Issue: 2010 > May > original article

Reversible cardiac valvular disease in catastrophic antiphospholipid syndrome

C.C. Teunisse, A.J. Kalsbeek, S.T. de Vries, S.J. Huisman, J.E. Boers, A. Breeman, J.R. Beukhof


Catastrophic antiphospholipid syndrome (CAPS) is a
severe form of antiphospholipid syndrome (APS). It
frequently leads to multiorgan failure with an approximate mortality rate of 50%. The heart is involved in about 50% of the patients with CAPS. We report two cases with CAPS and severe heart manifestations, documented by echocardiography. Both women show regression of the valvular regurgitation under treatment. Valve replacement therapy was no longer necessary. In earlier studies and case reports, cardiac valve involvement had been characterised by valve thickening and vegetations. We suppose that (sometimes reversible) microvascular disturbances lead to valvular regurgitation via papillary muscle dysfunction and myocardial stunning.