Interessamento cardiaco in corso di sindrome da anticorpi antifosfolipidi

The antiphospholipid syndrome (APS) is an acquired thrombotic disorder characterised by recurrent venous or arterial thrombosis and/or miscarriages, or both, associated with the presence in the serum of IgG or IgM anticardiolipin antibodies (aCL) and/or lupus anticoagulant (LAC). APS may occur as a primary disorder (PAPS) or associated with connective tissue diseases, mainly systemic lupus eythematosus (secondary APS). Cardiac manifestations of primary antiphospholipid syndrome have been documented and involve structural and valvular heart disease. Previous studies have demonstrated myocardial involvement in patients with PAPS in the form of valvular disease pericardial effusion, coronary artery occlusion and intracardiac thrombi. The pathogenesis of valvulopathy may involve interaction of aPL with antigens on the valve surface, resulting in valvulitis. Several authors show that significant morbidity from valvular dysfunction, mostly mitral regurgitation leading to congestive heart failure, occurs in 4% of PAPS patients and 6% of secondary APS, such as aCL-positive Systemic Lupus Erythematosus (SLE), respectively. There is also some evidence that aCL may also be accompanied by a decrease in the systolic and diastolic function of the left ventricle, and, at some extent, a right ventricular dysfunction may also be observed. The cardiac involvement is apparently related to the aCL levels. Cardiologic complications are common in PAPS, including left side regurgitate lesions that might be haemodynamically significant, acute myocardial infarction, pericardial effusion and pulmonary hypertension. The current therapy includes treatment with aspirin, anticoagulant drugs, immunosuppressive agents and steroids and the surgical approach, consisting in replacement and/or repair of the valves.