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Endocrine Abstracts (2014) 34 CMW4.1 | DOI: 10.1530/endoabs.34.CMW4.1

School of Medicine, Glasgow University, Glasgow, UK.


The cardiac problems encountered in girls and women with Turner’s syndrome include: congenital anomalies such as biscuspid aortic valve (30%) which is a risk factor for aortic stenosis and for future aortic rupture, coarctation of the aorta (12%), persistent left superior vena cava (13%) and partial anomalous pulmonary venous return (13%); a tendency towards hypertension (seen in 25% of adolescents) which appears essential in nature and which is not correlated with alterations in renal morphology (such as duplex and horsheshoe kidneys); aortic root dilation which may proceed to aortic rupture or dissection; and an increased incidence of atherosclerosis.

A thorough clinical examination of the cardiovascular systems at the time of diagnosis is essential, supplemented by an echocardiogram. Since preschool girls may be fearful, and hence difficult to assess, echocardiography should be repeated at the time of school entry.

Cardiovascular risk should be reduced by optimising blood pressure (BP) and preventing obesity during the childhood and adolescent years. BP should be measured with Dinamap annually up to school entry and then at each clinic visit. If systolic and/or diastolic blood pressure is >98th centile on more than one occasion then 24-h ambulatory blood pressure monitoring is indicated, to distinguish between labile hypertension related to anxiety; and sustained hypertension with failure of nocturnal dipping, which may need antihypertensive treatment (e.g. with Ca++ channel or β blockers). BP status may also be improved by using physiological induction and maintenance of puberty with transdermal rather than oral oestrogen.

At time of transfer to an adult endocrine or gynaecology clinic the heart and great vessels should be reassessed using MRI, which is superior to ultrasound in the detection of cardiac abnormality, including biscupid aortic valve. Aortic root dimensions are best expressed as aortic size index (ASI), to allow for the short stature. Adult management includes attention to obesity, hyperlipidaemia and BP, with regular cardiology follow-up1. Particularly careful surveillance is needed in order to pre-empt aortic dissection in pregnancy, and in women with ASI >2 cm/m2.

Reference: 1. Turtle et al. Cardiovascular risk in adults with TS. Clin Endocrinol 2013 78 639–645.

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