VALVULAR HEART-DISEASE IN PRIMARY ANTIPHOSPHOLIPID SYNDROME (PAPS) - CLINICAL AND MORPHOLOGICAL FINDINGS

Purpose. To describe clinically and pathologically the valvular lesion of the primary antiphospholipid syndrome(PAPS). Patients and Methods. We studied six patients with PAPS and valvulopathy. Four of them died and had autopsy and two had valvular replacement. The study comprised 18 heart valves, 16 from autopsy and two, one mitral and one aortic, resected at surgery. Results. Murmurs and echocardiographic findings k ept correlation with gross pathology. Abnormalities were found in one or more valves in all patients including two of five aortic, two of fi ve mitral, one of four pulmonary and two of four tricuspid. Go-existen ce of new and old lesions was observed. Pathologic findings included i ntravalvular thrombosis with focal necrosis and hemorrhage, vascular p roliferation, mild histiocytic/fibroblastic infiltration, laminated an d verrucous fibrin deposits, laminated and/or nodular fibrosis, and fo cal calcification. Conclusion. The PAPS valvular lesion consists mainl y of superficial or intravalvular fibrin deposits and its subsequent o rganization: vascular proliferation, fibroblast influx, fibrosis and c alcification. This results in valve thickening, fusion and rigidity le ading to functional abnormalities. Inflammation is not a prominent fea ture of this lesion.