Valvular heart disease in primary antiphospholipid syndrome (PAPS): clinical and morphological findings

Purpose. To describe clinically and pathologically the valvular lesion of the primary anti phospholipid 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 kept correlation with gross pathology. Abnormalities were found in one or more valves in all patients including two of five aortic, two of five mitral, one of four pulmonary and two of four tricuspid. Co-existence of new and old lesions was observed. Pathologic findings included intravalvular thrombosis with focal necrosis and hemorrhage, vascular proliferation, mild histiocytic/fibroblastic infiltration, laminated and verrucous fibrin deposits, laminated and/or nodular fibrosis, and focal calcifi cation. Conclusion. The PAPS valvular lesion consists mainly of superficial or intravalvular fibrin deposits and its subsequent organization: vascular proliferation, fibroblast influx, fibrosis and calcification. This results in valve thickening, fusion and rigidity leading to functional abnormalities. Inflammation is not a prominent feature of this lesion.