Body mass index has risen more steeply in tall than in short 3-year olds: serial cross-sectional surveys 1988-2003.

OBJECTIVE: To monitor the changing relationship between body mass index (BMI) and height in young children. DESIGN: Annual cross-sectional surveys using health-visitor-collected routine data 1988-2003. SETTING: Wirral, England. PARTICIPANTS: Fifty thousand four hundred and fifty-five children (49% female) each measured once at the age of 3 years. MAIN OUTCOME MEASURES: Weight, height and derived BMI (weight/height(2)) adjusted for age and sex (British 1990 revised reference) using standard deviation scores. RESULTS: From 1988 to 2003, mean BMI increased by 0.7 kg/m(2), whereas mean height fell by 0.5 cm. Over the same period, the weight-height correlation rose from 0.59 to 0.71 (P<0.0001) owing to BMI increasing faster in the taller than the shorter children. Among the shortest 10% of children, mean BMI rose by 0.12 (95% confidence interval: -0.05-0.28) kg/m(2) as against 1.38 (1.19-1.56) kg/m(2) among the tallest 10%, a 12-fold difference. Adjustment for age, sex, seasonality, birth-weight and deprivation did not alter the findings. CONCLUSIONS: Among 3-year-old children in Wirral, where BMI has been rising for 16 years, the largest increase in BMI has occurred in the tallest children, whereas in the shortest BMI has hardly changed. Tall stature has, therefore, become important for child obesity. It suggests a drive to increasing adiposity in young children that involves both growth and appetite, with fast growing and hungrier children now more exposed to the 'obesogenic' environment.

Evaluation of echocardiography on the neonatal unit.

BACKGROUND: Echocardiography is an investigation that is being used increasingly on the neonatal unit. There is some controversy as to whether this service can be provided safely and effectively by neonatologists or whether it should only be performed by paediatric cardiologists. AIMS: To describe (a) the indications for an echocardiogram, (b) the yield and range of positive findings, (c) the resulting changes in clinical management, and (d) the reliability of echocardiography in the hands of neonatologists when it is performed on the neonatal unit. METHODS: Information about all echocardiograms performed on the neonatal unit was collected prospectively. Indications for performing echocardiography, echocardiographic findings, and any resulting changes in clinical management were determined. The concordance of findings in infants who underwent echocardiograms performed by both a neonatologist and a paediatric cardiologist was described. RESULTS: A total of 157 echocardiograms were performed in 82 infants. Echocardiography identified 44 infants with a structural cardiac abnormality and a further 17 infants with a trivial abnormality. In addition, 13 babies were found to have an important functional abnormality. Echocardiography prompted a specific change in clinical management in 64 (78%) babies. In 31 of the 38 infants who had paired scans performed, there was complete concordance between the two examinations. No infants had scans that were completely different. Some discrepancy was identified in seven infants, but this did not prevent appropriate immediate clinical management. CONCLUSIONS: Echocardiography on the neonatal unit has a high yield for the diagnosis of structural and functional cardiac abnormalities, often results in a change in clinical management, and can be a reliable tool in the hands of neonatologists.

Prevalence of overweight and obese children between 1989 and 1998: population based series of cross sectional studies.

OBJECTIVE: To determine trends in weight, height, and body mass index in children between 1989 and 1998. DESIGN: Retrospective series of cross sectional studies of routinely collected data. SETTING: Primary care in the Wirral Health Authority. PARTICIPANTS: 35 662 infants aged 1-3 months (representing 88% of live births) and 28 768 children aged 2.9-4.0 years. 21 582 infants and children (25.1%) were excluded because of missing or inaccurate data. MAIN OUTCOME MEASURES: Weight, height, sex, and age routinely recorded by health visitors. Height, weight, and body mass index standardised for age and sex. SD score >1.04 for body mass index (>85th centile) was defined as overweight and >1.64 (>95th centile) as obese. Body mass index was not calculated in infants as it is difficult to interpret. RESULTS: From 1989 to 1998 there was a highly significant increasing trend in the proportion of overweight children (14.7% to 23.6%; P<0.001) and obese children (5.4% to 9.2%; P<0.001). There was also a highly significant increasing trend in the mean SD score for weight (0.05 to 0.29; P<0.001) and body mass index (-0.15 to 0.31; P<0.001) but not height. Infants showed a small but significantly increasing trend in mean SD score for weight (-0.17 to -0.05; P=0.005). CONCLUSIONS: From 1989 to 1998 there was a highly significant increase in weight and body mass index in children under 4 years of age. Routinely collected data are valuable in identifying anthropometric trends in populations.

Mechanical occlusion of the patent ductus arteriosus with Jackson coils.

The effectiveness and safety of a protocol for transcatheter patent ductus arteriosus (PDA) closure was assessed. Our goal is complete mechanical occlusion of the PDA in the catheterization laboratory by adding coils until it is no longer possible to cross the PDA with a guidewire. Detachable coil closure of a PDA with a narrowest diameter of 2.4 +/- 0.1 mm was attempted in 83 patients with a median age of 2.8 years (0.7 to 27.8 years) and whose median weight was 14.5 kg (6 to 61.6 kg). Coils were successfully implanted in 82 of 83 patients, and in 1 patient a large Rashkind double umbrella was used instead. Complete closure was obtained in 80 (97.6%) patients, 48 of those (59%) received more than one coil. Reintervention for residual shunting was required in only 1 patient and another patient has a trivial residual shunt. Device embolization occurred in three cases. Despite the use of multiple coils there was no evidence of significant left pulmonary artery stenosis. The fluoroscopy time increased from 14.0 +/- 2.0 minutes for a single coil to 25.3 +/- 2.9 minutes for multiple coils (p < 0.01). Attempting to obtain complete mechanical occlusion of the PDA during the implant procedure by adding extra coils reduces the need for reintervention for residual or recurrent shunting.

Tetralogy of Fallot: what operation, at which age.

BACKGROUND: The optimal management of tetralogy of Fallot is still under debate, particularly with respect to surgical approach and the age of operation. In recent times a transatrial-transpulmonary approach and primary repair in younger patients is favoured. The purpose of the present study was to analyze the result of our current surgical management by assessing the perioperative and intermediate term follow up in order to define the optimal strategy and timing of operation for our institution. METHODS: One hundred and thirty two patients with tetralogy of Fallot who underwent definitive repair between May 1993 and December 1998 were analyzed by reviewing their medical records and follow-up. Median age was 15. 5 (2.3-68.6) months and median weight was 8.8 (5-16) kg. Ten (7.57%) patients were under 6 months, 38 (28.78%) were between 6 and 12 months, 36 (27.27%) were between 12 and 18 months, 23 (17.42%) were between 18 and 24 months and 25 (18.93%) were more than 24 months age. During the study period there was a move to earlier surgery and differing methods of repair depending on the anatomy observed. Follow up was conducted by the referring cardiologist. Median follow up was 35.48 (8.07-74.93) months. RESULTS: Forty-two (31.8%) patients required a palliative procedure before total correction due to unfavourable anatomy. Subpulmonary infundibular obstruction with a fibrous component increased significantly with age (P<0.05). Operations were entirely transatrial in 14 (10.6%), transatrial and transpulmonary in 69 (52.2%), transatrial and transventricularly in 42 (31.8%) and a homograft conduit was used in seven (5.3%) patients. Younger patients had narrower pulmonary valves and required a transannular patch more frequently. All patients were in sinus rhythm, 28 (21.1%) showing right bundle branch block. Median hospital stay was 8 (5-54) days. No patient required reintervention during follow up and there was no early or late mortality. CONCLUSION: Correction of tetralogy of Fallot at younger age does not increase morbidity or mortality and has potential advantages. A surgical technique adapted to the anatomy of the right ventricular outflow tract, achieves the best results.

Comparison of the Sideris and Amplatzer septal occlusion devices.

The results of transcatheter atrial septal defect (ASD) occlusion with 2 different devices (Sideris adjustable buttoned device vs Amplatzer Septal Occluder) were compared in 2 consecutive series of patients. Comparative outcomes were assessed by whether a device was implanted or not, by complications and fluoroscopy time of implantation, and by the incidence of residual shunting on transthoracic echocardiography at follow-up. The patient and defect characteristics were similar in both groups. Twenty-eight of 33 Sideris devices and 37 of 39 Amplatzer devices were implanted. The fluoroscopy time for the Amplatzer implants was 13.4 minutes (range 8 to 41) compared with 23.7 minutes (range 11 to 60.6) for the Sideris implants (p <0.001). The complete occlusion rate for the Amplatzer device was 93% compared with 44% for the Sideris device at 1 year (p <0.001). In conclusion, the Amplatzer device produces higher occlusion rates of ASDs with shorter fluoroscopy times.

Balloon expandable stents for systemic venous pathway stenosis late after Mustard's operation.

OBJECTIVES: Description and evaluation of current experience with the use of balloon expandable stents for the relief of systemic venous pathway stenosis late after Mustard's operation. DESIGN: Retrospective observational study of technical procedures, angiographic, and haemodynamic findings. PATIENTS: Twenty long term survivors of Mustard's operation for transposition of the great arteries (TGA) with angiographic evidence of systemic venous pathway narrowing. INTERVENTION: Systemic venous pathway stenoses were stented using balloon expandable Palmaz stents. RESULTS: Twenty seven stents were deployed across 24 stenoses. Seventeen stents were placed in the inferior baffle (16 patients), with an increase in mean (range) minimum diameter from 9.6 (4.5-15.9) to 16.5 (11.9-22.2) mm (p = 0.007), and a reduction in mean pressure gradient from 3.1 (0-8) to 0.67 (0-3) mm Hg (p = 0.002). Eight stents were placed in the superior pathways of eight patients, with diameters widened from 9.1 (3.5-14.1) to 15.2 (8.7-19.2) mm (p = 0.018), and gradients reduced from 6.4 (2-11) to 0.9 (0-2) mm Hg (p = 0.02). Two badly deployed stents were safely withdrawn from their intracardiac positions and redeployed in the iliac vein. Transvenous pacemaker insertion was facilitated by prior stent insertion. CONCLUSIONS: The use of balloon expandable stents for late systemic pathway narrowing after Mustard's operation is safe and effective. The beneficial effects of stenting are likely to be more durable than those of balloon angioplasty alone, but longer term follow up is required.

Transcatheter occlusion of the patent ductus arteriosus with Cook detachable coils.

OBJECTIVE: To report initial experience with a new occlusion device for native and residual patent ductus arteriosus. DESIGN: Descriptive study of consecutive non-randomised patients undergoing a new method of patent ductus arteriosus closure with detachable coils. SETTING: Tertiary centres for paediatric cardiology. PATIENTS: 71 consecutive patients, aged 1.2-22 years, with a patent ductus arteriosus (PDA) underwent elective transcatheter closure. 45 had native PDAs (group A) with a minimum diameter of 1.0 mm-5.0 mm (median 2.0 mm). A further 26 had undergone one or more previous occlusion attempts (group B). INTERVENTIONS: A total of 133 detachable (Cook) spring coils were successfully implanted in 70 patients. The procedure was performed transvenously in 51 patients, retrograde arterially in 13, and by both routes in a further 6 patients. One 5 mm coil migrated but was successfully retrieved. MAIN OUTCOME MEASURES: In group A colour flow Doppler echocardiography showed that complete occlusion was achieved in 40/45 (89%) at 24 hours, 41/45 (91%) at 1 month, and 44/45 (98%) by 6 months post procedure. Occlusion rates in residual PDAs were 22/25 (88%) occluded at 24 hours, 23/25 (92%) at 1 month, and 24/25 (96%) at 6 months follow up. CONCLUSIONS: Transcatheter occlusion using detachable (Cook) spring coils is a safe and effective alternative to presently available devices. The delivery system allows full retrieval of the coil until a satisfactory position is obtained.

Prognosis of supravalve aortic stenosis in 81 patients in Liverpool (1960-1993).

OBJECTIVE: To determine the prognosis of supravalve aortic stenosis into early adult life and the factors affecting this prognosis. DESIGN: 81 patients with supravalve aortic stenosis were followed for a median duration of 8.3 (range 1 to 29) years. PATIENTS: 40 patients (49.4%) had Williams' syndrome, 18 (22.2%) familial supravalve aortic stenosis, 18 (22.2%) sporadic supravalve aortic stenosis, and five (6.2%) other syndromes. Nineteen patients had additional levels of left ventricular outflow tract obstruction. RESULTS: 47 patients (58%) underwent operation; 20% within a year of presentation. Multivariable analysis predicted that 88% of patients would undergo intervention within 30 years of follow up. The chance of intervention was increased by more severe aortic stenosis at presentation and the presence of multilevel obstruction in patients with sporadic supravalve aortic stenosis. Three deaths occurred before operation and 13 within a month of operation. Ten (62.5%) of the postoperative deaths were in patients with multilevel obstruction. Predicted survival 30 years after presentation was 66%. Risk factors for survival were age and severity of aortic stenosis at presentation. Multilevel obstruction did not emerge as a significant risk factor for death because of the high association with the severity of stenosis at presentation. 74% of survivors had mild or insignificant stenosis at follow up. CONCLUSIONS: Long-term survival is related to age and the severity of aortic stenosis at presentation. Most patients will require intervention, and most survivors will have mild stenosis.

Integrated care pathways: effective tools for continuous evaluation of clinical practice.

The critical examination of clinical practice should be an integral part of patient care. It includes the development and implementation of guidelines, together with continuous evaluation of clinical process and outcomes to improve the quality of care provided. Clinical audit has not been successful in achieving this. The use of Integrated Care Pathways facilitates the introduction of guidelines and the continuous evaluation of clinical practice. Improvements are achieved by frequently revising the pathways to reflect current, local best practice. Integrated Care Pathways define the expected course of events in the care of a patient with a particular condition, within a set time-scale. A pathway is divided into time intervals during which specific goals and expected progress are defined, together with appropriate investigations and treatment. A pathway reflects the activities of a multidisciplinary team and can incorporate established guidelines and evidence-based medicine. It is usually unique to the institution in which it was developed. The pathway forms part of the clinical record of every patient. All variations from the pathway are documented, and the reasons for the variations analysed. Solutions are developed to address the causes of potentially avoidable variation, and the pathway is revised to incorporate these improvements. Integrated Care Pathways provide a powerful audit tool, as all aspects of the process and outcome of clinical practice can be constantly monitored. Variations from set standards are minimized, and improvements are rapidly incorporated into routine practice and subsequently re-evaluated.

Morphology of left ventricular outflow tract structures in patients with subaortic stenosis and a ventricular septal defect.

OBJECTIVE: To compare the incidence and prognosis of subaortic stenosis associated with a ventricular septal defect and to define the morphological basis of subaortic stenosis. DESIGN: Presentation and follow up data on 202 patients with subaortic stenosis seen at the Royal Liverpool Children's Hospital between 1 January 1960 and 31 December 1991 were reviewed. Survivors were traced to assess their current clinical state. Necropsy specimens of 291 patients with lesions associated with subaortic stenosis were also examined. RESULTS: In the clinical study; 65 (32.1%) of the 202 patients with subaortic stenosis had a ventricular septal defect (excluding an atrioventricular septal defect). 32 of these patients had a short segment (fibromuscular) subaortic stenosis. 33 had subaortic stenosis produced by deviation of muscular components of the outflow tracts. In 17 patients (51.5%) this was caused by posterior deviation or extension of structures into the left ventricular outflow tract, resulting in obstruction above the ventricular septal defect. In the other 16 patients (48.5%) there was over-riding of the aorta with concordant ventriculoarterial connections, (without compromise to right ventricular outflow) producing subaortic stenosis below the ventricular septal defect. Additional fibrous obstruction occurred in 39% of the patients with deviated structures. The age at presentation was lower (P < 0.01) in patients with deviated structures (median (range) 0.4 (0 to 9.2) months) than in those with short segment obstruction (median (range) 4.2 (0 to 84.9) months). The incidence of aortic arch obstruction was higher (P < 0.002) in patients with deviated structures than in those with short segment obstruction (38%). In the morphological study 35 pathological specimens showed obstructive muscular structures in the left ventricular outflow tract either above or below the ventricular septal defect. 16 had either posterior deviation of the outlet septum or extension of the right ventriculoinfundibular fold, or both of these together into the left ventricle. 19 had anterior deviation of the outlet septum into the right ventricle with overriding of the aorta (without compromise to right ventricular outflow). The earliest age at which additional fibrous obstruction was seen was 9 months. The aortic valve circumference was small in 18% of specimens. FOLLOW UP: The median (range) duration of follow up in survivors from the clinical study was 6.6 (1 to 25.7) years. 16 patients with deviated musculature (49%) and 16 with short segment fibromuscular stenosis (50%) underwent operation for subaortic stenosis. Patients with deviated structures were younger at operation than those with short segment stenosis (P < 0.005). Patients with posterior deviation or extension of structures into the left ventricular outflow tract underwent operation for subaortic stenosis more frequently (P < 0.05) than those with anterior deviation of the outlet septum and aortic override. The ventricular septal defect required surgical closure more frequently (P < 0.005) in patients with deviation (93.9%) than in those with short segment obstruction (21.9%). There was no significant difference in the mortality between patients with deviation (27%) and those with short segment obstruction (12%). CONCLUSIONS: 32% of patients in the clinical study with subaortic stenosis had a ventricular septal defect. Only 51% of these had obstructive and deviated muscular structures in the left ventricular outflow tract. These patients had a significantly higher incidence of aortic arch obstruction and required surgery for subaortic stenosis at a younger age than those with short segment obstruction. The ventricular septal defect also required surgical closure more frequently in those patients with deviation. The morphological study defined the two sites of obstruction. The presence or absence and type of deviation should be clearly defined in all patients with a ventricular septal defect,

Balloon dilatation of the aortic valve after previous surgical valvotomy: immediate and follow up results.

OBJECTIVE: To evaluate the immediate and long-term results of transcatheter balloon dilatation of the aortic valve for restenosis after previous surgical valvotomy. DESIGN: Prospective follow up by clinical examination and cross sectional Doppler echocardiography of all patients fulfilling the above criteria. SETTING: Tertiary paediatric cardiology referral centre. PATIENTS AND METHODS: 22 patients (18 male, 4 female)--median (range) age 157.5 (12-254) months--underwent 25 balloon dilatation procedures at a median of 72 (8-155) months after surgery. The median age at surgical valvotomy was 82.5 (0.5-230) months and the systolic gradient across the aortic valve immediately after surgery was 31 (0-49) mm Hg. The indication for dilatation was a Doppler derived peak instantaneous gradient of > 60 mm Hg with grade 2 or less aortic regurgitation. A single balloon was used, and the median balloon to annulus ratio was 1 (0.9-1). RESULTS: After dilatation the catheter pullback gradient decreased acutely from 55 (35-75) to 30 (0-75) mm Hg (p < 0.01) and the Doppler gradient from 74 (52-92) to 40.5 (30-96) mm Hg (p < 0.01). In three patients who underwent a second dilatation of the aortic valve eight months after the first procedure, the pullback gradient decreased from 50 (50-60) to 15 (15-16) mm Hg. Aortic regurgitation grade increased from 1 (0-2) to 2 (1-3); only one patient had grade 3 regurgitation. Over a median follow up of 33 (2-67) months seven patients had aortic valve replacement for recurrent stenosis (six patients) or severe regurgitation (one patient with grade 3 regurgitation after dilatation, who had partial detachment of one of the valve leaflets). There was no significant difference for the pullback gradient (median of 19 v 32.5 mm Hg), Doppler gradient 24 hours after dilatation (33.5 v 50.5 mm Hg; p = 0.03), or the duration of follow up (27.5 v 18 months) between the 12 patients who did not require further dilatation or surgery and the 10 patients who did. CONCLUSIONS: Balloon dilatation of the aortic valve is a safe and feasible option for palliation of restenosis after surgical valvotomy for congenital aortic valve stenosis. In many patients, however, stenosis progressed and a further intervention was required.

Incidence and prognosis of obstruction of the left ventricular outflow tract in Liverpool (1960-91): a study of 313 patients.

OBJECTIVE: To determine the incidence of the various types of obstruction of the left ventricular outflow tract in patients born in the five health districts of Liverpool and to compare their prognosis into early adult life. DESIGN: Notes of all patients with obstruction of the left ventricular outflow tract born in the study area between 1960 and 1991 were reviewed. Patients with hypoplastic left ventricle, mitral valve atresia, and those with discordant atrioventricular or ventriculoarterial connections were excluded. Survivors were traced and assessed clinically; eight were lost to follow up. RESULTS: Obstruction of the left ventricular outflow tract occurred in 313 patients (67% male), giving an incidence of 6.1/10,000 live births. The median (range) age at presentation was 13.9 months (0-20 yr). Aortic valve stenosis occurred in 71.2%: subvalve in 13.7%, supravalve in 7.7%, and multilevel in 7.4%. The median (range) duration of follow up was 10.0 (1-29) yr. Aortic regurgitation at presentation occurred more often (p < 0.001) in patients with subvalve stenosis than in those with other types of obstruction, but there was an increased incidence (p < 0.001) at follow up in patients with valve stenosis. Ninety eight patients (31.3%) underwent operation. The reoperation rate was 27% for valve stenosis and 9% for subvalve obstruction. No patients with supravalve stenosis underwent reoperation. The median duration from first operation to aortic valve replacement (17 patients) was 12.3 years. Hazard analysis confirmed that the risk of death was higher in patients presenting at a younger age, with more severe stenosis, and those with subaortic, multilevel obstruction or a syndrome. Hazard analysis also showed that the risk of a clinical event (surgery, balloon dilatation, or endocarditis) was greater in patients who presented at a younger age, with more severe stenosis or aortic regurgitation, and in those with subvalve or multilevel obstruction. CONCLUSIONS: Aortic valve stenosis was the most common type of obstruction. Hazard analysis indicates that the age and severity of obstruction at presentation have a significant effect on survival and freedom from a clinical event. The risk of premature death in patients presenting with moderately severe valve stenosis is reasonably small, but increases considerably in those with subvalve, supravalve, and multilevel obstruction. Patients who present with mild valve stenosis have a good prognosis. The risk of sudden death is less than previous predictions. Patients with subvalve and multilevel obstruction, even when mild at presentation, are more likely to undergo intervention or develop endocarditis than those with valve or supravalve stenosis. Follow up into adult life is essential.

The progression of mild congenital aortic valve stenosis from childhood into adult life.

UNLABELLED: We studied 187 patients who presented with mild congenital aortic valve stenosis or a bicuspid aortic valve without stenosis at presentation; 63% were males. Information on all clinical events was obtained, and patients were traced to assess current clinical status. RESULTS: The median age at presentation was 2 years (range, 0-15). Additional cardiac lesions occurred in 51 patients, more commonly in patients presenting under 1 year of age (P < 0.0001). The median duration of follow-up was 10 years (range, 1-28); seven patients were lost to follow-up. Thirty-two patients progressed to require intervention (28 surgical, five balloon valvuloplasty) at a median age of 10.5 years. No patient who presented with a bicuspid aortic valve required intervention. Two patients developed endocarditis. There were eight deaths; four after surgery for aortic stenosis and four due to other cardiac lesions. There were no sudden deaths. Actuarial and hazard analysis showed that progression beyond mild stenosis was closely related to duration of follow-up. CONCLUSIONS: Congenital aortic valve stenosis is most frequently mild at presentation. Progression is related to duration of follow-up. Fewer than 20% of patients are likely to still have mild stenosis after 30 years. Follow-up into adult life is essential.