pericatheterization medical management, in selection of patients, in procedural techniques, and in the experience of operators.

Early surgical repair of anomalous origin of one pulmonary artery from the ascending aorta is feasible and safe even in newborn and premature babies with complete resolution of the pulmonary hypertension and normalisation of pulmonary vascular resistance.

The use of MPH in children with VCFS appears to be effective and relatively safe. A comprehensive cardiovascular evaluation for children with VCFS before and during stimulant treatment is recommended.

The objective of this study was to determine the procedural success rate of balloon angioplasty for branch pulmonary artery stenosis in terms of its clinical impact on the subsequent management of these patients. Most previous studies of balloon angioplasty have concentrated on the initial success rate (50-60%), complications (6-10%), recurrence rate ( approximately 15%), and technical issues. A favorable clinical impact was noted in only 35% of patients. Over a 3-year period (March 1990 to March 1993), 32 patients (17 boys, 15 girls) underwent 34 balloon angioplasty procedures. Their mean age at dilation was 7.6 +/- 4.3 years (range 1.1-19.0 years). Postoperative tetralogy of Fallot and tetralogy of Fallot with pulmonary atresia were the most frequent cardiac lesions (44%). The procedures were "technically" successful in 56% (19 of 34) of balloon dilations on the basis of at least two of the following criteria: an increase of >50% of the predilation diameter; an increase of >20% in the relative flow to the affected lung by radioisotope study; or a decrease in the systolic right ventricular/aortic pressure ratio from 85-100% to <60%. Twelve percent of the patients had transient complications (two deep vein thromboses, one unilateral pulmonary edema, and one pneumothorax). In 17 of 19 (89%) of the patients there was a favorable clinical impact on their subsequent care as based on one of the following criteria: resolution of the stenosis and avoidance of surgical intervention (n = 14); optimization of future surgical procedure (n = 3); reduction in right ventricular pressure to <60% of aortic pressure (n = 13). Five patients who had unsuccessful balloon angioplasty and one with initially successful balloon angioplasty later underwent endovascular stent placement, which also favorably influenced their clinical status. The success rate of balloon angioplasty for branch pulmonary artery stenosis, when measured by strict procedural criteria, is accompanied by a favorable clinical impact in more than 50% of patients. Hence this procedure should be the initial therapeutic modality in this setting despite the relatively high transient complication rate. The use of endovascular stents probably increases the favorable clinical impact.