The antenatal detection rate of major congenital heart disease in Scotland.

Ultrasonography has been used to detect fetal congenital heart disease since the 1980s, although there has been little uniformity in policy. In the UK, data published for 1993-1995 found an average detection rate of 23.4% with a Scottish rate of 9.6%. At the time of this study, 10 out of the 14 health boards in Scotland were implementing the 16-22-week fetal anomaly scan (FAS). The aims of this study were to determine the rate of antenatal diagnosis of major congenital heart disease (MCHD) throughout Scotland during 1 January to 31 December 2006, to document the regional differences in detection and to determine if implementation of a FAS significantly improved rates. Retrospective data were collected from the centralized Paediatric Cardiac Services in Edinburgh and Glasgow. The database contains information on all infants with cardiac disease in Scotland. All infants requiring cardiac intervention or dying in the first year of life were included. The results showed that the antenatal diagnosis rate for Scotland was 28% (95% CI 21.2%, 34.9%). The Scottish health boards that had implemented the FAS had a significantly higher antenatal detection rate (χ(2) test P = 0.037). In conclusion, antenatal detection rate of MCHD varies widely throughout Scotland. Implementation of the FAS has made a significant difference to antenatal diagnosis.

Transesophageal echocardiographic guidance of blade atrial septostomy in children.

Transesophageal echocardiography (TEE) was used to guide blade atrial septostomy in children requiring decompression of right or left atrium. In conjunction with biplane fluoroscopy, TEE was used to position the blade septostomy catheter and to monitor the pullback from left atrium to right atrium. Subsequent balloon dilatation of atrial septum was also carried out under TEE control. The use of TEE monitoring facilitated the procedure by providing optimal views of intracardiac structures while also limiting the total radiation exposure.

Transthoracic three-dimensional echocardiography in the preoperative assessment of atrioventricular septal defect morphology.

A prospective study of 3-dimensional (3-D) transthoracic echocardiographic definition of atrioventricular septal defect (AVSD) morphology and its dynamic changes during the cardiac cycle was performed. The information obtained from 2-D and 3-D transthoracic echocardiography (TTE) was compared with intraoperative findings in an unselected group of 15 patients with AVSD (median age 22 months). In all study patients, 3-D reconstructions provided anatomic views of the atrioventricular valve(s) en face from either atrial or ventricular perspectives that allowed comprehensive assessment of dynamic valve morphology and the mechanism of valve reflux. Left-sided valve function was correctly assessed by 2-D TTE in 11 of 15 patients (73%) and in 14 of 15 (93%) by 3-D TTE. In 6 of 15 patients (40%), the severity of right-sided valve reflux was described precisely by 2-D TTE and in 12 of 15 patients (80%) by 3-D TTE. Additionally, 3-D TTE supplemented the diagnostic information to that available from 2-D TTE on atrial and ventricular septal defects. Although primum atrial septal defects were depicted by 2-D and 3-D TTE in all 15 patients, the description of defect size was more precise by the 3-D TTE (80% vs. 100%, respectively). The presence of secundum atrial septal defect was correctly diagnosed by both TTE techniques in 10 of 15 patients. Disagreement regarding the size of the defect was present only in 2 of 10 patients by 2-D TTE. In another 2 patients, 3-D TTE described multiple defect fenestrations that were missed by 2-D TTE. Thus, the agreement score was 73% for 2-D and 100% for 3-D echo. The agreement for the presence and sizing of ventricular septal defects was 67% for 2-D and 93% for 3-D echo. We conclude that 3-D TTE provided accurate anatomic reconstructions of the common atrioventricular junction and that the use of dynamic 3-D TTE enhanced the anatomic diagnostic capability of standard 2-D TTE. Medica, Inc.

Assessment of atrial septal defect morphology by transthoracic three dimensional echocardiography using standard grey scale and Doppler myocardial imaging techniques: comparison with magnetic resonance imaging and intraoperative findings.

OBJECTIVE: To determine whether transthoracic three dimensional echocardiography is an accurate non-invasive technique for defining the morphology of atrial septal defects (ASD). METHODS: In 34 patients with secundum ASD, mean (SD) age 20 (17) years (14 male, 20 female), the measurements obtained from three dimensional echocardiography were compared to those obtained from magnetic resonance imaging (MRI) or surgery. Three dimensional images were constructed to simulate the ASD view as seen by a surgeon. Measured variables were: maximum and minimum vertical and horizontal ASD dimension, and distances to inferior and superior vena cava, coronary sinus, and tricuspid valve. In each patient two ultrasound techniques were used to acquire three dimensional data: standard grey scale imaging (GSI) and Doppler myocardial imaging (DMI). RESULTS: Good correlation was found in maximum ASD dimension (both horizontal and vertical) between three dimensional echocardiography and both MRI (GSI r = 0.96, SEE = 0.05 cm; DMI r = 0.97, SEE = 0.04 cm) and surgery (GSI r = 0.92, SEE = 0.06 cm; DMI r = 0.95, SEE = 0.06 cm). The systematic error was similar for both three dimensional techniques when compared to both MRI (GSI = 0.40 cm (27%); DMI = 0.38 cm (25%)) and surgery (GSI = 0.50 cm (29%); DMI = 0.37 cm (22%)). A significant difference was found in both horizontal and vertical ASD dimension changes during the cardiac cycle. This change was inversely correlated with age. These findings were consistent for both DMI and GSI technique. In children (age < or = 17 years), the feasibility of detecting structures and undertaking measurements was similar for both echo techniques. However, in adult ASD patients (age > or = 18 years) this feasibility was higher for DMI than for GSI. CONCLUSIONS: Transthoracic three dimensional imaging using both GSI and DMI accurately displayed the varying morphology, dimensions, and spatial relations of ASD. However, DMI was a more effective technique than GSI in describing ASD morphology in adults.

Stage IV malignant intrapericardial germ cell tumor: a case report.

The mediastinum is a common site for germ cell tumors. We describe an unusual case in an 18-month-old girl who presented with respiratory distress and cardiomegaly. An echocardiogram revealed a large pericardial effusion and a mass adjacent to the heart. She underwent detailed investigation and at open exploration was found to have a wholly intrapericardial malignant yolk sac tumor. Staging investigations revealed a bony metastasis. The serum marker alpha-fetoprotein was elevated. She was treated with chemotherapy and is in remission 1 year after completion of treatment. We review the methods of diagnosis and follow-up of children with these tumors.

Haemolytic uraemic syndrome in children.

Of the 4070 children admitted in the department of paediatrics, 830 (24%) presented with diarrhoea. Eleven of these had haemolytic ureamic syndrome (HUS) characterised by microangiopathic haemolytic anaemia, thrombocytopenia and acute renal failure. Only 3 had positive stool cultures (E. Coli 2, shigella dysenteriae 1). Two children expired while the rest recovered with conservative management and peritoneal dialysis. Thus HUS should be remembered as a complication of diarrhoea and a cause of acute renal failure in children.