311 Nonbacterial thrombotic (Libman-Sacks) endocarditis with mitral regurgitation in catastrophic antiphospholipid syndrome (a case study)

Antiphospholipid syndrome (APS) is one of several prothrombotic states in which thrombi occur within both the venous and arterial beds. A minority of patients with APS present with an acute and devastating syndrome characterized by multiple simultaneous vascular occlusions throughout the body. “Catastrophic APS” is defined by the clinical involvement of at least three different organ systems over a period of days or weeks with histological evidence of multiple occlusions of large or small vessels. We report the case of a 16-year-old girl referred to our paediatric cardiology unit for NHYA IV and a blowing systolic murmur at the apex radiating to the left axilla. Transthoracic echocardiography (TTE) revealed mitral valve leaflet thickening with vegetations (13 mm) on the edges of both leaflets and moderate mitral regurgitation (MR) (image 1). The diagnosis of Libman-Sacks or non-bacterial thrombotic endocarditis secondary to antiphospholipid syndrome was suggested by repeated negative blood cultures along with persistently elevated anticardiolipin antibody titers. The condition deteriorated to acute thrombotic microangiopathy affecting multiple organs with arterial hypertension and thrombocytopenia. Anticoagulation with warfarin was peformed and aspirin, corticosteroids and ACE inhibitors were given. TTE follow-up after 1.5 years revealed no recurrence of MR with normal mitral valve leaflets. Conclusion In catastrophic APS an aggressive therapeutic approach is warranted. Valve lesions may become more severe during long term follow-up. Download full-size image