Endocrine Abstracts

Introduction: Women with Turner syndrome (TS) have a 13-year reduction in life expectancy compared to the general population. Cardiovascular disease (CVD), whether congenital or acquired, is the cause of death in around half of these women. Therefore, early identification of congenital heart defects, aortic abnormalities and risk factors for CVD is extremely important, and may have a significant impact on long-term outcomes of CVD in TS.

Aim and methods: To determine whether patients attending our paediatric Turner clinic are being appropriately screened for CVD. The audit standard was set with current available guidelines. Twenty-five patients are under regular review at the dedicated paediatric Turner clinic at the Royal Hospital for Sick Children, Edinburgh. A retrospective review of the patients’ records was performed. Cardiovascular data were recorded from three consecutive clinic visits prior to December 2012.

Results: At diagnosis, 84% of patients had an assessment by a cardiologist and 95% had echocardiography (echo). Thirty-three percent of patients had an abnormality on echo. In one patient a biscupid aortic valve could not be excluded but no further imaging was organised. Seventy-seven percent of all clinic visits had at least one BP recorded. However, 71% of these readings were in the prehypertensive or hypertensive range, requiring repeated BP measurement and confirmation by the auscultatory method (which was not performed). No patient had an assessment of aortic dimensions.

Conclusions: To improve screening for CVD at our paediatric Turner clinic we have produced an algorithm for cardiovascular imaging and a proforma for BP measurement. All patients should have an assessment of aortic valve morphology at diagnosis, regular assessment of aortic dimensions, and undergo routine BP measurement from 3 years of age. We have found that the role of the specialist nurse at clinic is vital, in helping us to adhere to these standards.