Over a period of several months a 33-year-old man had recurrent pulmon
ary emboli. No thromboses could be demonstrated in the peripheral veno
us system. Transoesophageal echocardiography showed two spherical spac
e-occupying structures in the right ventricle which were removed opera
tively under the suspected diagnosis of multilobular myxomas. However,
their histological examination revealed pure thrombi that had grown b
y apposition. This unusual findings of right-ventricular thrombi could
not be explained pre- and intraoperatively by any local thrombi-favou
ring changes in the right heart. Tests of clotting mechanisms demonstr
ated lupus anticoagulans (kaolin-clotting-time mixture test: LA index
21.7 [normal: <15]), as well as an increased IgG cardiolipin antibody
concentration of 19.3 U/l). As no underlying disease was discovered, t
he diagnosis was by definition primary antiphospholipid syndrome. No f
urther thrombo-embolism has occurred during continuing oral anticoagul
ation with phenprocoumon.