ESPE Abstracts (2015) 84 P-3-1234

ESPE2015 Poster Category 3 Turner (14 abstracts)

Cardiovascular Assessment in Turner Syndrome: Current Practice in the United Kingdom

A Mason a , A Smyth b , S F Ahmed a & S C Wong a


aDevelopmental Endocrinology Research Group, Royal Hospital for Sick Children, Glasgow, UK; bTurner Syndrome Society United Kingom, Glasgow, UK


Background: In 2007, the Turner syndrome (TS) Consensus Study Group developed an international guideline for clinical care of girls and women with TS. Given emerging concerns of long term cardiovascular complications, the consensus recommends that cardiac MRI should be performed when girls are old enough to tolerate the procedure or at the time of transition and to be repeated at least every 5–10 years.

Method: We conducted a survey of cardiovascular (CVS) assessment in girls and women with TS in all tertiary paediatric endocrinology centres and all adult centres with dedicated TS clinical service in the UK.

Results: An online survey was sent to 49 consultants (20 paediatric, 29 adult). There were 26/49 (53%) responders.13/26 (50%) provided care in childhood. At diagnosis of TS, echo (9/12,75%) or echo & MRI (3/12,25%) were performed. In adolescence, echo (6/13, 46%) or MRI (3/13, 23%) were performed for CVS re-evaluation. However, 4/13 (31%) were not re-evaluated in paediatric care. Median age of re-evaluation was 16 years (range 10–16) or at the time of transition. In adulthood, echo & MRI (10/13, 77%), MRI (2/13, 15%) and echo (1/13) were performed respectively at frequency of 5 years or less. Aortic sized index was provided in imaging reports of 5/10 (50%) and 13/13 of paediatric and adult responders respectively. Blood pressure was measured in the paediatric clinic: annually 3/12 (25%), 6 monthly 6/12 (50%) and 3–4 monthly 3/12 (25%), whereas this was measured in the adult clinic: annually 10/13 (77%), 6 monthly 2/13 (15%) and at every clinic 1/13. Cardiovascular risk is discussed by the primary treating paediatrician in 7/11 (64%) and by the primary treating adult physician in 12/13 (92%). Written information on cardiovascular risks is provided in 3/10 (30%) and 2/12 (17%) of paediatric and adult clinics respectively. In high risk patients, a recommendation to carry medical bracelet/card is provided by 2/10 (20%) and 2/12 (17%) of paediatric and adult clinics respectively.

Conclusion: Despite the existing consensus, this survey, of clinicians providing care to individuals with TS in the UK, demonstrates wide variation in cardiovascular assessment especially in adolescence. This variability may relate to access to local expertise and specialist investigations. Uncertainties surrounding the value of investigations to clinical outcome of aortic dissection especially in childhood may also be a factor.

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