Lessons from a patient with Turner syndrome

2013 
### Learning Point for Clinicians Turner syndrome (TS) is associated with a reduction in life expectancy of up to 13 years compared to that of the general population, with cardiovascular disease, congenital or acquired, as the major cause of an early death. Therefore, women with TS require rigorous, long-term medical follow-up and careful monitoring for cardiovascular risk factors. Early identification of aortic root dilatation, allowing appropriate medical intervention, may prevent aortic dissection and rupture, reducing cardiovascular morbidity and mortality. A 23-year-old childcare assistant, with known TS (45XO), was referred to our cardiovascular risk clinic for hypertension (HTN) management. Reviewing her medical history, she had congestive heart failure at 1 month of age, secondary to coarctation of the aorta (CoA), for which she underwent a left subclavian flap aortoplasty. At 3 years of age, surgical correction of neck webbing was undertaken, followed by a repair of cleft palate and pharyngoplasty. She declined growth hormone replacement and started oestrogen replacement at 12 years old. Prior to referral to our clinic, the combined oral contraceptive pill (cOCP) had been stopped and she had started amlodipine 10 mg once daily. On examination her …
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