A 38-yr-old man with an unusual type of Ehlers-Danlos syndrome presented for elective abdominal aortic aneurysm repair. During surgery he developed acute myocardial ischaemia, resulting in abandonment of the procedure. He was shown subsequently to have severe triple vessel coronary artery disease. Silent ischaemia associated with severe coronary artery disease, although rare, may be associated with the syndrome and is difficult to recognize as other cardiac abnormalities are frequently present.
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