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OBJECTIVE: To determine among unselected and low risk populations the accuracy with which fetal echocardiography during the second trimester detects congenital heart disease. DESIGN: A systematic review of studies that assess the accuracy of fetal echocardiography. SETTING: District General Hospital and Tertiary referral centres. POPULATION: Women during the second trimester attending for ultrasound assessment. METHODS: General bibliographic databases (e.g. MEDLINE, EMBASE) and specialist computerised databases (e.g. Cochrane Library, National Research Register), grey literature, manual searching of reference lists of primary and review articles and personal contact with experts were used to identify studies. Studies were included if fetal echocardiography among unselected or low risk pregnant women was compared against a postnatal reference standard. Data were extracted on quality, study design and characteristics, and accuracy data to construct 2 x 2 tables. Data were synthesised qualitatively, and sensitivity and specificity with 95% confidence intervals were calculated. MAIN OUTCOME MEASURE: Sensitivity and specificity. RESULTS: Five primary studies met the inclusion criteria and comprised 60,901 subjects. One study assessed the accuracy of fetal echocardiography among an unselected population and four studies among low risk populations. All eligible studies found that fetal echocardiography helped to correctly diagnose normal fetus among unselected and low risk populations. Correct diagnosis of babies for congenital heart defects was higher among the unselected population (85%) than among the low risk populations (range from 35% to 86%); however, the potential for ascertainment bias and the choice of reference standard limits the validity of this finding. The variation in sensitivity across studies was not explainable by clinical factors such as scanning regime, operator skill and equipment. CONCLUSIONS: The evidence from this review about the accuracy of fetal echocardiography does not lend support to its routine use among unselected and low risk populations during the second trimester to detect congenital heart disease.

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The purpose of our study was to determine the ways in which adolescents with congenital cardiac disease believed that the condition had affected their life, and how these views were related to their perceived health. Interviews were conducted with a series of 37 adolescents, 17 girls and 20 boys, aged from 11 to 18, as they attended the clinics of 4 paediatric cardiologists in a teaching hospital in the United Kingdom. Transcripts of the interviews were analysed for recurring themes. A questionnaire was formed consisting of a set of questions for each theme, and additional items eliciting "perceived health", and administered to a second series of 74 adolescents, 40 boys and 34 girls, who were again aged from 11 to 18 years. Slightly less than half (46%) perceived their health as either "good" or "very good", and one-third (33%) rated it as "average". The majority (66%) felt themselves to be "the same" as, or only very slightly "different" from, their peers. The assessment of the seriousness of their condition by the adolescents, the degree to which they saw themselves as different from others, and their perceived health, were not related to the "complexity of the underlying medical condition" as rated by their physician. It was the psychosocial themes, such as exclusion from activities or the effect of the condition on relationships, that were most strongly related to the perception of their health by the adolescents. Improved education of parents, teachers and peers, and attendance at classes for cardiac rehabilitation, might help to ameliorate some of these problems.

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AIMS: To evaluate illness knowledge and understanding in children and adolescents with congenital and acquired heart disease, and whether the degree of understanding is related to age, sex, or complexity of the heart disease. DESIGN: Prospective cohort study. SETTING: Tertiary paediatric cardiac centre. METHODS: Patients' understanding of their congenital heart disease was assessed in a representative sample of volunteers aged between 7-18 years using semistructured interviews based upon Leventhal's illness representation model. RESULTS: 63 of 69 interviews were suitable for analysis. There were similar numbers of boys and girls and a wide distribution of heart defects. Only 30% of patients had a good understanding of their illness; 77% did not know the medical name of their condition, and 33% had a wrong or poor understanding of their illness. Understanding was unrelated to age, sex, or the nature of the heart disease. Understanding of illness duration was significantly related to age, but not to sex or to the nature of the disease. CONCLUSIONS: Illness understanding is poor in children and adolescents with heart disease, and many have an entirely wrong concept of their disease. Intensified efforts to ensure better patient and parental understanding are needed.

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We present a modification of the Norwood stage 1 operation, where the neo-aorta was reconstructed without using graft material. After extensive mobilization of the descending aorta the ductus arteriosus was excised and an end-to-end anastomosis was created between the main pulmonary artery and the distal ductal-aortic junction. The proximal ascending aorta was anastomosed side-to-side to the neo-aorta. Finally either a direct side-to-side anastomosis was created or a 3.5-mm Gore-Tex graft was implanted between the innominate artery and the right pulmonary artery. The first three patients had a favourable outcome: echocardiography showed good ventricular function and acceptable saturation (85%) at the follow up.

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Alastrim variola minor virus, which causes mild smallpox, was first recognized in Florida and South America in the late 19th century. Genome linear double-stranded DNA sequences (186,986 bp) of the alastrim virus Garcia-1966, a laboratory reference strain from an outbreak associated with 0.8% case fatalities in Brazil in 1966, were determined except for a 530-bp fragment of hairpin-loop sequences at each terminus. The DNA sequences (EMBL Accession No. Y16780) showed 206 potential open reading frames for proteins containing >/=60 amino acids. The amino acid sequences of the putative proteins were compared with those reported for vaccinia virus strain Copenhagen and the Asian variola major strains India-1967 and Bangladesh-1975. About one-third of the alastrim viral proteins were 100% identical to correlates in the variola major strains and the remainder were >/=95% identical. Compared with variola major virus DNA, alastrim virus DNA has additional segments of 898 and 627 bp, respectively, within the left and right terminal regions. The former segment aligns well with sequences in other orthopoxviruses, particularly cowpox and vaccinia viruses, and the latter is apparently alastrim-specific.

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OBJECTIVES: To describe the incidence and management of haemolysis after transcatheter coil occlusion of the arterial duct. DESIGN: Prospective clinical and echocardiographic follow up of patients who have undergone implantation of the Cook detachable duct occlusion coil. SETTING: Tertiary paediatric cardiac centre. PATIENTS: Five cases of haemolysis (two girls aged 6 and 11 months; three boys aged 6, 17, and 14 months) from a series of 137 duct coil implantations. MAIN OUTCOME MEASURES: The occurrence of clinically significant haemolysis after implantation of duct occlusion coils and resolution of haematuria after completion of duct occlusion. RESULTS: Haemolysis was detected in five of 137 procedures following implantation of Cook detachable duct coils. Four patients became symptomatic 12 hours after the procedure but in one haemolysis was detected three months later. Resolution of ongoing haemolysis was achieved within 48 hours of detection with further coil implantations, but haematuria persisted for up to 10 days. In one patient the extensive destruction of erythrocytes resulted in acute renal failure requiring peritoneal dialysis. CONCLUSIONS: Haemolysis is an important complication after duct coil implantation. It occurred in 3.6% of 137 procedures in this series and is most likely to occur in young patients with relatively large ducts. Further coil implantation to occlude the duct completely is not only successful but technically relatively straightforward and should be undertaken early if major complications such as severe anaemia and renal failure are to be avoided.

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AIM: To examine the incidence and natural history of left ventricular hypertrophy (LVH) associated with the shorter 2-3 week course of dexamethasone, now more usual, for chronic lung disease. METHOD: Thirty one infants, gestational age 23-34 (median 26) weeks, birthweight 500-2054 (median 815)g, received dexamethasone, starting at 0.4-0.6 mg/kg/day, at a median of 11 days of age (range 2-34), weaning over a period of 2-3 weeks. Eighteen preterm neonates were studied as controls over a similar time period. Serial echocardiographic measurements of end diastolic interventricular septum (IVSd) and left ventricular posterior wall (LVPWd) thicknesses were taken before, and up to 48 days after, starting dexamethasone. Maximum Doppler blood flow velocities from the left ventricular outflow tract (LVOT) were measured. RESULTS: Left ventricular hypertrophy (LVH) occurred in 29 babies (94%). Median hypertrophy of the IVSd in those receiving dexamethasone was 67% and LVPWd 56% of baseline measurements, significantly greater than control infants (p < 0.001). LVH appeared by a median of three days, peaking by a median of 10 days. All resolved by a median of 27 days. LVOT obstruction was not seen. There was no significant correlation with birthweight, gestation, blood pressure, or glucose tolerance. CONCLUSIONS: LVH developed in almost all preterm neonates receiving a 2-3 week course of dexamethasone, but was of little clinical importance and always resolved. Echocardiography is probably not required routinely in infants receiving such short course dexamethasone for chronic lung disease.

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Cowpox virus Brighton red strain (CPV) contains a gene, crmD, which encodes a 320-aa tumor necrosis factor receptor (TNFR) of 44% and 22% identity, respectively, to the CPV TNFR-like proteins, cytokine response modifiers (crm) CrmB and CrmC. The crmD gene was interrupted in three other cowpox strains examined and absent in various other orthopoxviruses; however, four strains of ectromelia virus (ECT) examined contained an intact crmD (97% identity to CPV crmD) and lacked cognates of crmB and crmC. The protein, CrmD, contains a transport signal; a 151-aa cysteine-rich region with 21 cysteines that align with human TNFRII ligand-binding region cysteines; and C-terminal region sequences that are highly diverged from cellular TNFR C-terminal region sequences involved in signal transduction. Bacterial maltose-binding proteins containing the CPV or ECT CrmD cysteine-rich region bound TNF and lymphotoxin-alpha (LTalpha) and blocked their in vitro cytolytic activity. Secreted viral CrmD bound TNF and LTalpha and was detectable after the early stage of replication, using nonreducing conditions, as 60- to 70-kDa predominant and 90- to 250-kDa minor disulfide-linked complexes that were able to be reduced to a 46-kDa form and deglycosylated to a 38-kDa protein. Cells infected with CPV produced extremely low amounts of CrmD compared with ECT. Possessing up to three TNFRs, including CrmD, which is secreted as disulfide-linked complexes in varied amounts by CPV and ECT, likely enhances the dynamics of the immune modulating mechanisms of orthopoxviruses.

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AIM: To establish reference ranges for cardiac dimensions and Doppler measurements in preterm infants. METHODS: 79 infants of less than 34 weeks' gestation were examined by echocardiography on days 0, 7, and 28 after birth, to produce a set of reference ranges and to examine changes in these indices over the first month of life. The following dimensions were measured: interventricular septum, left ventricular posterior wall, left interventricular diameter at end systole and diastole, left atrium, and aortic root; Doppler measurements were made of maximum blood flow velocity (Vmax) through the pulmonary, aortic, mitral, and tricuspid valves. RESULTS: Reference ranges are given. Cardiac dimensions correlated well with gestation and birth weight but Vmax did not. There was a significant increase in measurements over time. The "normal" preterm infant also appeared to often have asymmetrical septal hypertrophy. Antenatal dexamethasone administration did not appear to affect the measurements. CONCLUSIONS: There is a close correlation with both gestation and birth weight for all physical measurements. Echocardiograms in preterm babies clearly differ from those in older children and adults.

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OBJECTIVE: To assess immediate and medium term results of transcatheter laser valvotomy with balloon valvoplasty in selected infants with pulmonary atresia and intact ventricular septum. DESIGN: Prospective study. SETTING: Tertiary cardiac unit. PATIENTS: All infants with pulmonary atresia and intact septum with no more than minor tricuspid valve hypoplasia referred between November 1990 and June 1995. Laser valvotomy was attempted in nine infants of median age 4-5 days and median weight 3.6 kg. INTERVENTION: The pulmonary valve was perforated using a 0.018 inch fibreoptic guidewire attached to a NdYag laser and introduced through a catheter positioned beneath the valve. After perforation the valve was dilated with progressively larger balloons. MAIN OUTCOME MEASURES: Successful laser valvotomy and balloon dilatation, complications, pulse oximetry, right ventricular outflow velocities, and need for surgical treatment. RESULTS: Valvotomy was successful in all but one case, the failure being due to laser breakdown. After perforation the valve was dilated to 6-8 mm diameter. Prostaglandin E was withdrawn immediately in six of the eight duct dependent infants, and 28 and 49 days later in two. No patient required an aortopulmonary shunt. Two patients had repeat valvoplasty at 20 days and three months of age, respectively; one required infundibular resection and closure of the atrial septum at age four and one is awaiting similar treatment. CONCLUSIONS: Laser valvotomy with balloon valvoplasty is safe and effective treatment for selected patients with pulmonary atresia and intact ventricular septum and should be considered as first line treatment in place of surgical valvotomy.

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OBJECTIVE: Preliminary assessment of the use of stents for palliative relief of right ventricular infundibular stenosis as an alternative to palliative surgical ventricular outflow enlargement. DESIGN: Descriptive clinical study. PATIENTS: Four patients with right ventricular outflow obstruction, aged between 2 and 15 years. One had had previous palliative surgery for pulmonary atresia, one had hypoplastic pulmonary arteries after palliative surgery for tetralogy of Fallot, one had multiple congenital abnormalities, and one had hypertrophic cardiomyopathy. SETTING: Tertiary paediatric cardiac centre. METHODS: After initial echocardiographic diagnosis the extent of right ventricular outflow obstruction was assessed by angiography. Balloon expandable stainless steel stents (Johnson & Johnson) were deployed in the right ventricular infundibulum. MAIN OUTCOME MEASURES: Improvement in right ventricular outflow assessed by ventriculography and change in right ventricular/ left ventricular pressure ratio, change in systemic oxygen saturation, freedom from arrhythmias, and sustained improvement in echocardiographic indices of obstruction. RESULTS: Mean right to left ventricular pressure ratio fell from 0.95 to 0.35 in the three patients with intact ventricular septum. Oxygen saturation increased from 76% to 91% in the patient with tetralogy. No arrhythmias were detected. Improvement was maintained at mean follow up of 9.7 months in three cases, but one patient required stent enlargement 17 months later because of neoendothelial proliferation within the stent. CONCLUSION: Stent implantation provides an effective alternative to palliative surgical enlargement of the right ventricular infundibulum. Neoendothelial proliferation causes reduction in lumen in some cases, but this may respond to redilatation.

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Twenty-six years have passed since Grover first described transient acantholytic dermatosis. Since then, the concept of Grover's disease has been expanded to include persistent acantholytic dermatoses as well. Although its origin remains unknown, it may result from an isomorphic response to excessive heat, sweating, or xerosis. It is currently classified as a nonfamilial, non-immune-mediated, acantholytic disorder.

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OBJECTIVE: To assess the effectiveness of modified, controlled release Gianturco coils for transcatheter occlusion of the arterial duct. DESIGN: Prospective study, approved by local medical ethics committee. SETTING: Tertiary paediatric cardiac centre. PATIENTS: 43 patients with left to right shunting through the arterial duct, two of whom had a residual leak after surgical ligation and three had residual shunting after previous Rashkind umbrella implantation. INTERVENTION: Transcatheter delivery of one or more coils to the arterial duct. MAIN OUTCOME MEASURES: Complete occlusion of the arterial duct, based on intention to treat and judged by Doppler echocardiography. Absence of flow disturbance in the branch pulmonary arteries and the descending aorta following the procedure. Assessment of cost of the disposable items used. RESULTS: At a median follow up period of three months complete duct occlusion was achieved in 37 (86%) of the 43 patients. No flow disturbance in the branch pulmonary arteries or the descending aorta was detected in any patient. The median cost of disposable items used during the procedure was 342 pounds. CONCLUSIONS: The Cook detachable coil is an effective and financially attractive alternative to the Rashkind umbrella for closure of the arterial duct.

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Genome DNA terminal region sequences were determined for a Brazilian alastrim variola minor virus strain Garcia-1966 that was associated with an 0.8% case-fatality rate and African smallpox strains Congo-1970 and Somalia-1977 associated with variola major (9.6%) and minor (0.4%) mortality rates, respectively. A base sequence identity of > or = 98.8% was determined after aligning 30 kb of the left- or right-end region sequences with cognate sequences previously determined for Asian variola major strains India-1967 (31% death rate) and Bangladesh-1975 (18.5% death rate). The deduced amino acid sequences of putative proteins of > or = 65 amino acids also showed relatively high identity, although the Asian and African viruses were clearly more related to each other than to alastrim virus. Alastrim virus contained only 10 of 70 proteins that were 100% identical to homologs in Asian strains, and 7 alastrim-specific proteins were noted.

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OBJECTIVES: We sought to determine the incidence of late perfusion defects attributable to coronary artery mobilization in patients undergoing anatomic correction for complete transposition of the great arteries. BACKGROUND: Anatomic correction (arterial switch procedure) is currently the surgical treatment of choice for complete transposition. From its conception, there has been concern about the impact on myocardial perfusion of the coronary artery mobilization and reimplantation involved in the correction. Previous studies have demonstrated myocardial perfusion defects in patients after correction, although a causal relation between coronary mobilization, and perfusion abnormality has not been established. METHODS: In a case-comparison study designed to test this hypothesis, 29 children underwent imaging with technetium-99m 2-methoxy isobutyl isonitrile (technetium-99m mibi). Ten had undergone anatomic correction (arterial switch group; interval from operation 6.9 +/- 1.42 years [range 4.9 to 9.1]); 9 had required noncoronary open heart surgery for other cardiac lesions (post-bypass group; interval from operation 5.6 +/- 3.6 years [range 1.0 to 13.25]); and 10 had had no surgical procedure (control group). The latter group comprised children with atrial or ventricular septal defects who required a radionuclide study for shunt calculation. Planar studies were performed in all 29 children, and additional tomographic acquisition was achieved in 25. To assess reversibility of perfusion defects both an exercise and a rest planar study were performed in the arterial switch group. RESULTS: Perfusion abnormalities were observed in seven of the nine children in the postbypass group and in all 10 children in the arterial switch group. The frequency of perfusion defects in these two groups was similar, with at least 25% of the tomographic segments reported being abnormal. The control group had significantly fewer defects than the other two groups (p = 0.02), with only 8% of the tomographic segments judged to be abnormal. In all except one patient in the arterial switch group, the segments reported as abnormal on the planar exercise study were either abnormal or equivocal on the rest study, indicating a fixed abnormality. CONCLUSIONS: Although the precise etiology of these perfusion abnormalities cannot be defined from this study, these data suggest that their origin is related more to the insult of open heart surgery itself than to the coronary manipulation involved in the arterial switch procedure. The functional importance requires further study.

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We analyzed the 186,102 base pairs (bp) that constitute the entire DNA genome of a highly virulent variola virus isolated from Bangladesh in 1975. The linear, double-stranded molecule has relatively small (725 bp) inverted terminal repeat (ITR) sequences containing three 69-bp direct repeat elements, a 54-bp partial repeat element, and a 105-base telomeric end-loop that can be maximally base-paired to contain 17 mismatches. Proximal to the right-end ITR sequences are another seven 69-bp elements and a 53- and a 27-bp partial element. Sequence analysis showed 187 closely spaced open reading frames specifying putative major proteins containing > or = 65 amino acids. Most of the virus proteins correspond to proteins in current databases, including 150 proteins that have > 90% identity to major gene products encoded by vaccinia virus, the smallpox vaccine. Variola virus has a group of proteins that are truncated compared with vaccinia virus counterparts and a smaller group of proteins that are elongated. The terminal regions encode several novel proteins and variants of other poxvirus proteins that potentially augment variola virus transmissibility and virulence for its only natural host, humans.

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Smallpox eradication culminated the most successful antimicrobial campaign in medical history. To characterize further the linear double-stranded DNA genome of the aetiological agent of smallpox, we have determined the entire nucleotide sequence of the highly virulent variola major virus, strain Bangladesh-1975 (VAR-BSH; 186,102 base pairs, 33.7% G + C; Genbank accession number, L22579). Here we highlight features of the molecule and focus on a few of the 187 putative proteins that probably contribute to pathogenicity and virus host-range properties. One hundred and fifty proteins were markedly similar to those of vaccinia virus (smallpox vaccine), for which a complete sequence has been reported for strain Copenhagen (VAC-CPN; 191,636 base pairs, 33.3% G + C). The remaining 37 proteins reflected variola-specific sequences or open reading frame divergences for variant proteins, which are often truncated or elongated compared with their vaccinia counterparts.

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