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Cardiac disease in the antiphospholipid syndrome: recommendations for treatment. Committee consensus report

Hospital for Special Surgery, New York, USA, LockshinM{at}hss.edu

F Tenedios

Cornell-Weill Medical Center, New York, USA

M Petri

Johns Hopkins University, Baltimore, Maryland, USA

G McCarty

University of Virginia, Charlottesville, Virginia, USA

R Forastiero

Universidad Favaloro, Buenos Aires, Argentina

S Krilis

St George Hospital, UNSW, Korgarah, New South Wales, Australia

A Tincani

Ospedale Civile di Brescia, Brescia, Italy

D Erkan

Hospital for Special Surgery, New York, USA

M A Khamashta

Lupus Unit, The Rayne Institute, London, UK

Y Shoenfeld

Chaim Sheba Medical Center, Tel-Hashomer, Israel

The Committee reviewed cardiac involvement in the antiphospholipid antibody syndrome. The Committee’s recommendations are:

Valve abnormalities: anticoagulation is recommended for symptomatic patients with valvulopathy. Prophylactic antiplatelet therapy may be appropriate for asymptomatic patients (recommended by 13/17 experts in an independent review). Committee members disagreed whether corticosteroid therapy is helpful, but agree that distinguishing among presumptive valvulitis (valve thickening on echocardiogram), valve deformity and vegetations is important, as treatment implications may differ.

Occlusive arterial disease (angina, myocardial infarction): the Committee recommends aggressive treatment of all risk factors for atherosclerosis (hypertension, hypercholesterolaemia, smoking) and liberal use of folic acid, B vitamins and cholesterol-lowering drugs (preferably statins). Hydroxychloroquine for cardiac protection in APS patients may be considered. The Committee also recommends warfarin anticoagulationfor those who have suffered thrombosis in the absence of atherosclerosis, but recognizesthat developingdata may support the use of antiplateletagents instead.

Intracardiac thrombi: the Committee recommends intensive warfarin anticoagulation, and consultation with cardiac surgeons when appropriate.

Ventricular dysfunction: the Committee has no recommendations on this aspect of cardiac disease. Pulmonary hypertension: the Committee recommends intensive anticoagulationwith warfarin and clinical trials of bosentan, epoprostenol and other new agents.

Key Words: antiphospholipid • heart • pulmonary hypertension • valve

Lupus, Vol. 12, No. 7, 518-523 (2003)
DOI: 10.1191/0961203303lu391oa


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