The occurrence of cardiac manifestations and their relationship with t
he lupus anticoagulant (LA) in SLE was studied iii 74 patients who wer
e followed up for 22 years (median), of which 16 years were after the
initial LA testing. Pericarditis was the most common cardiac event occ
uring in 16 (22%) patients but it did not correlate with LA. Valvular
heart disease, coronary artery disease, left ventricular failure and/o
r cor pulmonale were observed in 16 (22%) patients. Taken together, th
eir occurrence was associated with a history of leg ulcers (odds 3.8,
P = 0.028) but not with LA or other common clinical manifestations of
the antiphospholipid syndrome. Valvular heart disease in five patients
was significantly associated with LA (P = 0.05). Cor pulmonale due to
chronic pulmonary embolism was present in two patients with LA. Myoca
rdial infarctions in five patients occurred late in the course of dise
ase but in relatively young patients (mean 43 years). Fatal myocardial
infarction in the absence of atherosclerosis in two LA-positive patie
nts supports a pathogenetic role for LA in these cases. In conclusion,
of the various cardiac complications in SLE, valvular heart disease a
nd cor pulmonale appear to be connected with the antiphospholipid synd
rome. Both conditions should be actively sought in patients with LA to
decrease possible adverse events (arterial emboli and right ventricul
ar failure) affecting the patients' prognosis.