EXTENDED REPORT Close association between valvar heart disease and central nervous system manifestations in the antiphospholipid syndrome

Background: Heart valves lesions and central nervous system involvement are among the most common manifestations of the antiphospholipid syndrome (APS). Objective: To evaluate possible interrelations between these manifestations in a large group of APS patients. Methods: 284 APS patients were evaluated retrospectively, 159 of whom had primary APS. Cardiac–CNS associations were determined for the entire study population, and for subgroups of patients with primary APS or APS associated with systemic lupus erythematosus (SLE). Results: Significant associations where found between cardiac vegetations and epilepsy (p,0.02), and between cardiac valve thickening or dysfunction and migraine (p = 0.002). Borderline association was found between valvar vegetations and migraine (p = 0.09). A significant association was also found between all valvar lesions and stroke or transient ischaemic attacks. Subanalyses showed that patients with primary APS had significant associations between cardiac valve pathology and all CNS manifestations, while patients with APS associated with SLE had no such associations. Conclusions: The study suggests potential differences in biological behaviour between primary APS and APS associated with SLE. The presence of cardiac valve pathology may be a risk factor for several types of CNS involvement in PAPS.