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D. S. Crossland, S. Jackson, R. Lyall, J. Burn, J. J. O’Sullivan. Department of Paediatric Cardiology, Freeman Hospital

Aims: To compare the education, career advice, and employment outcome of patients with congenital heart disease (CHD) with controls.

Methods: 299 adult CHD patients underwent a questionnaire based interview by a trained nurse. They gave the same questionnaire to a friend to act as a control, 177 controls replied.

Results: The mean school leaving age was 16.4 in both the CHD and control group. Compared with matched controls significantly more of the CHD group had time off school (48/176 (27%) v 7/176 (4%), p<0.0001) and more had gone onto educational or vocational courses (145/174 (83%) v 123/174 (71%), p = 0.004). Similar proportions of both groups received career advice while at school (84% CHD group, 80% controls). Fewer of those with CHD found their career advice helpful (26/134 (19%) v 41/134 (31%), p 0.037) and more with CHD were given advice against certain occupations (71/169 (42%) v 19/169 (11%), p<0.0001). Significantly more of the adults with CHD were unemployed than matched controls (51/156 (33%) v 25/156 (16%), p<0.0001) and unemployed for more than a year (37/151 (25%) v 5/151 (3%), p<0.0001). Proportionally more CHD patients who had received career advice were employed than CHD patients who had not (157/216 (73%) v 25/54 (46%), p = 0.0002). This was not seen in controls.

Conclusions: Children with CHD are more likely to be unemployed as adults than controls. They are less likely to find their career advice helpful and more likely to receive advice against certain occupations. Receiving career advice is associated with employment in CHD patients. There seems to be a need for helpful, positive, yet medically appropriate career advice for teenagers with CHD. Including career advisors in an adolescent or transition clinic would be an ideal forum for this.


J. Dixon, K. Foster, J. P. Wyllie, C. Wren. Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne

Introduction: Intravenous adenosine is the first line treatment for SVT in infants and children. The recommended initial dose of adenosine in the RCPCH Medicines for children is 50 μg/kg, followed by increments of 50 μg/kg. The APLS guidelines suggest incremental doses of 50, 100, and 250 μg/kg.

Method: In consecutive infants and children with SVT, we made a retrospective analysis of doses of adenosine administered in paediatric units before discussion with, or transfer to, the regional paediatric centre. We noted the sequence of doses given and their efficacy in terminating tachycardia.

Results:Infants: adenosine was given to 23 infants with 32 episodes of SVT. The initial dose ranged 50–200 μg/kg with a mean of 115 μg/kg. The first dose was 50 μg/kg in 38% of episodes. The effective dose ranged 50–500 μg/kg with a mean of 202 μg/kg. A dose of 50 μg/kg was effective in only 9% of infants and 81% needed more than 100 μg/kg to terminate SVT. Children: adenosine was given to 13 children with 21 episodes of SVT. The initial dose ranged 50–150 μg/kg with a mean of 73 μg/kg. The first dose was 50 μg/kg in 62% of episodes. The effective dose ranged 50–500 μg/kg with a mean of 150 μg/kg. A dose of 50 μg/kg was effective in only 4% of children and 60% needed more than 100 μg/kg to terminate SVT.

Conclusions: Current recommendations for the initial dose of adenosine are too low. A starting dose of 150 μg/kg is appropriate for both infants and children.


O. J. Rackham, K. Thorburn, S. J. Kerr. Paediatric Intensive Care Unit, Alder Hey Hospital, Royal Liverpool Children’s Hospital NHS Trust, Liverpool

Introduction: The aims of this study were to determine the number of infants in the Mersey and North West regions with congenital heart disease for whom palivizumab may be appropriate and to examine the potential impact of introducing prophylaxis with palivizumab on the patients and the trust.

Methods: Infants matching the population recently studied by the Cardiac Synagis Group were identified from the hospital cardiology department database. The number of patients under the care of the paediatric cardiologists admitted to Alder Hey Hospital with RSV bronchiolitis over the past three seasons was identified from hospital coding records and the cardiology department database.

Results: There are 131 eligible patients per year. Over the past three RSV seasons 39 such infants have been admitted to Alder Hey with RSV bronchiolitis. This represents a hospitalisation rate of 10%, as was seen in the Cardiac Synagis Group study. Using a monthly dose of 15 mg/kg for five doses, the cost per patient is £2650 for the season. To treat the 131 patients seen at Alder Hey would therefore cost £346 800 per year. Applying the reductions in hospital admissions seen in the Cardiac Synagis Group study to our population would produce an expected reduction in patients hospitalised from 13 to 7 per year, reducing the total ward length of stay from 169 to 76 days and paediatric intensive care unit (PICU) stay from 93 to 21 days. This amounts to a potential saving of £190 800 per year. Reducing inter-hospital transfers to more distant PICUs for referrals refused because of lack of PICU beds could save £50 000.

Discussion: We estimate the net cost of introducing palivizumab for this population to be £106 000 per year (£800 per patient treated or £17 700 per admission avoided). There would, of course, be additional costs involved in setting up this service and additional savings/benefits. This cost is comparable with other new biological therapies now routinely used such as etanercept for juvenile arthritis. There are currently no other obvious therapies that have the potential to reduce hospital and intensive care admissions during the winter months when beds are at their most scarce.


B. Krishnan, D. Calver, R. Tulloh. Departments of Paediatric Cardiology and Ophthalmology, Guy’s and St Thomas’ Hospitals NHS Trust

Introduction: Now that the survival rate for children with hypoplastic left heart syndrome (HLHS) has significantly improved, an increased incidence of eye disease has been observed. In the absence of published literature, a review of the surviving children was performed.

Methods: The notes of 60 consecutive surviving patients more than 1 year old with HLHS were reviewed. A search for attendance at ophthalmology outpatients or ward review was undertaken. The cardiology discharge letters were also inspected for mention of any concerns about the patients’ vision. An additional search was carried out on the computerised ophthalmology database and compared with the database of HLHS children.

Results: Eight of the children (13.3%) were found to have ocular disease, which is significantly more than the general population. There were five boys and three girls, ages 1–7 years old. Five had moderate myopia, one had hypermetropia, two had convergent squint, one had oculomotor apraxia and congenital saccadic initiation failure, and one had optic atrophy. Two children have had strabismus operations and one remains registered partially sighted.

Conclusion: The cause of such abnormalities may be related to the complexity of cardiac surgery with hypotension in the postoperative period for Norwood 1. Surprisingly none had ptosis or pupillary dilation, which would have been expected, considering the surgery performed on the aortic arch close to the recurrent laryngeal nerve. It may be that the venous pressure in the superior caval vein being significantly higher than normal after the hemi-Fontan operation (12 compared with 6 mm Hg) is responsible for reduced perfusion of the extraocular muscles. It is therefore important that children with HLHS should undergo routine ophthalmology review after the second operation to detect and manage ocular disease correctly.


M. Mansour, F. Schreuder, G. Morris, M. A. P. Milling. Department of Paediatrics, Singleton Hospital and Welsh Centre for Burns and Plastic Surgery, Morriston Hospital, Swansea

Aims: To define the incidence of congenital heart defects detected by routine pre-operative echocardiography in patients with cleft lip and/or palate.

Methods: Data from all patients referred for primary surgery of cleft lip and/or palate over an 18 months period from April 2001 were reviewed retrospectively.

Results: Fifteen (27%) of the 55 patients in the group had congenital heart defects, compared with a reported incidence of 10% in the literature. In three (20%) of these infants their cardiac abnormality significantly affected the surgical management of their clefts. Antibiotic prophylaxis against endocarditis was indicated in 40% of the detected cardiac lesions.

Conclusions: The use of routine pre-operative echocardiography in infants with cleft lip and/or palate is recommended to exclude significant heart defects and to provide guidance on prophylaxis against endocarditis.


C. G. Steward1, R. P. Martin2, A. M. Hayes2, A. P. Salmon4, M. M. Williams5, L. A. Tyfield5, S. J. Davies, R. A. Newbury-Ecob3. Departments of Paediatric Haematology1, Cardiology2 and Genetics3, Royal Hospital for Children, Bristol; 4Wessex Cardiothoracic Unit, Southampton; 5Molecular Genetics, Southmead Hospital, Bristol; 6Institute of Medical Genetics, Cardiff

Barth syndrome (BTHS) is an X linked disorder characterised by cardiac and skeletal myopathy, severe and cyclical neutropenia, early growth retardation, and excessive excretion of urinary organic acids (notably 3-methylglutaconic acid). Caused by mutations in the tafazzin (TAZ) gene at Xq2 leading to defective cardiolipin metabolism (L4-CL), BTHS mimics respiratory chain disease because L4-CL is a common species in mitochondria. Mortality is high in early life due to fulminant cardiac failure and/or severe sepsis and in adolescence or beyond to unexplained arrhythmia. Some pedigrees contain an excess of late foetal death.

Only 50 of affected families were documented worldwide by November 2002 and BTHS is therefore thought to be rare. We challenge this view, however, being aware of 10 unrelated families affected by BTHS in south west England and south Wales alone. All had cardiomyopathy, intermittent neutropenia, organic aciduria, and TAZ gene mutations. At least 10 males are thought to have died of the disease in recent generations of these families.

The commonest presentation is with an acute and unpredictable cardiomyopathy in the first decade, usually in infancy. This is easily mistaken for “viral cardiomyopathy”, especially as neutropenia may suggest viral bone marrow suppression. Other presentations include motor delay, myopathy, neutropenia, and recurrent sepsis. Infection, which presents a particular threat because of the extra load placed on a failing heart, may be prevented by administration of G-CSF. Hence BTHS patients may present to a number of paediatric specialities. We propose that lack of awareness of BTHS has led to underdiagnosis of the condition. The genetic implications for families and potential for treatment make early diagnosis vital. We believe that BTHS should be recognised as an important cause of sudden infant death in boys.

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