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A 31-year-old man presented with progressive reduction in temperature and sensation in his left lower leg. CT angiography disclosed thrombosis in the left popliteal artery and right profunda artery. He was investigated for source of possible emboli.
Transoesophageal echocardiography showed a mass on the right coronary cusp of the aortic valve, measuring approximately 1.5×1.5 cm, consistent with endocarditis. There was no significant aortic stenosis or regurgitation (panel A).
A thorough history and septic screen were negative; therefore he was investigated for non-infective causes of endocarditis. Although antinuclear antibodies were negative, anticardiolipin antibodies were strongly positive, suggesting a diagnosis of primary antiphospholipid syndrome. There was no regression of the valve mass with anticoagulation and in view of the embolic events, he underwent valve-sparing surgery to remove the mass lesion and was subsequently re-anticoagulated before discharge. A histological examination showed an organising necrotic fibrinous mass with chronic inflammatory cells, and a culture did not grow any pathogens (panel B).
A diagnosis was made of Libman–Sacks endocarditis complicated by arterial emboli, as a first presentation of antiphospholipid syndrome.
Although Libman–Sacks endocarditis was first described in systemic lupus erythematosus, it is more common to find these features in patients with primary or secondary antiphospholipid syndrome, the autoantibodies thought to be promoters of thrombus formation. Between one and two-thirds of these patients have heart valve abnormalities. In addition, thrombotic phenomena are more common if antiphospholipid antibodies are positive. Recommended treatment consists of long-term anticoagulation which will usually proceed to regression of valve disease, valve surgery being reserved for those with continuing endocarditis and embolic phenomena.
Acknowledgments
This article has been adapted from Aldous S. An interesting presentation of antiphospholipid syndrome Heart 2008;94:421