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141 Cardcardiovascular Manifestations of Turner Syndrome in a Tertiary Centre Population
  1. Agnes Hamilton-Baillie1,
  2. Andrew Toogood2,
  3. Paul Clift2
  1. 1University Hospitals Birmingham
  2. 2University Hospitals Birmingham NHS Foundation Trust

Abstract

Introduction Turner Syndrome is associated with increased risk of congenital heart defects. We sought to detail cardiovascular abnormalities in a tertiary centre Turner Syndrome clinic.

Method A comprehensive list was compiled of 67 Turner Syndrome patients under QEHB care. MRI and echocardiographic scans were reviewed for evidence of cardiovascular anomalies. Case notes were reviewed for previous interventions, use of oestrogen, and evidence of hypertension. The use of ACE inhibitors (ACEi)/angiotensin II receptor antagonists (A2A) was recorded.

Results 57/67 (85%) of patients had undergone either MRI or echocardiographic evaluation. The following abnormalities were found: bicuspid aortic valve (BAV) 25/67 (37%), coarctation 16/67 (24%), aortic dilatation 10/67 (15%), arterial aberrancy 10/67 (15%), systemic venous anomaly 6/67 (9%), and pulmonary venous anomaly 6/67 (9%).

Interventions (surgery or catheter based) occurred in 18/67 patients (27%), 13/67 (19%) had BP >140/90 and 19/67 (28%) were on either ACEi or A2A. 39 patients had indexed MRI measurements available of which 11/39 (28%) has an enlarged aorta defined by indexed aortic dimension >2.0 cm/m2, with 2/39 (5%) with an indexed aortic dimension >2.5 cm/m2.

Conclusions Cardiovascular abnormalities (including bicuspid aorta, coarctation and increased aortic size – all risk factors for dissection) are common in Turner Syndrome patients. Patients with Turner Syndrome require regular cardiovascular imaging to identify and monitor these abnormalities.

  • Turner Syndrome
  • Cardiovascular abnormality
  • Cardiovascular imaging

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