Task force report on non-criteria manifestations: Cardiac valve disease

Abstract

Cardiac valve disease is the most common cardiac manifestation of antiphospholipid syndrome (APS). An international consensus statement published in 2006 provided relevant definitions of heart valve lesions in APS. According to this consensus, valve lesions include valve thickness >3 mm, localized thickening involving the leaflet’s proximal or middle portion, and irregular nodules on the atrial face of the edge of the mitral valve and/or the vascular face of the aortic valve. The pathogenic role of antiphospholipid antibodies (aPLs) in the development of heart valve lesions is unknown. Furthermore, the prevalence of valvular disease in primary APS patients as well as aPL-positive systemic lupus erythematosus patients is controversial. The main reasons for these controversies are the different methodologies employed to detect valve lesions (transthoracic (TTE) or transesophageal (TEE) echocardiography) and the cutoff levels in the assessment of aPL. The “Task Force on Catastrophic APS and Non-criteria APS Manifestations” developed in 2010 as part of the 13th International Congress on aPL held in Galveston, Texas, USA, made the following recommendations: (a) in patients with APS and previous thrombosis, mainly with arterial involvement, a TTE is recommended; (b) with normal valves and in the absence of atherosclerotic factors, follow-up TTE might not be necessary; (c) if heart valve lesions exist, serial echocardiographic follow-ups are warranted; and (d) large-scale studies examining the accuracy of heart valve lesions detected by TEE for the diagnosis of APS are warranted.