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A. Peacock, S. J. Murugan, J. M. Parsons, J. L. Gibbs.Leeds General Infirmary, Leeds, UK

Background: We noted several infants presenting to our tertiary paediatric cardiac unit with delayed diagnosis of congenital cyanotic heart disease. Failure to diagnose cyanotic heart disease in neonates is likely to be associated with significant morbidity and mortality.

Aims: To analyse the presentation of infants and children with cyanotic heart disease and examine the factors associated with late presentation.

Methods: All patients with newly diagnosed cyanotic congenital heart disease presenting to our tertiary paediatric cardiac centre at Leeds over a one year period were included in the study. Data were collected retrospectively by case note review. We noted final diagnosis, age at presentation, ethnicity, antenatal diagnosis, and whether diagnosis was missed on postnatal discharge examination and at routine general practitioner examinations at 6 weeks and at 8 months.

Results: There were 69 newly diagnosed patients with cyanotic heart disease. The median age at presentation was 2 days (range 1 day to 579 days). 31% were non-whites. Fallot’s tetrology was the most common diagnosis (33%), followed by isolated transposition of great arteries (17%). 16% of patients were diagnosed antenatally; 22% presented prior to neonatal discharge examination; 26% were detected because of concerns at discharge examination. The diagnosis of cyanotic heart disease was missed in 25 patients (36%) at neonatal discharge examination. Nearly half of them were non-whites (48%). 10 children (15%) with cyanotic heart disease were not diagnosed at the 6 week examination carried out by the general practitioner. One child was also missed at the 8 month general practitioner examination and was eventually diagnosed with tetrology of Fallot’s at 19 months.

Conclusion: There is wide variation at the time of presentation. More than one third of neonates with cyanotic heart disease were missed by routine neonatal discharge examination. This study highlights the need for improved screening tests such as pulse oximetry as an adjunct to clinical examination at the time of neonatal discharge.


C. P. H. Ibrahim, R. Johnson, A. F. Corno, M. Pozzi, D. Kitchiner.Royal Liverpool Children’s NHS Trust, Alder Hey, Liverpool, UK

Background: Epicardial pacing wires (atrial ± ventricular) are routinely placed in the majority of children undergoing intracardiac surgery at our institution. They are used for cardiac pacing or for diagnosing arrhythmias. The pacing wires are removed on the fourth postoperative day on the cardiac ward if the child is stable.

Aims: To review the use of epicardial pacing wires in the postoperative period. To determine the characteristics of children who require pacing and to assess whether these can be used to optimise the timing of wire removal.

Methods: All children undergoing cardiac surgery over a 3 month period were studied. They were classified into simple lesions (eg intra-cardiac shunts and valvular lesions) or complex heart disease for analysis.

Results: 52 children were studied (M 27:F 25). 29% of the children had only atrial wires inserted and the remaining had both atrial and ventricular wires. 14 (26%) required pacing. The significant differences between patients requiring and not requiring pacing are described in table 1. Four of the 14 paced children (8% of all cases) required pacing commencing after day 3. All four had electrolyte disturbances at the time and also at least one other listed in table 1.The median time for wire removal was the 7 days. Eight (16%) of the children had complications after wire removal. The most common was pericardial effusion of which two were treated with aspirin and the rest conservatively. One line could not be removed completely and was internalised.

Abstract G163, table 1

Conclusions: Requirement for pacing in the postoperative period is associated with longer bypass times, complex heart disease, high oxygen requirements, electrolyte and acid base abnormalities, renal failure, and inotrope use. Few children require pacing after the third day. In children without the above mentioned risk factors, the pacing wires could safely be removed on the third postoperative day, allowing for earlier discharge.


G. R. Ciotti, A. Rasheed, F. Annan, W. Blumenow, M. Murray, A. M. Dalzell.Royal Liverpool Children Hospital NHS Trust, Alder Hey, Liverpool, UK

Aims: To evaluate the importance of multidisciplinary approach in assessing patients with congenital heart disease and nutritional problems.

Methods: Over a 6 month period, 70 patients, 40 female and 30 male, have been assessed by our multidisciplinary team (gastroenterologist, cardiologist, speech and language therapist, dietician, and liaison nurse).

Results: The median age of the patients was 2.5 years (range 9 months to 8 years). Thirty five patients (50%) were cyanotic and seventeen patients (25%) had an identifiable syndrome. Forty seven patients (67%) required feeding via gastrostomy; seven (10%) required medical antireflux therapy, seven (10%) antifailure treatment, thirty five (50%) required videofluoroscopy, and seven (10%) had evidence of pulmonary aspiration. In 17 patients (24%) feeding problems were encountered after cardiac surgery. Within a period of 6 months 11 patients (15.7%) were discharged from our clinic, but seven required further cardiac follow up.

Conclusion: Nutritional and feeding problems are common in patients with congenital heart disease even after total surgical correction. Early multidisciplinary evaluation and management of feeding difficulties facilitates management. Prospective studies should be performed to evaluate the impact on feeding practices and cardiac outcome in this group of patients.


S. Alwany, M. Sirakova, M. H. El Habbal.Department of Paediatrics, Hull Royal Infirmary and Hull and York Medical School, Hull, UK

Aims: To determine whether inflammatory mediators sICAM-1 and IL8, and stem cell factor (SCF) are different between patients with secondum atrial septal defect and children with normal hearts.

Methods: We examined children with large atrial septal defect that required surgery (age range 3 to 6 years, median 4 years, n = 20; 9 males and 11 females) comparing with normal healthy children (age range 2.5 to 6.5 years, median 5 years, n = 20; 9 males and 11 females). All patients and controls had echocardiography and Doppler examinations. We measured serum sICAM-1, IL8, and SCF using ELISA.

Results: All patients had isolated atrial septal defect measuring 15–23 mm in diameter (median 20 mm). All controls had normal hearts and intact inter-atrial septa. Levels of serum sICAM-1 ranged between 49 to 346 ng/ml (median 203 ng/ml) in patients and 150 to 246 ng/ml (median 198 ng/ml) in controls; p 0.53. Serum IL8 was higher in patients than controls (4 to 75 pg/ml, median 30 pg/ml v 5 to 34 pg/ml, median 17 pg/ml; p 0.004). Levels of serum SCF were similar in both patients and controls 636 to 1050 pg/ml, median 822 pg/ml v 538 to 1034 pg/ml, median 792 pg/ml; p 0.5). When correlating levels of IL8 to SCF and sICAM-1 patients had low correlation coefficients compared to controls (for correlating IL8 to SCF, r = 0.51 in patients vr = 0.75 in controls; for IL8 to sICAM-1, r = 0.45 in patients vr = 0.85 in controls, for sICAM-1 to SCF, r = 0.49 in patients vr = 0.82 in controls; p<0.001).

Conclusion: In children with secundum atrial septal defect, serum levels of IL8 are elevated. The interactions between IL8, sICAM-1, and SCF are inadequate, which may account for persistence of the defects.


J. V. DeGiovanni1, P. Miller1, S. Shebani1, S. A. Thorne2, P. Clift2, M. Togeig3, A. Duke3, R. Patel4.1Birmingham Children’s Hospital, Birmingham, UK; 2University Hospital Birmingham, UK; 3Glenfield Hospital, Leicester, UK; 4Manchester Royal Infirmary, Manchester, UK

Aim: This is a multicentre study designed to describe our experience and results of closing ventricular septal defects using the Amplatzer devices (AGA Medical Corporation, Minnesota USA).

Methods: Between September 2001 and November 2004, 61 patients with a ventricular septal defect were considered for transcatheter closure. The indications included heart failure, left ventricular volume overload, haemolysis, history of endocarditis, and career reasons. The lower age considered acceptable for a transcatheter procedure as opposed to surgery was 8 kg. The procedure time, fluoroscopy time, and patient demographics were recorded. Evaluation of the ventricular septal defect was carried out on transthoracic and transoesophageal echocardiography as well as angiography and the procedures were carried out under general anaesthesia.

Results: There were 39 with perimembranous ventricular septal defect, of whom 32 received an Amplatzer perimembranous device and six an Amplatzer muscular device; one was abandoned due to transient heart block during sheath insertion. Of the muscular congenital ventricular septal defects, five were in the outlet septum, six mid-muscular, and four muscular inlet; all these received a muscular Amplatzer ventricular septal defects device. Four had a residual post-surgery ventricular septal defect and three developed a ventricular septal defect following trauma and all received muscular Amplatzer ventricular septal defect devices; in one of the trauma cases, the procedure failed as the ventricular septal defect was too large even for the largest device available.

Results: A device was successfully implanted in 59 patients and failure in two was due to heart block (one) and a very large ventricular septal defectsin another (one). There were no patients with persistent arrhythmias or heart block, no device embolisations, and no deaths. Residual shunts, albeit small, have been detected in the perimembranous but not in the muscular ventricular septal defects following device closure. In one patient with perimembranous ventricular septal defect, aortic regurgitation developed due to device encroachment on the aortic leaflets but none developed significant tricuspid regurgitation. Follow up included clinical examination, ECG, and echocardiography. There were no vascular thromboembolic events during or after the procedure and all patients received antiplatelet drugs for 6 months. The majority were discharged within 24 hours of the procedure.

Conclusion: Transcatheter closure of congenital ventricular septal defects in various locations can be closed safely and effectively using an Amplatzer ventricular septal defect occluder of a type appropriate to the position of the defect. The results are encouraging but long term follow up is essential to establish whether late problems may arise particularly with regards to the tricuspid and aortic valves as well as the AV node.


M. Walsh, D. Coleman, D. Duff, P. Oslizlok, K. Walsh.1University College Dublin, Dublin, Ireland

Aims: The aim of our study is to look at a patient group that has had a transcatheter Amplatzer asymmetric occlusion devices inserted, for perimembranous ventricular septal defects, to look at any possible adverse events and to examine the success rate for the procedure.

Methods: We collected data on all patients having a PMVSD occlusion device inserted from between October 2002 and October 2004. We looked at the indication for PMVSD closure, any perioperative or post-operative rhythm disturbances, and any complications following device closure.

Results: There were a total of 24 patients who had PMVSD closures. In 13 cases the indication for closure was significant left to right shunting, in 10 cases it was due to patient symptoms and in one case the reason for closure was an episode of previous endocarditis. All except two patients had a hospital stay of 2 days. The youngest patient having a closure was 16 months with a weight of 8.2 kg. Eleven patients had no other abnormalities apart from a PMVSD, and one patient had a trisomy 21. Two patients had a patent ductus arteriosus closed at the same time. The Amplatzer device was deployed on first attempt in all except one case, and was successfully redeployed in this case. There was mild aortic regurgitation following the procedure in four cases. Six patients had residual left to right shunting on day 1, four of which had resolved by 6 weeks. One patient needed a repeat procedure 19 months following the original closure. Two patients developed partial right bundle branch block (RBBB), and one patient developed partial left bundle branch block. One patient developed a postoperative sinus tachycardia with RBBB, requiring treatment with digoxin. One of our patients developed complete heart block, presenting with syncope 5 days later. She required a permanent pacemaker which she no longer uses.

Conclusion: Transcatheter closure is a safe and effective alternative to the standard surgical treatment in selected children with PMVSDs. There remains a question over whether small defects without any left ventricular volume overload should be closed. This decision involved weighing up the long term risk of endocarditis against any possible complications that may occur as a consequence of the procedure. We have shown a 93% success rate for closure with minimal morbidity when compared with surgical closure. There is also a considerable reduction in the cost of treating these patients, and given that most patients can return to the ward, less demand on intensive care beds when compared with a surgical closure.


A. W. R. Kelsall, D. K. White.Addenbrookes NHS Trust, Cambridge, UK

Aims: To establish the number of neonatal patent ductus arteriosus ligations performed in the UK in 2002, identify where the procedure was undertaken and determine the 30 day survival.

Methods: A retrospective postal survey was performed. Questionnaires were sent to the lead paediatrician in every hospital with a special care/neonatal intensive care unit for information on the number of infants referred for patent ductus arteriosus ligation, where the procedure was performed, and outcome. A separate questionnaire was sent to the paediatric cardiothoracic centres for information on neonates referred in for patent ductus arteriosus ligation. Cross referencing the responses identified neonates from units where there had been no formal response. Additional information was requested from the central cardiac audit database.

Results: There was a 71% response rate with 165 replies from 234 questionnaires sent out. Paediatricians from 105 hospitals indicated that no neonates had been referred for patent ductus arteriosus ligation, 12 units of varying sizes did not have a database and could not provide any information. Data were available from seven cardiothoracic centres. Responses indicate that 185 neonates underwent patent ductus arteriosus ligation in 14 centres in the UK. The numbers of ligations performed in centres ranged between 1 and 44. Two units that were not recognised paediatric cardiothoracic centres performed nine ligations, both no longer undertake the procedure. Outcome is known for 145 (78%) of the infants: there were 133 (72%) survivors and 12 (6%) deaths at 30 days, the outcome for 40 (22%) babies was unknown.

Conclusions: Despite major difficulties with case ascertainment the 30 day survival in this national survey is in line with other presented series. The numbers of patent ductus arteriosus ligations identified in this study are almost certainly an underestimate. Given that there is known to be a high late mortality in this group of infants a prospective study with longer term follow up is required.


S. Allan, A. Houston, T. Richens.Yorkhill NHS Trust, Glasgow, UK

Introduction: Over the past 4 years we have administered intravenous inotropes in a cardiology ward setting in selected patients with chronic inotrope dependency. Most of these patients have dilated cardiomyopathy. This study reviews the safety profile of inotropes in a carefully monitored ward setting.

Methods: The case notes of all patients who received inotropes on the ward in a 4 year period were reviewed, documenting adverse events and outcomes.

Results: Fourteen patients (9 female, 5 male) aged 1 day to 14 years (median 18 months) received intravenous inotropes. Ward inotrope therapy was given for a total of 309 days. In 10 patients inotropes were administered by peripheral line only, in one by central line only, and in three by a combination of routes. Therapy was commenced in PICU on six occasions and on the ward on 10 occasions. Eight patients received a combination of dobutamine and milrinone, two had milrinone only, and two had dobutamine only. Transfer from ward to ITU was necessary in seven patients with one child requiring several transfers.

Outcome: Of the two children that died, one suffered a bradycardia mediated cardiac arrest on the ward (no detectable problem with inotrope delivery). The second child died on intensive care waiting for a cardiac transplant (see table 1).

Abstract G163, table 2

Abstracts G163, table 3. Complications after wire removal

Abstract G169, table 1

Adverse events: Interruption during changeover of infusion was in a patient on a dobutamine infusion. She rapidly deteriorated haemodynamically and required intensive care. She eventually received a successful cardiac transplant (see table 2).

Abstract G169, table 2

Conclusion: Under carefully controlled conditions, IV inotropes can be safely administered in a ward setting. Using an inotrope with a longer half life such as milrinone, may improve safety during infusion changeover. Issues regarding training and education of staff, both nursing and medical, must be established to allow the continued use of inotropes in a safe environment.